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1. Masui F, Yokoyama R, Soshi S, Beppu Y, Asanuma K, Fujii K: A malignant peripheral nerve-sheath tumour responding to chemotherapy. J Bone Joint Surg Br; 2004 Jan;86(1):113-5
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] A malignant peripheral nerve-sheath tumour responding to chemotherapy.
  • A malignant peripheral nerve-sheath tumour developed in the right S1 nerve root in a man aged 30 causing back pain and sciatica.
  • CT and MRI revealed a destructive tumour of the sacrum invading the retroperitoneal space.
  • The tumour was not resectable with an adequate margin.
  • Chemotherapy, consisting of high-dose ifosfamide followed by a combination of vincristine, doxorubicin and cyclophosphamide, was given with success.
  • Malignant peripheral nerve-sheath tumours are thought to respond weakly to chemotherapy, but the response in our patient was complete.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Nerve Sheath Neoplasms / drug therapy

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  • (PMID = 14765877.001).
  • [ISSN] 0301-620X
  • [Journal-full-title] The Journal of bone and joint surgery. British volume
  • [ISO-abbreviation] J Bone Joint Surg Br
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 5J49Q6B70F / Vincristine; 80168379AG / Doxorubicin; 8N3DW7272P / Cyclophosphamide; UM20QQM95Y / Ifosfamide
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2. Kosmas C, Tsakonas G, Evgenidi K, Gassiamis A, Savva L, Mylonakis N, Karabelis A: Malignant peripheral nerve sheath tumor in neurobifromatosis type-1: two case reports. Cases J; 2009;2:7612

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Malignant peripheral nerve sheath tumor in neurobifromatosis type-1: two case reports.
  • INTRODUCTION: Malignant peripheral nerve sheath tumors are rare soft tissue sarcomas.
  • They are considered to carry a poor prognosis with current therapeutic approaches.
  • Successful treatment depends on a multimodal approach.
  • CASE PRESENTATION: The authors report two cases with malignant peripheral nerve sheath tumors arising from pre-existing neurofibromas in the grounds of neurofibromatis-type I.
  • Complete surgical removal of all lesions is considered before and after induction chemotherapy.
  • Correlation of the response to chemotherapy in the context of the immuno-histopathological features of the tumors is also discussed with reference to the existing literature.
  • CONCLUSION: A need for a multidisciplinary approach with chemotherapy, surgery and radiotherapy is anticipated in the management of malignant peripheral nerve sheath tumors as described in these two reported cases.
  • It is felt that further research on the molecular aspects of malignant peripheral nerve sheath tumors and neurofibromatis-type I will optimize treatment strategies in the future.

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  • [Cites] Mol Cancer Ther. 2008 May;7(5):1237-45 [18483311.001]
  • [Cites] Am J Hum Genet. 2001 May;68(5):1110-8 [11283797.001]
  • [Cites] J Surg Oncol. 2008 Mar 15;97(4):340-9 [18286466.001]
  • [Cites] J Pediatr (Rio J). 2007 Nov-Dec;83(6):571-3 [18046492.001]
  • [Cites] Oncol Rep. 2007 Oct;18(4):763-7 [17786333.001]
  • [Cites] Paediatr Drugs. 2007;9(4):239-48 [17705563.001]
  • [Cites] Anticancer Res. 2007 Jul-Aug;27(4A):1957-60 [17649804.001]
  • [Cites] Ann Oncol. 2004 Nov;15(11):1667-72 [15520069.001]
  • [Cites] Am J Med Genet. 1997 May 16;70(2):138-43 [9128932.001]
  • [Cites] J Clin Oncol. 2003 Dec 15;21(24):4586-91 [14673046.001]
  • [Cites] Am J Surg Pathol. 2003 Oct;27(10):1337-45 [14508395.001]
  • [Cites] Cancer Res. 2002 Mar 1;62(5):1573-7 [11894862.001]
  • [Cites] Oncol Rep. 2002 May-Jun;9(3):627-30 [11956640.001]
  • [Cites] Lancet. 2001 Oct 27;358(9291):1421-3 [11705489.001]
  • [Cites] J Clin Pathol. 2001 Aug;54(8):631-6 [11477120.001]
  • [Cites] Acta Neurochir (Wien). 2001;143(4):357-63; discussion 363-4 [11437289.001]
  • [Cites] Mol Cancer Ther. 2008 Apr;7(4):890-6 [18413802.001]
  • (PMID = 19830003.001).
  • [ISSN] 1757-1626
  • [Journal-full-title] Cases journal
  • [ISO-abbreviation] Cases J
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Other-IDs] NLM/ PMC2740005
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3. Slomiany MG, Dai L, Bomar PA, Knackstedt TJ, Kranc DA, Tolliver L, Maria BL, Toole BP: Abrogating drug resistance in malignant peripheral nerve sheath tumors by disrupting hyaluronan-CD44 interactions with small hyaluronan oligosaccharides. Cancer Res; 2009 Jun 15;69(12):4992-8
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Abrogating drug resistance in malignant peripheral nerve sheath tumors by disrupting hyaluronan-CD44 interactions with small hyaluronan oligosaccharides.
  • Malignant peripheral nerve sheath tumors (MPNST) develop in approximately 10% of neurofibromatosis type-1 patients and are a major contributing factor to neurofibromatosis-1 patient mortality and morbidity.
  • MPNSTs are multidrug resistant, and thus long-term patient survival rates are poor after standard doxorubicin or multiagent chemotherapies.
  • We show that the hyaluronan receptor CD44 forms complexes with multidrug transporters, BCRP (ABCG2) and P-glycoprotein (ABCB1), in the plasma membrane of human MPNST cells.
  • Treatment of MPNST cells with the hyaluronan oligomers causes disassembly of CD44-transporter complexes and induces internalization of CD44, BCRP, and P-glycoprotein.
  • Consequently, the oligomers suppress drug transporter activity and increase sensitivity to doxorubicin treatment in culture.
  • In vivo, systemic administration of hyaluronan oligomers inhibits growth of MPNST xenografts.
  • Moreover, the oligomers and doxorubicin act synergistically in vivo, in that combined suboptimal doses induce tumor regression to a greater extent than the additive effects of each agent alone.
  • These findings indicate that constitutive hyaluronan-CD44 interactions contribute to drug transporter localization and function at the plasma membrane, and that attenuating hyaluronan-CD44 interactions sensitizes MPNSTs to doxorubicin in vitro and in vivo.
  • These results also show the potential efficacy of hyaluronan oligomers, which are nontoxic and nonimmunogenic, as an adjuvant for chemotherapy in MPNST patients.
  • [MeSH-major] Antigens, CD44 / metabolism. Drug Resistance, Neoplasm. Hyaluronic Acid / metabolism. Nerve Sheath Neoplasms / drug therapy. Oligosaccharides / therapeutic use
  • [MeSH-minor] Cell Line, Tumor. Doxorubicin / therapeutic use. Drug Synergism. Humans. Immunoprecipitation. Microscopy, Confocal

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  • [Cites] J Biol Chem. 2001 Dec 28;276(52):48679-92 [11606575.001]
  • [Cites] J Biol Chem. 2000 Sep 1;275(35):26967-75 [10871609.001]
  • [Cites] Appl Immunohistochem Mol Morphol. 2002 Mar;10(1):34-9 [11893033.001]
  • [Cites] Nat Rev Cancer. 2002 Jan;2(1):48-58 [11902585.001]
  • [Cites] J Clin Neurosci. 2002 May;9(3):282-8 [12093135.001]
  • [Cites] J Biol Chem. 2002 Oct 11;277(41):38013-20 [12145277.001]
  • [Cites] J Child Neurol. 2002 Aug;17(8):548-54; discussion 571-2, 646-51 [12403552.001]
  • [Cites] Glia. 2003 Jun;42(4):350-8 [12730955.001]
  • [Cites] Hum Genet. 2003 Jul;113(2):178-87 [12728312.001]
  • [Cites] J Biol Chem. 2003 Jul 11;278(28):25285-8 [12738783.001]
  • [Cites] Glia. 2004 Feb;45(3):297-306 [14730703.001]
  • [Cites] J Biol Chem. 2004 Jun 25;279(26):26991-7007 [15090545.001]
  • [Cites] Nat Rev Cancer. 2004 Jul;4(7):528-39 [15229478.001]
  • [Cites] Biochem Pharmacol. 2004 Oct 1;68(7):1401-10 [15345330.001]
  • [Cites] Cytometry A. 2004 Oct;61(2):105-16 [15382145.001]
  • [Cites] J Biol Chem. 1983 Jul 10;258(13):8086-91 [6190806.001]
  • [Cites] J Biol Chem. 1993 Oct 15;268(29):21493-6 [8104940.001]
  • [Cites] J Neurooncol. 1995 Dec;26(3):171-84 [8750183.001]
  • [Cites] Int J Cancer. 1998 Jul 29;77(3):396-401 [9663602.001]
  • [Cites] J Biol Chem. 2005 Mar 11;280(10):8875-83 [15632176.001]
  • [Cites] J Biol Chem. 2005 May 27;280(21):20310-5 [15784621.001]
  • [Cites] Cancer Res. 2005 Aug 1;65(15):6660-7 [16061646.001]
  • [Cites] Nat Rev Cancer. 2005 Jul;5(7):557-64 [16069817.001]
  • [Cites] Arch Otolaryngol Head Neck Surg. 2006 Jul;132(7):771-8 [16847188.001]
  • [Cites] Blood Rev. 2006 Nov;20(6):333-42 [16920238.001]
  • [Cites] J Biol Chem. 2006 Nov 17;281(46):34936-41 [16959784.001]
  • [Cites] Arch Otolaryngol Head Neck Surg. 2007 Mar;133(3):281-8 [17372087.001]
  • [Cites] Cancer Res. 2007 Apr 1;67(7):2908-11 [17409392.001]
  • [Cites] Biochem J. 2007 Jun 1;404(2):327-36 [17324121.001]
  • [Cites] Cancer Lett. 2007 Jul 18;252(2):225-34 [17276588.001]
  • [Cites] J Biol Chem. 2007 Jul 20;282(29):20999-1004 [17540771.001]
  • [Cites] J Natl Cancer Inst. 2007 Oct 3;99(19):1441-54 [17895480.001]
  • [Cites] J Biol Chem. 2007 Dec 21;282(51):36777-81 [17981795.001]
  • [Cites] Int J Cancer. 2008 Mar 1;122(5):1012-8 [17985348.001]
  • [Cites] J Surg Oncol. 2008 Mar 15;97(4):340-9 [18286466.001]
  • [Cites] J Invest Dermatol. 2008 Apr;128(4):957-71 [17943188.001]
  • [Cites] Clin Cancer Res. 2008 Mar 15;14(6):1804-13 [18347183.001]
  • [Cites] J Biol Chem. 2008 May 23;283(21):14335-44 [18326857.001]
  • [Cites] Drug Resist Updat. 2008 Jun;11(3):110-21 [18490190.001]
  • [Cites] Pediatrics. 2009 Jan;123(1):124-33 [19117870.001]
  • [Cites] Cancer Res. 2009 Feb 15;69(4):1293-301 [19176383.001]
  • [Cites] Eur J Cancer. 2002 Feb;38(3):418-26 [11818209.001]
  • (PMID = 19470767.001).
  • [ISSN] 1538-7445
  • [Journal-full-title] Cancer research
  • [ISO-abbreviation] Cancer Res.
  • [Language] eng
  • [Grant] United States / NCRR NIH HHS / RR / C06 RR015455; United States / NCI NIH HHS / CA / R01 CA073839; United States / NCI NIH HHS / CA / R01 CA073839; United States / NCI NIH HHS / CA / R01 CA082867; United States / NCI NIH HHS / CA / R01 CA082867
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antigens, CD44; 0 / Oligosaccharides; 80168379AG / Doxorubicin; 9004-61-9 / Hyaluronic Acid
  • [Other-IDs] NLM/ NIHMS463086; NLM/ PMC3655760
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4. Miller SJ, Rangwala F, Williams J, Ackerman P, Kong S, Jegga AG, Kaiser S, Aronow BJ, Frahm S, Kluwe L, Mautner V, Upadhyaya M, Muir D, Wallace M, Hagen J, Quelle DE, Watson MA, Perry A, Gutmann DH, Ratner N: Large-scale molecular comparison of human schwann cells to malignant peripheral nerve sheath tumor cell lines and tissues. Cancer Res; 2006 Mar 1;66(5):2584-91
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Large-scale molecular comparison of human schwann cells to malignant peripheral nerve sheath tumor cell lines and tissues.
  • Malignant peripheral nerve sheath tumors (MPNST) are highly invasive soft tissue sarcomas that arise within the peripheral nerve and frequently metastasize.
  • To identify molecular events contributing to malignant transformation in peripheral nerve, we compared eight cell lines derived from MPNSTs and seven normal human Schwann cell samples.
  • We found that MPNST lines are heterogeneous in their in vitro growth rates and exhibit diverse alterations in expression of pRb, p53, p14(Arf), and p16(INK4a) proteins.
  • All MPNST cell lines express the epidermal growth factor receptor and lack S100beta protein.
  • Global gene expression profiling using Affymetrix oligonucleotide microarrays identified a 159-gene molecular signature distinguishing MPNST cell lines from normal Schwann cells, which was validated in Affymetrix microarray data generated from 45 primary MPNSTs.
  • Expression of Schwann cell differentiation markers (SOX10, CNP, PMP22, and NGFR) was down-regulated in MPNSTs whereas neural crest stem cell markers, SOX9 and TWIST1, were overexpressed in MPNSTs.
  • Previous studies have implicated TWIST1 in apoptosis inhibition, resistance to chemotherapy, and metastasis.
  • Reducing TWIST1 expression in MPNST cells using small interfering RNA did not affect apoptosis or chemoresistance but inhibited cell chemotaxis.
  • Our results highlight the use of gene expression profiling in identifying genes and molecular pathways that are potential biomarkers and/or therapeutic targets for treatment of MPNST and support the use of the MPNST cell lines as a primary analytic tool.
  • [MeSH-major] Nerve Sheath Neoplasms / genetics. Schwann Cells / physiology
  • [MeSH-minor] Apoptosis / physiology. Cell Line, Tumor. Cell Movement / physiology. Drug Resistance, Neoplasm. Gene Expression Profiling. Humans. Nuclear Proteins / biosynthesis. Nuclear Proteins / genetics. Oligonucleotide Array Sequence Analysis. RNA, Small Interfering / genetics. Transfection. Twist Transcription Factor / biosynthesis. Twist Transcription Factor / genetics


5. Sar C, Eralp L: Metastatic spinal neurofibrosarcoma. Arch Orthop Trauma Surg; 2002 Mar;122(2):106-8
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Metastatic spinal neurofibrosarcoma.
  • Neurofibrosarcomas are rare tumors usually arising in somatic soft tissues or peripheral nerves.
  • Four cases of metastatic neurofibrosarcoma to the spine have been reported before.
  • A 30-year-old woman with neurofibromatosis and a history of previous neurofibrosarcoma resection presented with back pain.
  • After the completion of adjuvant chemotherapy, a solitary pulmonary nodule was detected.
  • Though local control can be achieved in more than 80% of the patients with neurofibrosarcoma by wide surgical resection followed by adjuvant chemo- and radiotherapy, most patients die of systemic metastasis.
  • The current patient survived 50 months after the initial resection of a forearm neurofibrosarcoma.
  • Prolonged survival with the help of chemo- and radiotherapy justifies our aggressive surgical strategy for the treatment of spinal metastasis in order to achieve neurologic cure and spinal stability.
  • [MeSH-major] Neurofibrosarcoma / secondary. Spinal Neoplasms / secondary. Spinal Neoplasms / therapy
  • [MeSH-minor] Adult. Biopsy, Needle. Bone Neoplasms / pathology. Bone Neoplasms / therapy. Chemotherapy, Adjuvant. Combined Modality Therapy. Female. Follow-Up Studies. Forearm. Humans. Immunohistochemistry. Magnetic Resonance Imaging. Radiotherapy, Adjuvant. Spinal Fusion. Treatment Outcome

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  • (PMID = 11880913.001).
  • [ISSN] 0936-8051
  • [Journal-full-title] Archives of orthopaedic and trauma surgery
  • [ISO-abbreviation] Arch Orthop Trauma Surg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Germany
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6. Kolarov V, Stanić J, Eri Z, Zvezdin B, Kojičić M, Hromis S: Intrathoracic malignant peripheral nerve sheath tumor with poor outcome: a case report. Bosn J Basic Med Sci; 2010 Nov;10(4):328-30
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Intrathoracic malignant peripheral nerve sheath tumor with poor outcome: a case report.
  • We report a case of intrathoracic malignant peripheral nerve sheath tumor in a 65-year old woman revealed after a few-month history of progressive dyspnea, appetite and body mass loss.
  • The chest magnetic resonance (MR) examination revealed the presence of a large tumor occupying the mediastinum and a major portion of the right hemithorax.
  • The diagnostic tumor sample was obtained by parasternal biopsy in local anesthesia.
  • The surgical resection of the tumor could not be performed due to its excessive size, intrathoracic involvement and bad respiratory reserves of a patient.
  • The chemotherapy and irradiation were performed as palliative measures.
  • The lethal outcome appeared 10 months after the diagnosis was established.
  • [MeSH-major] Mediastinal Neoplasms / diagnosis. Mediastinal Neoplasms / therapy. Nerve Sheath Neoplasms / diagnosis. Nerve Sheath Neoplasms / therapy. Peripheral Nerves / pathology
  • [MeSH-minor] Aged. Anesthesia / methods. Biopsy / methods. Fatal Outcome. Female. Humans. Lung / pathology. Magnetic Resonance Imaging / methods. Mediastinum / pathology. Pulmonary Disease, Chronic Obstructive / complications. Thorax / pathology. Treatment Outcome

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  • [Cites] Cancer Res. 2002 Mar 1;62(5):1573-7 [11894862.001]
  • [Cites] J Chin Med Assoc. 2006 Jan;69(1):37-41 [16447925.001]
  • [Cites] Chest. 2005 Oct;128(4):2893-909 [16236967.001]
  • [Cites] Ann Thorac Cardiovasc Surg. 2008 Feb;14(1):42-7 [18292741.001]
  • [Cites] Pediatr Ann. 2007 Oct;36(10):632, 634-5 [17969530.001]
  • [Cites] World J Surg Oncol. 2006 Aug 22;4:55 [16923196.001]
  • (PMID = 21108617.001).
  • [ISSN] 1840-4812
  • [Journal-full-title] Bosnian journal of basic medical sciences
  • [ISO-abbreviation] Bosn J Basic Med Sci
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Bosnia and Herzegovina
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7. Lee CS, Huh JS, Chang JW, Park JK: The early detection of recurrence of malignant peripheral nerve sheath tumor by frequent magnetic resonance imaging. J Korean Neurosurg Soc; 2010 Jan;47(1):51-4

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] The early detection of recurrence of malignant peripheral nerve sheath tumor by frequent magnetic resonance imaging.
  • Surgery has a key role in the treatment of malignant peripheral nerve sheath tumors (MPNSTs), but the resectability of paraspinal MPNSTs is only 20%.
  • Therefore, spinal MPNSTs show frequent recurrence and poor prognosis.
  • Local recurrence is much more common than metastasis for MPNSTs, and surgery still has a key role in the treatment of local recurrence.
  • However, no evidence-based follow-up protocol has been established for MPNST.
  • The authors performed gross total resection in a 34-year-old woman presented with thoracic MPNST.
  • Adjuvant radiotherapy and chemotherapy were not administered since these adjuvant therapies generally do not improve survival in MPNST and may cause additional neurovascular damage.
  • The tumor recurred locally on two occasions without overt symptoms at 21 and 24 months postoperatively.

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  • [Cites] Neurosurg Focus. 2007;22(6):E6 [17613223.001]
  • [Cites] Neurosurg Focus. 2007;22(6):E13 [17613204.001]
  • [Cites] Cancer. 1986 May 15;57(10):2006-21 [3082508.001]
  • [Cites] Int J Radiat Oncol Biol Phys. 1998 Sep 1;42(2):351-60 [9788415.001]
  • [Cites] Neurosurg Focus. 2007;22(6):E12 [17613203.001]
  • (PMID = 20157379.001).
  • [ISSN] 1598-7876
  • [Journal-full-title] Journal of Korean Neurosurgical Society
  • [ISO-abbreviation] J Korean Neurosurg Soc
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Korea (South)
  • [Other-IDs] NLM/ PMC2817516
  • [Keywords] NOTNLM ; Follow-up / Malignant peripheral nerve sheath tumor / Recurrence
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8. Kim JG, Sung WJ, Kim DH, Kim YH, Sohn SK, Lee KB: Malignant peripheral nerve sheath tumor in neurofibromatosis type I: unusual presentation of intraabdominal or intrathoracic mass. Korean J Intern Med; 2005 Mar;20(1):100-4
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Malignant peripheral nerve sheath tumor in neurofibromatosis type I: unusual presentation of intraabdominal or intrathoracic mass.
  • A malignant peripheral nerve sheath tumor (MPNST) is an extremely rare soft tissue tumor in the general population.
  • On the other hand, there is a higher incidence of MPNST in patients with neurofibromatosis type I (von Recklinghausen's disease).
  • This paper reports two patients, a 31 year-old woman with multiple neurofibromatosis presenting as an intraabdominal malignant peripheral nerve sheath tumor, and a 33 year-old woman with an intrathoracic malignant peripheral nerve sheath tumor.
  • The patients were treated with chemotherapy followed by radiotherapy.
  • However, one patient died as a result of disease progression 21 months after the diagnosis and the other patient is currently being treated with radiotherapy.
  • [MeSH-major] Abdominal Neoplasms / diagnosis. Nerve Sheath Neoplasms / diagnosis. Neurofibromatosis 1 / complications. Thoracic Neoplasms / diagnosis

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  • [Cites] Cancer. 1986 May 15;57(10):2006-21 [3082508.001]
  • [Cites] J Neurosurg Sci. 1989 Jul-Sep;33(3):253-7 [2614511.001]
  • [Cites] Cancer. 1981 May 15;47(10):2503-9 [6791802.001]
  • [Cites] Cancer. 1980 Jan 1;45(1):126-9 [6766081.001]
  • [Cites] Virchows Arch A Pathol Anat Histol. 1979 Jul 27;383(1):43-57 [157612.001]
  • [Cites] Cancer. 1977 May;39(5):1955-8 [858125.001]
  • [Cites] Eur J Cancer. 2003 Jan;39(1):64-9 [12504660.001]
  • [Cites] Korean J Intern Med. 2001 Sep;16(3):201-4 [11769579.001]
  • [Cites] N Engl J Med. 1986 Apr 17;314(16):1010-5 [3083258.001]
  • [Cites] Cancer. 1963 Aug;16:1015-27 [14050005.001]
  • [Cites] Scand Cardiovasc J. 1998;32(3):173-5 [9764434.001]
  • [Cites] Lancet. 1997 Dec 6;350(9092):1647-54 [9400508.001]
  • [Cites] Nihon Kyobu Geka Gakkai Zasshi. 1996 Jun;44(6):864-8 [8753103.001]
  • [Cites] Science. 1990 Jul 13;249(4965):181-6 [2134734.001]
  • [Cites] N Engl J Med. 1981 Dec 31;305(27):1617-27 [6796886.001]
  • (PMID = 15906964.001).
  • [ISSN] 1226-3303
  • [Journal-full-title] The Korean journal of internal medicine
  • [ISO-abbreviation] Korean J. Intern. Med.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Korea (South)
  • [Other-IDs] NLM/ PMC3891405
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9. Neville H, Corpron C, Blakely ML, Andrassy R: Pediatric neurofibrosarcoma. J Pediatr Surg; 2003 Mar;38(3):343-6; discussion 343-6
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Pediatric neurofibrosarcoma.
  • BACKGROUND/PURPOSE: Neurofibrosarcoma is rare in children, and the natural history and prognostic factors are not well described.
  • METHODS: The charts of children with neurofibrosarcoma were reviewed retrospectively.
  • RESULTS: From 1944 to 2001, 38 patients under the age of 21 were diagnosed with neurofibrosarcoma.
  • The average age at diagnosis was 13.8 years (range, 3 to 19.9 years).
  • The tumor site was as follows: extremity, 19 patients; trunk, 9 patients; head and neck, 7 patients; and retroperitoneum, 3 patients.
  • The average tumor size was 10 cm.
  • Thirty-two patients achieved a complete response, 2 a partial response, and 4 progressed while on therapy.
  • Of the 15 patients with a distant relapse, 73% (11) relapsed in the lung.
  • Nine patients were treated with chemotherapy, 9 with radiation, and 9 with both chemotherapy and radiation.
  • Outcome was not significantly affected by gender, presence of neurofibromatosis, site, margin, or use of adjuvant therapy.
  • CONCLUSION: Neurofibrosarcoma remains a rare disease in children with insufficient contemporary numbers to assess efficacy of therapy.
  • Prognosis remains poor with a high incidence of relapse, particularly in the lungs, suggesting that more aggressive therapies to control both local and distant relapses are needed.
  • [MeSH-major] Neurofibrosarcoma / epidemiology. Soft Tissue Neoplasms / epidemiology
  • [MeSH-minor] Adolescent. Adult. Chemotherapy, Adjuvant. Child. Child, Preschool. Combined Modality Therapy. Female. Genetic Predisposition to Disease. Humans. Lung Neoplasms / epidemiology. Lung Neoplasms / secondary. Male. Neoplasms, Second Primary / epidemiology. Neurofibromatoses / epidemiology. Prognosis. Radiotherapy, Adjuvant. Retrospective Studies. Survival Rate. Texas / epidemiology. Treatment Outcome

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  • [Copyright] Copyright 2003, Elsevier Science (USA). All rights reserved.
  • (PMID = 12632346.001).
  • [ISSN] 1531-5037
  • [Journal-full-title] Journal of pediatric surgery
  • [ISO-abbreviation] J. Pediatr. Surg.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 18
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10. Katz D, Lazar A, Lev D: Malignant peripheral nerve sheath tumour (MPNST): the clinical implications of cellular signalling pathways. Expert Rev Mol Med; 2009;11:e30
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Malignant peripheral nerve sheath tumour (MPNST): the clinical implications of cellular signalling pathways.
  • Malignant peripheral nerve sheath tumour (MPNST) is a rare malignancy accounting for 3-10% of all soft tissue sarcomas.
  • Most MPNSTs arise in association with peripheral nerves or deep neurofibromas and may originate from neural crest cells, although the specific cell of origin is uncertain.
  • Approximately half of MPNSTs occur in the setting of neurofibromatosis type 1 (NF1), an autosomal dominant disorder with an incidence of approximately one in 3500 persons; the remainder of MPNSTs develop sporadically.
  • In addition to a variety of clinical manifestations, approximately 8-13% of NF1 patients develop MPNSTs, which are the leading cause of NF1-related mortality.
  • Surgical resection is the mainstay of MPNST clinical management.
  • However, because of invasive growth, propensity to metastasise, and limited sensitivity to chemotherapy and radiation, MPNST has a guarded to poor prognosis.
  • Five-year survival rates of only 20-50% indicate an urgent need for improved therapeutic approaches.
  • Recent work in this field has identified several altered intracellular signal transduction cascades and deregulated tyrosine kinase receptors, posing the possibility of personalised, targeted therapeutics.
  • However, expanded knowledge of MPNST molecular pathobiology will be needed to meaningfully apply such approaches for the benefit of afflicted patients.
  • [MeSH-major] Nerve Sheath Neoplasms / diagnosis. Nerve Sheath Neoplasms / metabolism. Neurofibromin 1 / metabolism. Protein Kinases / metabolism. Proto-Oncogene Proteins p21(ras) / metabolism

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  • (PMID = 19835664.001).
  • [ISSN] 1462-3994
  • [Journal-full-title] Expert reviews in molecular medicine
  • [ISO-abbreviation] Expert Rev Mol Med
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Neurofibromin 1; EC 2.7.- / Protein Kinases; EC 2.7.1.1 / MTOR protein, human; EC 2.7.1.1 / TOR Serine-Threonine Kinases; EC 2.7.10.1 / EGFR protein, human; EC 2.7.10.1 / Receptor, Epidermal Growth Factor; EC 2.7.10.1 / Receptors, Platelet-Derived Growth Factor; EC 2.7.10.1 / Receptors, Vascular Endothelial Growth Factor; EC 3.6.5.2 / Proto-Oncogene Proteins p21(ras)
  • [Number-of-references] 135
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11. Zambrana F, Vicente F, García-Manrique T, Pereira S, Sáinz De Zaitigui J, De La Cruz Merino L: Primary intracranial malignant peripheral nerve sheath tumour responding to chemotherapy. Clin Transl Oncol; 2010 Mar;12(3):231-3
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Primary intracranial malignant peripheral nerve sheath tumour responding to chemotherapy.
  • Malignant peripheral nerve sheath tumours (MPNST) are a rare variety of soft tissue sarcomas (STS) arising from major peripheral nerve branches and typically located in the lower extremity, chest wall or the retroperitoneum.
  • It is a biologically aggressive neoplasm for which the treatment of choice is surgery, but usually requires a multimodality approach, having been generally labelled as chemoresistant.
  • We present a case of MPNST located intracranially with a good response to chemotherapy.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Brain Neoplasms / drug therapy. Brain Neoplasms / pathology. Nerve Sheath Neoplasms / drug therapy

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  • [Cites] Cancer. 1981 May 15;47(10):2503-9 [6791802.001]
  • [Cites] J Clin Oncol. 1987 Apr;5(4):601-12 [3559651.001]
  • [Cites] Br J Neurosurg. 1999 Dec;13(6):550-7 [10715722.001]
  • [Cites] J Surg Oncol. 2008 Mar 15;97(4):340-9 [18286466.001]
  • [Cites] Am J Dermatopathol. 2000 Jun;22(3):217-29 [10871064.001]
  • [Cites] Am J Med Genet. 1999 Mar 26;89(1):23-30 [10469433.001]
  • [Cites] Cancer. 2006 Sep 1;107(5):1065-74 [16881077.001]
  • [Cites] J Clin Oncol. 2005 Nov 20;23(33):8422-30 [16293873.001]
  • [Cites] World J Surg Oncol. 2006 Aug 22;4:55 [16923196.001]
  • (PMID = 20231129.001).
  • [ISSN] 1699-3055
  • [Journal-full-title] Clinical & translational oncology : official publication of the Federation of Spanish Oncology Societies and of the National Cancer Institute of Mexico
  • [ISO-abbreviation] Clin Transl Oncol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Italy
  • [Chemical-registry-number] 80168379AG / Doxorubicin; UM20QQM95Y / Ifosfamide
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12. Chybicka A, Bogusławska-Jaworska J, Rosińska B, Wecławek-Tompol J, Armata J, Balcerska A, Balwierz W, Bubala H, Drabko K, Eliasinska A, Kedziora M, Sońta-Jakimczyk D, Sopylo B, Kołecki P, Kowalczyk J, Matysiak M, Rokicka-Milewska R, Stefaniak MJ, Stańczak E, Stencel D, Wysocki M, Płoszyńska A: [G-CSF and GM-CSF in treatment of neutropaenia after chemotherapy in children with neoplasms]. Med Wieku Rozwoj; 2000;4(1 Suppl 2):121-9
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  • [Title] [G-CSF and GM-CSF in treatment of neutropaenia after chemotherapy in children with neoplasms].
  • A total number of 608 cycles of G-CSF and/or GM-CSF was applied in 280 patients aged from 6 months to 20 years during neutropaenia associated with chemotherapy of children's neoplasms (NHL-124, NBL-42, RMS-36, Nephroblastoma-18, Osteosarcoma-17, Ewing's Sarcoma-14, Hepatoblastoma-6, Neurofibrosarcoma-6, PNET-5, Medulloblastoma-3, Fibrohistiocytoma-3, Angiosarcoma-2, other - 4).
  • G-CSF - Neupogen (Filgastrim, Hoffman La Roche - 492 cycles) and GM-CSF - Leucomax (Molgramostim, Shering Plough - 116 cycles) were administered 5 mg/kg/day s.c.
  • Forty one children with malignancies (NHL -21 cases, solid tumours -17) treated before cytokines were in use served as a control group.
  • Our study demonstrated that G-CSF and GM-CSF therapy, gives a shorter period of neutropaenia, reduction of the number of febrile days, decreased frequency of infection and shortened its duration.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / adverse effects. Granulocyte Colony-Stimulating Factor / therapeutic use. Granulocyte-Macrophage Colony-Stimulating Factor / therapeutic use. Neutropenia / drug therapy
  • [MeSH-minor] Adolescent. Adult. Case-Control Studies. Child. Child, Preschool. Female. Humans. Infant. Male. Neoplasms / drug therapy. Survival Analysis. Time Factors

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  • (PMID = 12021471.001).
  • [Journal-full-title] Medycyna wieku rozwojowego
  • [ISO-abbreviation] Med Wieku Rozwoj
  • [Language] pol
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Poland
  • [Chemical-registry-number] 143011-72-7 / Granulocyte Colony-Stimulating Factor; 83869-56-1 / Granulocyte-Macrophage Colony-Stimulating Factor
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13. Ishimaru A, Hasegawa J, Shinbo K, Arahara T, Kinoshita Y, Shimoyama E, Korehisa M, Oonuki M, Miyazawa T, Itabashi M: [A case of retroperitoneal tumor successfully resected thanks to effective chemotherapy]. Gan To Kagaku Ryoho; 2009 Jun;36(6):1007-11

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [A case of retroperitoneal tumor successfully resected thanks to effective chemotherapy].
  • First, pathological diagnosis of sarcoma was made by fine needle aspiration, then secondary pathological diagnosis of suspected malignant schwannoma was made by a partial resection.
  • The final pathological diagnosis by operation was immature teratoma with embryonal carcinoma.
  • The preoperative diagnosis was difficult in this case, and the tumor had grown too large after partial resection to surgically resect.
  • The effective chemotherapy reduced this tumor enough to allow successful resection.
  • [MeSH-minor] Carcinoma, Embryonal / drug therapy. Carcinoma, Embryonal / pathology. Carcinoma, Embryonal / surgery. Humans. Male. Young Adult

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  • (PMID = 19542726.001).
  • [ISSN] 0385-0684
  • [Journal-full-title] Gan to kagaku ryoho. Cancer & chemotherapy
  • [ISO-abbreviation] Gan To Kagaku Ryoho
  • [Language] jpn
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Japan
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14. Simon Z, Ress Z, Toldi J, Trauninger A, Miltényi Z, Illés A: Rare association of Hodgkin lymphoma, Graves' disease and myasthenia gravis complicated by post-radiation neurofibrosarcoma: coincidence or genetic susceptibility? Int J Hematol; 2009 May;89(4):523-8
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  • [Title] Rare association of Hodgkin lymphoma, Graves' disease and myasthenia gravis complicated by post-radiation neurofibrosarcoma: coincidence or genetic susceptibility?
  • With Hodgkin lymphoma (HL), other (autoimmune) diseases may occasionally occur or associate, whereas as a late treatment-complication, second tumour may develop.
  • Due to the residual mediastinal tumour CRu was declared but later on no progression/relapse could be proved by PET.
  • In 2000 Graves's disease, in 2001 myasthenia gravis was diagnosed, which showed resistance for immunosuppressant drugs, thus plasmapheresis, intravenous immunoglobulin treatments were applied.
  • In 2005, the residual mediastinal tumour started progressive growth, which leads to thoracotomy in which the tumour was removed, it was malignant peripheral nerve sheath tumour.
  • The disease showed progression despite the chemotherapy applied and the patient died in 2007 due to respiratory failure.
  • The etiologic role of genetic predisposition and immune regulatory disorder must definitely be thought of, as the possibility of mere coincidence is extremely small.
  • Malignant peripheral nerve sheath tumour is a rare complication of irradiation, which underlines the importance of the risk or/and response adapted therapy of HL.
  • [MeSH-major] Genetic Predisposition to Disease / genetics. Graves Disease / complications. Hodgkin Disease / complications. Myasthenia Gravis / complications. Neurofibrosarcoma / radiotherapy
  • [MeSH-minor] Adult. Autopsy. Fatal Outcome. Female. Humans. Positron-Emission Tomography. Tomography, X-Ray Computed


15. Kinebuchi Y, Noguchi W, Igawa Y, Nishizawa O: Recurrent retroperitoneal malignant nerve sheath tumor associated with neurofibromatosis type 1 responding to carboplatin and etoposide combined chemotherapy. Int J Clin Oncol; 2005 Oct;10(5):353-6
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Recurrent retroperitoneal malignant nerve sheath tumor associated with neurofibromatosis type 1 responding to carboplatin and etoposide combined chemotherapy.
  • A 25-year-old man was referred to our hospital with left flank pain, and computed tomography (CT) and magnetic resonance imaging (MRI) revealed large retroperitoneal masses.
  • Physical examination revealed many café-au-lait spots and superficial neurofibromas, and a diagnosis of neurofibromatosis type 1 (von Recklinghausen's disease) was made.
  • The tumor was resected, and the pathological diagnosis was malignant peripheral nerve sheath tumor (MPNST).
  • He received four courses of chemotherapy with carboplatin and etoposide, and the metastatic lung lesions were markedly decreased.
  • After chemotherapy, complete resection of the remaining lung lesions was performed, and there has been no recurrence to date.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Nerve Sheath Neoplasms / drug therapy. Neurofibromatosis 1 / complications. Peripheral Nervous System Neoplasms / drug therapy. Retroperitoneal Neoplasms / drug therapy

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  • [Cites] Crit Rev Oncol Hematol. 1995 Oct;20(3):193-201 [8748009.001]
  • [Cites] Oncol Rep. 2002 May-Jun;9(3):627-30 [11956640.001]
  • [Cites] Lancet. 2003 May 3;361(9368):1552-4 [12737880.001]
  • [Cites] Ear Nose Throat J. 2003 Nov;82(11):862-5 [14661436.001]
  • [Cites] Urology. 1985 Sep;26(3):209-17 [3929440.001]
  • [Cites] Int J Radiat Oncol Biol Phys. 1998 Sep 1;42(2):351-60 [9788415.001]
  • [Cites] Radiographics. 2003 May-Jun;23 (3):790-4 [12740477.001]
  • (PMID = 16247664.001).
  • [ISSN] 1341-9625
  • [Journal-full-title] International journal of clinical oncology
  • [ISO-abbreviation] Int. J. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Japan
  • [Chemical-registry-number] 6PLQ3CP4P3 / Etoposide; BG3F62OND5 / Carboplatin
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16. Rawal A, Yin Q, Roebuck M, Sinopidis C, Kalogrianitis S, Helliwell TR, Frostick S: Atypical and malignant peripheral nerve-sheath tumors of the brachial plexus: report of three cases and review of the literature. Microsurgery; 2006;26(2):80-6

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Atypical and malignant peripheral nerve-sheath tumors of the brachial plexus: report of three cases and review of the literature.
  • Tumor involvement of the brachial plexus is uncommon.
  • The most common intrinsic neoplasms involving the brachial plexus are benign neurilemmomas and neurofibromas that are usually associated with neurofibromatosis-1 (NF-1).
  • Malignant peripheral nerve-sheath tumors (MPNST) are rare at this site, arising spontaneously or in the context of NF-1.
  • This presentation discusses the clinical presentation, pathology, and management of these tumors, which usually occur in young adults.
  • MPNST are intermediate or high-grade sarcomas with a high risk of local and distant spread.
  • Approximately 50% of MPNST arise in patients with NF-1, and therefore these patients should be thoroughly investigated for any new symptoms or masses.
  • MPNST of the brachial plexus should be treated with an adequate wide local excision, with adjuvant high-dose radiotherapy pre- or postoperatively.
  • The role of chemotherapy in the treatment of MPNST is not clearly defined, but it may have some benefit in salvaging treatment failures.
  • [MeSH-major] Brachial Plexus. Nerve Sheath Neoplasms / pathology. Nerve Sheath Neoplasms / surgery

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  • (PMID = 16538633.001).
  • [ISSN] 0738-1085
  • [Journal-full-title] Microsurgery
  • [ISO-abbreviation] Microsurgery
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 24
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17. Morgan JA, George S, Desai J, St Amand M, Horton D, Wilkins E, Manola J, Demetri GD: Phase II study of gemcitabine/vinorelbine (GV) as first or second line chemotherapy in patients with metastatic soft tissue sarcoma (STS). J Clin Oncol; 2004 Jul 15;22(14_suppl):9009

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Phase II study of gemcitabine/vinorelbine (GV) as first or second line chemotherapy in patients with metastatic soft tissue sarcoma (STS).
  • : 9009 Background: Both single agent gemcitabine and vinorelbine have efficacy for treatment of STS.
  • Combination GV is active therapy for metastatic carcinoma, with acceptable toxicity.
  • This single institution phase II trial has been undertaken to determine response rates and toxicity of combination GV for treatment of metastatic STS.
  • Gemcitabine at 800mg/m2 was infused over 90 minutes on days 1 and 8 of a 21 day cycle, following vinorelbine at 25mg/m2.
  • Pts with grade 3 or 4 toxicity were subsequently treated at the same doses on days 1 and 15 of a 28 day cycle.
  • Histology was uterine or extremity leiomyosarcoma (LMS) in 9, high grade pleomorphic sarcoma in 2, and 1 each with carcinosarcoma, pleomorphic liposarcoma, malignant peripheral nerve sheath tumor (MPNST), desmoplastic small round cell tumor, rhabdomyosarcoma, and small round cell sarcoma.
  • No treatment related deaths have occurred.
  • Of the remaining 9, 4 have been treatment related, including cough, nausea, vomiting, and SGPT elevation.
  • There have been two confirmed PRs, one high grade uterine LMS progressing after single agent doxorubicin, and one small round cell tumor recurring within 6 months of completion of a 5 drug Ewing's regimen.
  • One metastatic MPNST has exhibited stable disease for greater than 4 months.
  • Median time to progression has been 4.2 months and median survival 6.25 months.
  • CONCLUSIONS: GV appears to be a well-tolerated and potentially effective regimen for first or second line treatment of metastatic STS.

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  • (PMID = 28013692.001).
  • [ISSN] 1527-7755
  • [Journal-full-title] Journal of clinical oncology : official journal of the American Society of Clinical Oncology
  • [ISO-abbreviation] J. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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18. Nickenig C, Buecklein V, Lindner LH, Abdel-Rahman S, Kuhlencordt M, Hiddemann W, Issels RD: Ifosfamide, carboplatin, and etoposide (ICE) in combination with regional hyperthermia (RHT) in chemotherapy-pretreated nonresponders with locally advanced high-risk soft tissue sarcoma (HR-STS). J Clin Oncol; 2009 May 20;27(15_suppl):10581

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Ifosfamide, carboplatin, and etoposide (ICE) in combination with regional hyperthermia (RHT) in chemotherapy-pretreated nonresponders with locally advanced high-risk soft tissue sarcoma (HR-STS).
  • : 10581 Background: Regional hyperthermia (RHT) improves outcome in combination with neoadjuvant chemotherapy as first-line therapy in locally advanced HR-STS (Issels et al., Abstract 10009, ASCO 2007).
  • Efficacy of ICE combined with RHT as second-line treatment strategy in pts with locally advanced HR-STS pretreated with anthracycline-based chemotherapy ± RHT was evaluated.
  • METHODS: Between 9/97 and 6/08, 49 pts were treated with ICE + RHT (median age: 51 years, range: 21-74 years), with high-grade (G2 24 pts; G3 25 pts) STS histology (20 Lipo-Sa; 6 Leiomyo-Sa, 6 MPNST, 5 NOS, 2 DSRCT, 10 others).
  • As first-line therapy 35 pts had received chemotherapy combined with RHT and 14 pts without RHT.
  • Hematological toxicity grade III (11 pts)/ IV (23 pts) occurred in 34 pts (69 %), 3 pts (6 %) suffered from therapy-related deaths due to infection during cytopenia (2 pts) or postsurgery-related complications (1 pt).
  • OR rates after initial chemotherapy with or without RHT were 17 % (4 PR of 24 pts) and 45% (1 CR + 4 PR of 11 pts), respectively (p=0.13).
  • CONCLUSIONS: Second-line ICE combined with RHT is feasible and effective in pts with locally advanced HR-STS non-responding to first-line anthracycline-based chemotherapy with or without RHT. (Supported by Deutsche Krebshilfe and HelmholtzZentrum münchen - German Research Center for Environmental Health) No significant financial relationships to disclose.

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  • (PMID = 27963873.001).
  • [ISSN] 1527-7755
  • [Journal-full-title] Journal of clinical oncology : official journal of the American Society of Clinical Oncology
  • [ISO-abbreviation] J. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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19. Kawai A, Chuman H, Makimoto A, Ito Y, Yamaguchi U, Morimoto Y, Beppu Y: Ifosfamide - etoposide chemotherapy in patients with advanced adult soft tissue sarcomas. J Clin Oncol; 2004 Jul 15;22(14_suppl):9062

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Ifosfamide - etoposide chemotherapy in patients with advanced adult soft tissue sarcomas.
  • : 9062 Background: Doxorubicin and ifosfamide are considered to be the two most active agents in the treatment of adult soft tissue sarcoma (STS).
  • There are several conflicting data about the effect of ifosfamide - etoposide combination chemotherapy in adult patients with STS.
  • At least 4 weeks interval was present since previous chemotherapy.
  • The response was evaluated after at least two chemotherapy courses.
  • Histological diagnoses comprised 6 synovial sarcoma, 5 MFH, 5 MPNST, 2 liposarcoma, 2 leiomyosarcoma, and 2 others.
  • Evaluation sites were metastatic disease in 13, local recurrence in 5 and inoperable primary tumor in 4.
  • Of 8 patients who had received previous anthracycline containing chemotherapy, two responded.
  • Two of five patients with MPNST and MFH had objective tumor regression.
  • Treatment was well tolerated except for neutropenic fever that occurred in 10% of the courses.

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  • (PMID = 28014085.001).
  • [ISSN] 1527-7755
  • [Journal-full-title] Journal of clinical oncology : official journal of the American Society of Clinical Oncology
  • [ISO-abbreviation] J. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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20. Cappellano AM, Grande MT, Oliveira SN, Zambaldi LJ, Araujo M, Freitas FA, Cardinalli IA, Brandalise SR, Aguiar SS: A novel translocation (x;4)(p22.1;q32) in a child with malignant Schwannoma. J Clin Oncol; 2004 Jul 15;22(14_suppl):8572

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] A novel translocation (x;4)(p22.1;q32) in a child with malignant Schwannoma.
  • : 8572 Background: Malignant Schwannoma is a rare entity that represents 2% of the peripheral nerve sheath tumors.
  • A novel unreported translocation t(X;4)(p22.1;q32) in a 6 years old boy with malignant paravertebral Schwannoma, without prior exposure to radiotherapy or chemotherapy was diagnosed and demonstrated.
  • METHODS: Fresh tumor was minced and digested with Trypsin-EDTA 1:250 for 15 minutes at 37oC.
  • When mitotic activity was identified the cells were harvested and also the tumor within 11 days.
  • CONCLUSION: Cloning and characterization of the breakpoint region of this tumor may contribute to the discovery of new genes that are important in this rare disease.

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  • (PMID = 28013856.001).
  • [ISSN] 1527-7755
  • [Journal-full-title] Journal of clinical oncology : official journal of the American Society of Clinical Oncology
  • [ISO-abbreviation] J. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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21. Amin MU, Shafique M: Isolated malignant peripheral nerve sheath tumor of retroperitoneum. J Coll Physicians Surg Pak; 2007 Apr;17(4):226-7

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Isolated malignant peripheral nerve sheath tumor of retroperitoneum.
  • A case of an isolated Malignant Peripheral Nerve Sheath Tumor (MPNST) of the retroperitoneum without neurofibromatosis is presented.
  • The tumor was located deep in the retroperitoneum with metastasis to the ribs.
  • Patient was further managed with radiotherapy and chemotherapy.
  • MPNST in such a location is very unusual.

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  • (PMID = 17462183.001).
  • [ISSN] 1022-386X
  • [Journal-full-title] Journal of the College of Physicians and Surgeons--Pakistan : JCPSP
  • [ISO-abbreviation] J Coll Physicians Surg Pak
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Pakistan
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22. Gachiani J, Kim D, Nelson A, Kline D: Surgical management of malignant peripheral nerve sheath tumors. Neurosurg Focus; 2007;22(6):E13

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Surgical management of malignant peripheral nerve sheath tumors.
  • OBJECT: The aim of this study was to describe the presentation of patients harboring soft tissue sarcomas involving the nerves, most of which were malignant peripheral nerve sheath tumors (MPNSTs), and provide an algorithm for their treatment.
  • METHODS: The authors retrospectively analyzed data on 43 surgically treated soft tissue sarcomas involving the nerves, 34 (79%) of which were MPNSTs.
  • Tumor classifications are presented, together with patient numbers, locations of MPNSTs, surgical techniques, and adjunctive treatments.
  • RESULTS: The 34 MPNSTs were surgically treated during a period of 40 years.
  • Most of these lesions (19 MPNSTs [56%]) were located in the brachial plexus, whereas the rest were located on other major nerves.
  • Neurofibromatosis Type 1-associated tumors (12 lesions) represented 35% of the total number of MPNSTs.
  • CONCLUSIONS: Malignant peripheral nerve sheath tumors are rare.
  • The role of chemotherapy is still being defined.
  • [MeSH-major] Nerve Sheath Neoplasms / diagnosis. Nerve Sheath Neoplasms / surgery
  • [MeSH-minor] Adolescent. Adult. Aged. Aged, 80 and over. Antineoplastic Agents / therapeutic use. Diagnosis, Differential. Disease Management. Humans. Middle Aged. Retrospective Studies

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  • (PMID = 17613204.001).
  • [ISSN] 1092-0684
  • [Journal-full-title] Neurosurgical focus
  • [ISO-abbreviation] Neurosurg Focus
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents
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23. Rizvi S, Mehboob J, Asghar AH, Mateen A, Raza T, Hameed A: Omental caking: a rare manifestation of malignant peripheral nerve sheath tumour. J Coll Physicians Surg Pak; 2010 Aug;20(8):554-5
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  • [Title] Omental caking: a rare manifestation of malignant peripheral nerve sheath tumour.
  • Malignant peripheral nerve sheath tumour (MPNST) is a very rare tumour with an incidence of one per 100,000 and constitutes between 3 to 10% of all soft tissue sarcomas.
  • Mass responded well to chemotherapy comprising of Ifosfamide and Doxorubicin.
  • [MeSH-major] Nerve Sheath Neoplasms / pathology. Nerve Sheath Neoplasms / radiography. Omentum. Peritoneal Neoplasms / pathology. Peritoneal Neoplasms / radiography. Tomography, X-Ray Computed

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  • (PMID = 20688026.001).
  • [ISSN] 1022-386X
  • [Journal-full-title] Journal of the College of Physicians and Surgeons--Pakistan : JCPSP
  • [ISO-abbreviation] J Coll Physicians Surg Pak
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Pakistan
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24. Carli M, Ferrari A, Mattke A, Zanetti I, Casanova M, Bisogno G, Cecchetto G, Alaggio R, De Sio L, Koscielniak E, Sotti G, Treuner J: Pediatric malignant peripheral nerve sheath tumor: the Italian and German soft tissue sarcoma cooperative group. J Clin Oncol; 2005 Nov 20;23(33):8422-30
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  • [Title] Pediatric malignant peripheral nerve sheath tumor: the Italian and German soft tissue sarcoma cooperative group.
  • PURPOSE: To assess the value of chemotherapy and radiotherapy in children with malignant peripheral nerve sheath tumors (MPNSTs) and to identify risk factors associated with outcome.
  • Seventeen percent of patients had neurofibromatosis type 1 (NF1).
  • Chemotherapy was administered to 74% of patients; radiotherapy was administered to 38% of patients.
  • RESULTS: With a median follow-up of 7 years, 5-year overall survival (OS) and progression-free survival (PFS) were 51% and 37%, respectively.
  • Univariate analysis identified IRS groups, size, invasiveness, primary site, age, and presence of NF1 as prognostic factors; multivariate analysis identified absence of NF1, tumor invasiveness T1, IRS groups I to II and extremity of primary site as independent favorable factors for OS.
  • The overall response rate to primary chemotherapy, including minor responses, in group III patients was 45%.
  • CONCLUSION: MPNST is an aggressive tumor for which complete surgical resection is the mainstay of successful treatment.
  • Postoperative radiotherapy may have a role in improving local control in patients with minimal residual tumor.
  • The reported responses to primary chemotherapy suggest that it may be effective in patients with tumor considered unresectable at diagnosis.
  • [MeSH-major] Nerve Sheath Neoplasms / drug therapy. Nerve Sheath Neoplasms / radiotherapy
  • [MeSH-minor] Adolescent. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Child. Child, Preschool. Combined Modality Therapy. Disease-Free Survival. Female. Germany / epidemiology. Humans. Infant. Infant, Newborn. Italy / epidemiology. Male. Multivariate Analysis. Proportional Hazards Models. Risk Factors. Survival Rate. Treatment Outcome

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  • [ErratumIn] J Clin Oncol. 2006 Feb 1;24(4):724. Koscielniak, Eura [corrected to Koscielniak, Ewa]
  • (PMID = 16293873.001).
  • [ISSN] 0732-183X
  • [Journal-full-title] Journal of clinical oncology : official journal of the American Society of Clinical Oncology
  • [ISO-abbreviation] J. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Clinical Trial; Journal Article; Multicenter Study; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
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25. Jankulovski N, Stankov O, Banev S, Petrovski D, Mickovski A, Mitevski A, Filipovski V, Popov Z: Isolated malignant peripheral nerve sheath tumor of kidney capsule. Prilozi; 2008 Dec;29(2):361-69
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  • [Title] Isolated malignant peripheral nerve sheath tumor of kidney capsule.
  • The occurrence of an isolated malignant peripheral nerve sheath tumor (MPNST) of the kidney capsule is extremely rare and its presence may only be expressed by an insidious onset of non-specific and misleading symptoms with a predominance of lower back pain.
  • A computer tomography (CT) scan (as the imaging procedure of choice) will demonstrate the tumor location and its relation to the surrounding structures.
  • Tumor excision in toto is considered the treatment of choice, but it can be hazardous, especially if the tumor is adhering to the surrounding structures.
  • If malignancy can safely be excluded, a laparoscopic excision should be considered as an alternative treatment as recurrence is unlikely.
  • Definition of the originating nerve might not always be possible, and a minor degree of neurological impairment has therefore to be anticipated.
  • A case of an isolated MPNST of the kidney capsule without neurofibromatosis is presented.
  • The tumor was located in the fatty and fibrous capsule.
  • The patient was further managed with radiotherapy and chemotherapy.
  • An MPNST in such a location is very unusual.

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  • (PMID = 19259060.001).
  • [ISSN] 0351-3254
  • [Journal-full-title] Prilozi
  • [ISO-abbreviation] Prilozi
  • [Language] ENG
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Germany
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26. Ishihara S, Honda Y, Asato T, Nonaka M, Nakagawa S, Hirashima K, Hayashi N, Baba H, Iyama K: Interdigitating dendritic cell sarcoma of the ileum recurred in multiple lymph nodes and duodenum three years after operation without chemotherapy. Pathol Res Pract; 2010 Jul 15;206(7):514-8
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  • [Title] Interdigitating dendritic cell sarcoma of the ileum recurred in multiple lymph nodes and duodenum three years after operation without chemotherapy.
  • Here we describe a case of a 47-year-old man with interdigitating dendritic cell sarcoma (IDCS) in the ileum.
  • The ileal tumor, measuring 2cm, was detected and resected with regional lymphadenectomy.
  • At that time, a pathologic diagnosis of malignant peripheral nerve sheath tumor was made.
  • The patient, who was not treated with chemotherapy, showed no signs of recurrence.
  • Oval to spindle-shaped atypical cells, which resembled ileal tumor cells, infiltrated into the lymph node and duodenum.
  • Based on the histologic and immunohistochemical analysis, the histopathologic diagnosis of IDCS was confirmed.
  • To our knowledge, five cases of IDCS arising in the intestinal tract have been reported to date, and only one case, treated with both surgery and chemotherapy, led to remission.
  • This is the first case that has a comparatively favorable prognosis without chemotherapy after surgery.
  • [MeSH-major] Dendritic Cell Sarcoma, Interdigitating / pathology. Duodenal Neoplasms / pathology. Ileal Neoplasms / pathology. Lymph Nodes / pathology. Neoplasm Recurrence, Local / pathology
  • [MeSH-minor] Antineoplastic Agents / therapeutic use. Biomarkers, Tumor / analysis. Diagnostic Errors. Digestive System Surgical Procedures. Humans. Immunohistochemistry. Male. Middle Aged. Nerve Sheath Neoplasms / pathology

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  • [Copyright] Copyright 2010 Elsevier GmbH. All rights reserved.
  • (PMID = 20399026.001).
  • [ISSN] 1618-0631
  • [Journal-full-title] Pathology, research and practice
  • [ISO-abbreviation] Pathol. Res. Pract.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Biomarkers, Tumor
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27. Amin A, Saifuddin A, Flanagan A, Patterson D, Lehovsky J: Radiotherapy-induced malignant peripheral nerve sheath tumor of the cauda equina. Spine (Phila Pa 1976); 2004 Nov 1;29(21):E506-9
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  • [Title] Radiotherapy-induced malignant peripheral nerve sheath tumor of the cauda equina.
  • OBJECTIVES: To report a rare case of radiation-induced malignant peripheral nerve sheath tumor of the cauda equina 10 years after treatment for testicular seminoma.
  • SUMMARY OF BACKGROUND DATA: Development of malignant peripheral nerve sheath tumor after irradiation is well recognized and often associated with a dismal prognosis.
  • There have been isolated reports of malignant peripheral nerve sheath tumor developing in sites of previous irradiation for testicular seminoma.
  • Ten years previously, he had undergone right radical inguinal orchidectomy and adjuvant para-aortic radiotherapy as treatment for Stage I testicular seminoma.
  • Magnetic resonance imaging demonstrated an inoperable intra- and extradural tumor leading to significant cauda equina compression.
  • CT-guided biopsy revealed a diagnosis of malignant peripheral nerve sheath tumor, most likely due to previous radiotherapy.
  • His clinical condition did not improve, and he underwent a course of palliative chemotherapy.
  • CONCLUSIONS: Postirradiation malignant peripheral nerve sheath tumors are rare and occur in a population at high risk of developing second malignancies.
  • The authors report the fourth case resulting from adjuvant radiotherapy for testicular seminoma, with the present report being the first report of extensive intradural tumor leading to cauda equina syndrome.
  • [MeSH-major] Cauda Equina. Neoplasms, Radiation-Induced / etiology. Nerve Sheath Neoplasms / etiology. Peripheral Nervous System Neoplasms / etiology. Radiotherapy, Adjuvant / adverse effects
  • [MeSH-minor] Accidental Falls. Adult. Antineoplastic Agents / therapeutic use. Cervical Intraepithelial Neoplasia. Humans. Male. Orchiectomy. Palliative Care. Polyradiculopathy / etiology. Postoperative Complications / etiology. Retrospective Studies. Sciatica / complications. Seminoma / radiotherapy. Seminoma / surgery. Testicular Neoplasms / radiotherapy. Testicular Neoplasms / surgery

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  • (PMID = 15507791.001).
  • [ISSN] 1528-1159
  • [Journal-full-title] Spine
  • [ISO-abbreviation] Spine
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents
  • [Number-of-references] 21
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28. Manger T, Pross M, Haeckel C, Lippert H: Malignant peripheral nerve sheath tumor of the esophagus. Dig Surg; 2000;17(6):627-631
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Malignant peripheral nerve sheath tumor of the esophagus.
  • BACKGROUND: Sarcomas of the esophagus are rare representing 0.1-1.5% of all esophageal tumors.
  • We report a case of malignant peripheral nerve sheath tumor (MPNST) of the esophagus in a 60-year-old woman.
  • METHODS: The diagnosis was made preoperatively on endoscopic biopsy and confirmed after tumor resection by immunohistochemistry as well as electron microscopy.
  • RESULTS: Our therapeutic concept for the first case of a high-grade MPNST (malignant schwannoma) of the esophagus resulted in a recurrence-free interval of 4 years.
  • CONCLUSION: The therapy of choice was abdominal-thoracic en bloc esophagectomy with tumor-free resection margins and esophageal reconstruction with the stomach.
  • After R0-resection we found no indication for adjuvant radio- and/or chemotherapy.
  • [MeSH-major] Esophageal Neoplasms / surgery. Esophagectomy. Nerve Sheath Neoplasms / surgery

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  • [Copyright] Copyright 2000 S. Karger AG, Basel.
  • (PMID = 11155010.001).
  • [ISSN] 0253-4886
  • [Journal-full-title] Digestive surgery
  • [ISO-abbreviation] Dig Surg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Switzerland
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29. Moon SJ, Lee JK, Seo BR, Kim JH, Kim SH, Lee KH, Lee MC: An intraosseous malignant peripheral nerve sheath tumor of the cervical spine: a case report and review of the literature. Spine (Phila Pa 1976); 2008 Sep 1;33(19):E712-6
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  • [Title] An intraosseous malignant peripheral nerve sheath tumor of the cervical spine: a case report and review of the literature.
  • OBJECTIVES: To report a rare case of intraosseous malignant peripheral nerve sheath tumors (MPNST), and review the pertinent medical literature.
  • SUMMARY OF BACKGROUND DATA: The spinal MPNST that develops from spinal nerve roots and secondary bony erosion is well-known entity.
  • However, primary intraosseous MPNSTs of the spine are extremely rare.
  • METHODS: A 41-year-old male presented with a 1-month history of radiating pain to his right shoulder and arm.
  • Complete excision of the tumor and posterior stabilization were performed through a posterior approach.
  • The tumor was noted to originate from the posterior element of C7.
  • RESULTS: The histopathology was diagnostic for a MPNST.
  • Adjuvant chemotherapy was administered after surgery.
  • CONCLUSION: We report an intraosseous MPNST of the cervical spine.
  • Complete surgical excision and adjuvant chemotherapy resulted in a good functional outcome.
  • MPNST should be added to the differential diagnosis of primary bone tumors causing spinal cord compression.
  • [MeSH-major] Cervical Vertebrae / pathology. Nerve Sheath Neoplasms / pathology. Spinal Neoplasms / pathology
  • [MeSH-minor] Adult. Antigens, CD / metabolism. Antigens, CD34 / metabolism. Biomarkers, Tumor / metabolism. Cell Adhesion Molecules / metabolism. Chemotherapy, Adjuvant. Combined Modality Therapy. Disease-Free Survival. Humans. Ki-67 Antigen / metabolism. Magnetic Resonance Imaging. Male. S100 Proteins / metabolism. Spinal Cord Compression / etiology. Spinal Cord Compression / pathology. Vimentin / metabolism

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  • (PMID = 18758353.001).
  • [ISSN] 1528-1159
  • [Journal-full-title] Spine
  • [ISO-abbreviation] Spine
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antigens, CD; 0 / Antigens, CD34; 0 / Biomarkers, Tumor; 0 / CD99 protein, human; 0 / Cell Adhesion Molecules; 0 / Ki-67 Antigen; 0 / S100 Proteins; 0 / Vimentin
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30. Kasper B, Lehnert T, Bernd L, Mechtersheimer G, Goldschmidt H, Ho AD, Egerer G: High-dose chemotherapy with autologous peripheral blood stem cell transplantation for bone and soft-tissue sarcomas. Bone Marrow Transplant; 2004 Jul;34(1):37-41
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  • [Title] High-dose chemotherapy with autologous peripheral blood stem cell transplantation for bone and soft-tissue sarcomas.
  • The role of high-dose chemotherapy (HDCT) with PBSCT in the treatment of bone and soft-tissue sarcomas is not established.
  • In total, 27 patients (15 female, median age at TPL 30.6 years (range: 13-59)) were analyzed (Ewing sarcoma family n=8, osteosarcoma n=6, MPNST (malignant peripheral nerve sheath tumor) n=4, synovial sarcoma n=3, liposarcoma n=2, leiomyosarcoma n=2, rhabdomyosarcoma n=1, meningosarcoma n=1).
  • Following chemotherapy and surgery complete remission (CR) (n=9), partial remission (PR) (n=10), stable disease (SD) (n=2) and progressive disease (PD) (n=6) were reached prior HDCT.
  • Although the role of HDCT in the treatment of sarcomas is not defined, a subgroup of patients who achieved CR before HDCT could benefit from this therapy.
  • [MeSH-major] Antineoplastic Agents / therapeutic use. Bone Neoplasms / therapy. Peripheral Blood Stem Cell Transplantation / methods. Sarcoma / therapy
  • [MeSH-minor] Adolescent. Adult. Female. Humans. Male. Middle Aged. Remission Induction. Retrospective Studies. Survival Analysis. Transplantation, Autologous. Treatment Outcome


31. Lambrou NC, Mirhashemi R, Wolfson A, Thesiger P, Penalver M: Malignant peripheral nerve sheath tumor of the vulva: a multimodal treatment approach. Gynecol Oncol; 2002 May;85(2):365-71
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Malignant peripheral nerve sheath tumor of the vulva: a multimodal treatment approach.
  • BACKGROUND: Malignant peripheral nerve sheath tumors (MPNSTs) are rare in the gynecological population and have a high risk for local and distant failures.
  • Multimodal management of a patient with MPNST of the vulva and review of the literature are outlined.
  • CASE: A 34-year-old woman presented with a complaint of a rapidly increasing pelvic mass, pain, and difficulty ambulating.
  • A disfiguring 20 x 20-cm vulvar mass was identified and a recurrent MPNST diagnosed.
  • Therapy included external-beam radiation, anterior pelvic exenteration with pelvic reconstruction, and adjuvant chemotherapy without complication.
  • CONCLUSION: It is recommended that for malignant peripheral nerve sheath tumors of the vulva, complete surgical resection be performed with adjuvant radiation and chemotherapy in selected cases.
  • [MeSH-major] Nerve Sheath Neoplasms / therapy. Vulvar Neoplasms / therapy
  • [MeSH-minor] Adult. Chemotherapy, Adjuvant. Female. Humans. Neurofibromatosis 1 / complications. Radiotherapy, Adjuvant

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  • [Copyright] (c) 2002 Elsevier Science (USA).
  • (PMID = 11972402.001).
  • [ISSN] 0090-8258
  • [Journal-full-title] Gynecologic oncology
  • [ISO-abbreviation] Gynecol. Oncol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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32. Ploegmakers MJ, Pruszczynski M, De Rooy J, Kusters B, Veth RP: Angiosarcoma with malignant peripheral nerve sheath tumour developing in a patient with klippel-trénaunay-weber syndrome. Sarcoma; 2005;9(3-4):137-40

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Angiosarcoma with malignant peripheral nerve sheath tumour developing in a patient with klippel-trénaunay-weber syndrome.
  • PURPOSE: We discuss the coexistence of Klippel-Trénaunay-Weber syndrome with various malignancies, the possible histogenetic pathways and therapeutic implications.
  • METHODS: Our patient underwent an above-knee amputation for biopsy-proven malignant vascular tumour, first thought to be a composite hemangio-endothelioma and/or angiosarcoma with lung metastases.
  • RESULTS: In the amputated extremity, a vascular malformation was found with tumour showing various components with foci of angiosarcoma adjacent to diffuse neurofibroma and areas with high-grade malignant peripheral nerve sheath tumour.
  • Amputation and palliative chemotherapy were indicated, but he died of pulmonary and cerebral metastases 2 months postoperatively.
  • DISCUSSION: This case describes an angiosarcoma with malignant peripheral nerve sheath tumour developing in a patient with Klippel-Trénaunay-Weber syndrome.

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  • (PMID = 18521421.001).
  • [ISSN] 1357-714X
  • [Journal-full-title] Sarcoma
  • [ISO-abbreviation] Sarcoma
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Egypt
  • [Other-IDs] NLM/ PMC2395629
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33. Ambrosini G, Sambol EB, Carvajal D, Vassilev LT, Singer S, Schwartz GK: Mouse double minute antagonist Nutlin-3a enhances chemotherapy-induced apoptosis in cancer cells with mutant p53 by activating E2F1. Oncogene; 2007 May 24;26(24):3473-81
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Mouse double minute antagonist Nutlin-3a enhances chemotherapy-induced apoptosis in cancer cells with mutant p53 by activating E2F1.
  • MDM2 is a critical negative regulator of the p53 tumor suppressor protein.
  • Recently, small-molecule antagonists of MDM2, the Nutlins, have been developed to inhibit the p53-MDM2 interaction and activate p53 signaling.
  • However, half of human cancers have mutated p53 and they are resistant to Nutlin treatment.
  • Here, we report that treatment of the p53-mutant malignant peripheral nerve sheath (MPNST) and p53-null HCT116 cells with cisplatin (Cis) and Nutlin-3a induced a degree of apoptosis that was significantly greater than either drug alone.
  • Nutlin-3a also increased the cytotoxicity of both carboplatin and doxorubicin in a series of p53-mutant human tumor cell lines.
  • In the human dedifferentiated liposarcoma cell line (LS141) and the p53 wild-type HCT116 cells, Nutlin-3a induced downregulation of E2F1 and this effect appeared to be proteasome dependent.
  • In contrast, in MPNST and HCTp53-/- cells, Nutlin-3a inhibited the binding of E2F1 to MDM2 and induced transcriptional activation of free E2F1 in the presence of Cis-induced DNA damage.
  • Downregulation of E2F1 by small interfering RNA significantly decreased the level of apoptosis induced by Cis and Nutlin-3a treatment.
  • Moreover, expression of a dominant-negative form of E2F1 rescued cells from apoptosis, whereas cells overexpressing wild-type E2F1 showed an increase in cell death.
  • Nutlin-3a therefore may provide a therapeutic benefit in tumors with mutant p53 provided it is combined with chemotherapy.
  • [MeSH-major] Antineoplastic Agents / pharmacology. Apoptosis / drug effects. E2F1 Transcription Factor / metabolism. Imidazoles / pharmacology. Piperazines / pharmacology. Tumor Suppressor Protein p53 / genetics
  • [MeSH-minor] Animals. Carboplatin / pharmacology. Cisplatin / pharmacology. DNA-Binding Proteins / drug effects. DNA-Binding Proteins / metabolism. Doxorubicin / pharmacology. Humans. Mice. Nuclear Proteins / drug effects. Nuclear Proteins / metabolism. Proto-Oncogene Proteins c-bcl-2 / drug effects. Proto-Oncogene Proteins c-bcl-2 / metabolism. Proto-Oncogene Proteins c-mdm2 / metabolism. Transcription, Genetic. Tumor Cells, Cultured. Tumor Suppressor Proteins / drug effects. Tumor Suppressor Proteins / metabolism

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  • (PMID = 17146434.001).
  • [ISSN] 0950-9232
  • [Journal-full-title] Oncogene
  • [ISO-abbreviation] Oncogene
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / DNA-Binding Proteins; 0 / E2F1 Transcription Factor; 0 / E2f1 protein, mouse; 0 / Imidazoles; 0 / Nuclear Proteins; 0 / PMAIP1 protein, human; 0 / Piperazines; 0 / Proto-Oncogene Proteins c-bcl-2; 0 / Tumor Suppressor Protein p53; 0 / Tumor Suppressor Proteins; 0 / nutlin 3; 0 / tumor suppressor protein p73; 80168379AG / Doxorubicin; BG3F62OND5 / Carboplatin; EC 6.3.2.19 / MDM2 protein, human; EC 6.3.2.19 / Proto-Oncogene Proteins c-mdm2; Q20Q21Q62J / Cisplatin
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34. Zou CY, Smith KD, Zhu QS, Liu J, McCutcheon IE, Slopis JM, Meric-Bernstam F, Peng Z, Bornmann WG, Mills GB, Lazar AJ, Pollock RE, Lev D: Dual targeting of AKT and mammalian target of rapamycin: a potential therapeutic approach for malignant peripheral nerve sheath tumor. Mol Cancer Ther; 2009 May;8(5):1157-68
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  • [Title] Dual targeting of AKT and mammalian target of rapamycin: a potential therapeutic approach for malignant peripheral nerve sheath tumor.
  • The mammalian target of rapamycin (mTOR) pathway may constitute a potential target for the treatment of malignant peripheral nerve sheath tumors (MPNST).
  • Consequently, we hypothesized that dual phosphatidylinositol 3-kinase (PI3K)/AKT-mTOR blockade might be applicable for MPNST treatment.
  • Expression of activated mTOR downstream targets (p4EBP1 and pS6RP) and pAKT was evaluated immunohistochemically in a tissue microarray of human MPNSTs (n = 96) and benign neurofibromas (n = 31).
  • Results were analyzed by Wilcoxon rank-sum tests. mTOR and AKT pathways in human MPNST cell lines, and the effects of rapamycin (mTOR inhibitor), LY294002 (dual PI3K/mTOR inhibitor), and PI-103 (potent dual PI3K/AKT-mTOR inhibitor) on pathway activation were evaluated by Western blot.
  • Acridine orange staining/fluorescence-activated cell sorting analysis, electron microscopy, and Western blot evaluated autophagy induction. p4EBP1, pS6Rp, and pAKT levels were found to be significantly higher in MPNST versus neurofibroma (P < 0.05 for all markers).
  • mTOR and AKT pathways were found to be highly activated in MPNST cell lines.
  • MPNST cells were sensitive to rapamycin; however, rapamycin enhanced pAKT and peIF4E expression.
  • PI-103 abrogated MPNST cell growth and induced G(1) cell cycle arrest potentially through repression of cyclin D1.
  • PI-103 did not elicit apoptosis but significantly induced autophagy in MPNST cells.
  • These results suggest further study of combined PI3K/AKT and mTOR inhibition as a novel therapy for patients harboring MPNST.


35. Yone K, Ijiri K, Hayashi K, Yokouchi M, Takenouchi T, Manago K, Nerome Y, Ijichi O, Ikarimoto N, Komiya S: Primary malignant peripheral nerve sheath tumor of the cauda equina in a child case report. Spinal Cord; 2004 Mar;42(3):199-203
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Primary malignant peripheral nerve sheath tumor of the cauda equina in a child case report.
  • STUDY DESIGN: A case report of primary malignant peripheral nerve sheath tumor (MPNST) of the cauda equina in a child is presented, and the literature is reviewed.
  • OBJECTIVE: To discuss the problems involved in the treatment of primary intradural MPNSTs.
  • MRI revealed an intradural tumor at L3-L5 level.
  • Following laminectomy of L3, L4 and L5, the tumor was removed en bloc.
  • Based on pathological and immunohistological findings, the tumor was diagnosed as an MPNST.
  • RESULTS: Although adjuvant chemotherapy was administered local recurrence and cerebral and spinal metastases of the tumor were found 6 months after the operation.
  • Following additional incomplete removal of the recurrent tumor, radiation therapy was administered.
  • Although recurrent and metastatic tumors disappeared or diminished in size by radiation, tumors increased in size thereafter, despite additional adjuvant chemotherapy.
  • CONCLUSIONS: Reported clinical outcomes for patients with primary intradural MPNST are very poor.
  • Although no gold standard for the treatment of tumors has been established yet, surgical removal of tumors combined with postoperative high-dose radiation may be recommended.
  • [MeSH-major] Cauda Equina / pathology. Nerve Sheath Neoplasms / pathology. Nerve Sheath Neoplasms / secondary. Peripheral Nervous System Neoplasms / pathology

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  • (PMID = 15001982.001).
  • [ISSN] 1362-4393
  • [Journal-full-title] Spinal cord
  • [ISO-abbreviation] Spinal Cord
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
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36. Friedrich RE, Hartmann M, Mautner VF: Malignant peripheral nerve sheath tumors (MPNST) in NF1-affected children. Anticancer Res; 2007 Jul-Aug;27(4A):1957-60

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Malignant peripheral nerve sheath tumors (MPNST) in NF1-affected children.
  • BACKGROUND: Malignant peripheral nerve sheath tumors (MPNST) constitute a heterogeneous group of malignant tumors that probably arise from cells of the peripheral nerve sheath.
  • Association of MPNST with neurofibromatosis type 1 (NF1) is frequently reported.
  • MPNST contribute significantly to the reduced life-span of NF1-patients.
  • At present there are only sparse data on MPNST in NF1-children.
  • The aim of this study was to determine the outcome of children affected with NF1 who developed an MPNST.
  • MATERIALS AND METHODS: Over the period of 1985 to 2005, we followed 52 NF1 patients with MPNST at our outpatient department.
  • RESULTS: Out of this cohort, 8 patients with MPNST were aged 1 to 17 years at the time of MPNST diagnosis (mean age: 12 years; 5 girls and 3 boys).
  • We noticed the following characteristics: MPNST arose from plexiform neurofibromas (PNF) with invasive or displacing growth pattern on MRI.
  • Many patients reported pain and neurological deficits at the time of presentation.
  • Diagnosis of MPNST in this age group took longer compared to adults.
  • This cohort did not show longer survival periods than adults with MPNST.
  • Adjunctive treatment with chemotherapy or radiation had no lasting effect.
  • The overall survival time of this small cohort was 30.5 months.
  • Those children who died showed a median survival time after diagnosis of 20 months.
  • The longest survival of 112 months was achieved for a girl who presented with MPNST of the distal upper arm and underwent amputation.
  • The NF1 mutation analysis in the MPNST pediatric age group revealed the same mutational spectrum as the adult group.
  • CONCLUSION: Our data reveal MPNST in children with NF1.
  • Children cannot verbalize physical alterations adequately; therefore the correct diagnosis might be hampered in these patients.
  • [MeSH-major] Nerve Sheath Neoplasms / complications. Nerve Sheath Neoplasms / diagnosis. Neurofibromatosis 1 / complications

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  • (PMID = 17649804.001).
  • [ISSN] 0250-7005
  • [Journal-full-title] Anticancer research
  • [ISO-abbreviation] Anticancer Res.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Greece
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37. Minovi A, Basten O, Hunter B, Draf W, Bockmühl U: Malignant peripheral nerve sheath tumors of the head and neck: management of 10 cases and literature review. Head Neck; 2007 May;29(5):439-45
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  • [Title] Malignant peripheral nerve sheath tumors of the head and neck: management of 10 cases and literature review.
  • BACKGROUND: This study analyzes the management and outcomes of a series of 10 malignant peripheral nerve sheath tumors (MPNST) of the head and neck.
  • METHODS: From 1984 to 2004, 10 patients underwent surgical treatment of a MPNST.
  • RESULTS: Eight tumors were located at the lateral skull base; 2 involved the vagus nerve in isolation.
  • Seventy percent of the tumors could be resected completely.
  • Negative prognostic indicators were advanced tumor stage, early recurrence, and presumably also the presence of von Recklinghausen's disease.
  • CONCLUSIONS: Although rare, MPNST is one of the most aggressive tumors in the head and neck area.
  • Complete tumor removal is the mainstay of treatment and most important prognostic factor of MPNST.
  • The role of adjuvant chemotherapy remains controversial.
  • [MeSH-major] Head and Neck Neoplasms / mortality. Head and Neck Neoplasms / therapy. Neoplasm Recurrence, Local / mortality. Nerve Sheath Neoplasms / mortality. Nerve Sheath Neoplasms / therapy
  • [MeSH-minor] Adolescent. Adult. Aged. Chemotherapy, Adjuvant. Female. Humans. Male. Middle Aged. Prognosis. Radiotherapy, Adjuvant. Retrospective Studies. Survival Rate

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  • [Copyright] (c) 2006 Wiley Periodicals, Inc.
  • (PMID = 17163467.001).
  • [ISSN] 1043-3074
  • [Journal-full-title] Head & neck
  • [ISO-abbreviation] Head Neck
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 34
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38. Adamson DC, Cummings TJ, Friedman AH: Malignant peripheral nerve sheath tumor of the spine after radiation therapy for Hodgkin's lymphoma. Clin Neuropathol; 2004 Sep-Oct;23(5):245-55
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  • [Title] Malignant peripheral nerve sheath tumor of the spine after radiation therapy for Hodgkin's lymphoma.
  • We report the development of a malignant peripheral nerve sheath tumor (MPNST) in 2 patients after irradiation for Hodgkin's lymphoma.
  • Clinicians should be aware of this uncommon, but important fatal complication of radiation therapy.
  • He presented to our neurosurgery service with a left C6 radiculopathy 6 years later.
  • She did well with extensive radiotherapy until 5 years later when she developed severe right arm and chest pain secondary to recurrent lymphoma.
  • After aggressive radio- and chemotherapy, she presented to the neurosurgery service with a right Horner's syndrome, right C6 radiculopathy, and weakness of her right triceps and wrist extensors.
  • Both patients obtained magnetic resonance imaging revealing intradural extramedullary cervical nerve root associated mass lesions.
  • Two years after radiation therapy for his Hodgkin's lymphoma, the first patient underwent a C6 laminectomy at an outside institution for resection of a benign neurofibroma.
  • Four years later, he underwent a posterior C5-7 laminectomy with lateral mass plate fusion and partial excision of a recurrent mass diagnosed as a MPNST.
  • The second patient underwent a C5-6 hemilaminectomy and partial resection of a tumor also pathologically consistent with MPNST.
  • We present 2 case reports of patients who developed neurofibrosarcomatous tumors with malignant transformation after undergoing radiation therapy for Hodgkin's lymphoma.
  • Despite prompt surgical resection, these tumors exhibited aggressive behavior.
  • Numerous cases of soft tissue tumors have been described to arise in areas of prior radiation therapy; however, there have been rare reports of de novo MPNST after radiation therapy, especially in the setting of Hodgkin's lymphoma.
  • Postirradiation MPNST should be considered in the differential diagnosis of a painful, enlarging mass in a previously irradiated area.
  • [MeSH-major] Hodgkin Disease / radiotherapy. Lymphatic Irradiation / adverse effects. Neoplasms, Radiation-Induced / pathology. Nerve Sheath Neoplasms / etiology. Spinal Neoplasms / etiology
  • [MeSH-minor] Adult. Diagnosis, Differential. Fatal Outcome. Humans. Magnetic Resonance Imaging. Male


39. Johansson G, Mahller YY, Collins MH, Kim MO, Nobukuni T, Perentesis J, Cripe TP, Lane HA, Kozma SC, Thomas G, Ratner N: Effective in vivo targeting of the mammalian target of rapamycin pathway in malignant peripheral nerve sheath tumors. Mol Cancer Ther; 2008 May;7(5):1237-45
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  • [Title] Effective in vivo targeting of the mammalian target of rapamycin pathway in malignant peripheral nerve sheath tumors.
  • Malignant peripheral nerve sheath tumors (MPNST) are chemoresistant sarcomas with poor 5-year survival that arise in patients with neurofibromatosis type 1 (NF1) or sporadically.
  • We tested three drugs for single and combinatorial effects on collected MPNST cell lines and in MPNST xenografts.
  • The mammalian target of rapamycin complex 1 inhibitor RAD001 (Everolimus) decreased growth 19% to 60% after 4 days of treatment in NF1 and sporadic-derived MPNST cell lines.
  • Treatment of subcutaneous sporadic MPNST cell xenografts with RAD001 significantly, but transiently, delayed tumor growth, and decreased vessel permeability within xenografts.
  • RAD001 combined with the epidermal growth factor receptor tyrosine kinase inhibitor erlotinib caused additional inhibitory effects on growth and apoptosis in vitro, and a small but significant additional inhibitory effect on MPNST growth in vivo that were larger than the effects of RAD001 with doxorubicin.
  • The results support the consideration of RAD001 therapy in NF1 patient and sporadic MPNST.
  • The preclinical tests described allow rapid screening strata for drugs that block MPNST growth, prior to tests in more complex models, and should be useful to identify drugs that synergize with RAD001.

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  • [Cites] Biochem Biophys Res Commun. 2006 Jun 9;344(3):869-80 [16631613.001]
  • [Cites] Curr Opin Oncol. 2006 Jul;18(4):360-2 [16721131.001]
  • [Cites] Oncogene. 2006 Oct 16;25(48):6416-22 [17041626.001]
  • [Cites] Genes Dev. 2006 Oct 15;20(20):2820-32 [17043309.001]
  • [Cites] Int Immunopharmacol. 2006 Dec 20;6(13-14):2018-22 [17161356.001]
  • [Cites] Mol Ther. 2007 Feb;15(2):279-86 [17235305.001]
  • [Cites] J Pharmacokinet Pharmacodyn. 2007 Jun;34(3):373-400 [17431753.001]
  • [Cites] Curr Biol. 2008 Jan 8;18(1):56-62 [18164202.001]
  • [Cites] Cancer Res. 2008 Feb 15;68(4):1170-9 [18281493.001]
  • [Cites] J Clin Invest. 2000 May;105(9):1233-41 [10791998.001]
  • [Cites] J Biol Chem. 2000 May 5;275(18):13571-9 [10788473.001]
  • [Cites] Cancer Res. 2006 Feb 1;66(3):1500-8 [16452206.001]
  • [Cites] Cancer Res. 2006 Mar 1;66(5):2584-91 [16510576.001]
  • [Cites] Pediatr Blood Cancer. 2006 Jun;46(7):745-54 [16124003.001]
  • [Cites] Am J Hum Genet. 2001 May;68(5):1110-8 [11283797.001]
  • [Cites] J Clin Oncol. 2001 Jul 1;19(13):3267-79 [11432895.001]
  • [Cites] Nat Med. 2002 Feb;8(2):128-35 [11821896.001]
  • [Cites] Cancer Res. 2002 Mar 1;62(5):1573-7 [11894862.001]
  • [Cites] J Med Genet. 2002 May;39(5):311-4 [12011145.001]
  • [Cites] J Neuropathol Exp Neurol. 2002 Aug;61(8):702-9 [12152785.001]
  • [Cites] Genes Dev. 2002 Oct 15;16(20):2627-32 [12381661.001]
  • [Cites] Glia. 2003 Jun;42(4):350-8 [12730955.001]
  • [Cites] Trends Biochem Sci. 2004 Jan;29(1):32-8 [14729330.001]
  • [Cites] Nat Med. 2004 Jun;10(6):594-601 [15156201.001]
  • [Cites] Genes Dev. 2004 Aug 15;18(16):1926-45 [15314020.001]
  • [Cites] Nature. 2004 Sep 9;431(7005):200-5 [15306821.001]
  • [Cites] Brain Pathol. 2004 Jul;14(3):297-303 [15446585.001]
  • [Cites] Clin Exp Immunol. 1977 Apr;28(1):1-18 [324671.001]
  • [Cites] Cell. 1992 Apr 17;69(2):265-73 [1568246.001]
  • [Cites] Nature. 1992 Apr 23;356(6371):713-5 [1570015.001]
  • [Cites] Nature. 1993 May 13;363(6425):170-2 [8483501.001]
  • [Cites] Cancer Res. 1995 May 1;55(9):1982-8 [7728769.001]
  • [Cites] Oncogene. 1996 Feb 1;12(3):507-13 [8637706.001]
  • [Cites] Virchows Arch. 1998 Nov;433(5):435-41 [9849858.001]
  • [Cites] Cancer Cell. 2005 Jan;7(1):65-75 [15652750.001]
  • [Cites] Mol Cancer Ther. 2005 Jan;4(1):101-12 [15657358.001]
  • [Cites] Science. 2005 Feb 18;307(5712):1098-101 [15718470.001]
  • [Cites] Cell. 2005 Mar 25;120(6):747-59 [15797377.001]
  • [Cites] Cell. 2005 Apr 22;121(2):179-93 [15851026.001]
  • [Cites] Curr Biol. 2005 Apr 26;15(8):702-13 [15854902.001]
  • [Cites] Proc Natl Acad Sci U S A. 2005 Jun 14;102(24):8573-8 [15937108.001]
  • [Cites] J Clin Oncol. 2005 Nov 20;23(33):8422-30 [16293873.001]
  • [Cites] Clin Cancer Res. 2005 Dec 15;11(24 Pt 1):8782-8 [16361566.001]
  • [Cites] J Pharmacol Exp Ther. 2006 Jan;316(1):456-65 [16239399.001]
  • [Cites] Proc Natl Acad Sci U S A. 2006 Jan 31;103(5):1475-9 [16432179.001]
  • (PMID = 18483311.001).
  • [ISSN] 1535-7163
  • [Journal-full-title] Molecular cancer therapeutics
  • [ISO-abbreviation] Mol. Cancer Ther.
  • [Language] ENG
  • [Grant] United States / NINDS NIH HHS / NS / NS028840-09; United States / NINDS NIH HHS / NS / R01 NS028840; United States / NINDS NIH HHS / NS / R01 NS028840-09; United States / NINDS NIH HHS / NS / R01 NS28840-17
  • [Publication-type] Comparative Study; Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Immunosuppressive Agents; 0 / Quinazolines; 9HW64Q8G6G / Everolimus; DA87705X9K / Erlotinib Hydrochloride; EC 2.7.- / Protein Kinases; EC 2.7.1.1 / MTOR protein, human; EC 2.7.1.1 / TOR Serine-Threonine Kinases; EC 2.7.1.1 / mTOR protein, mouse; EC 2.7.11.1 / Ribosomal Protein S6 Kinases, 70-kDa; EC 2.7.11.1 / ribosomal protein S6 kinase, 70kD, polypeptide 2; W36ZG6FT64 / Sirolimus
  • [Other-IDs] NLM/ NIHMS187346; NLM/ PMC2855168
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40. Galanis E, Okuno SH, Nascimento AG, Lewis BD, Lee RA, Oliveira AM, Sloan JA, Atherton P, Edmonson JH, Erlichman C, Randlev B, Wang Q, Freeman S, Rubin J: Phase I-II trial of ONYX-015 in combination with MAP chemotherapy in patients with advanced sarcomas. Gene Ther; 2005 Mar;12(5):437-45
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  • [Title] Phase I-II trial of ONYX-015 in combination with MAP chemotherapy in patients with advanced sarcomas.
  • We, therefore, undertook a phase I/II study of ONYX-015, days 1-5 every month administered intratumorally under radiographic guidance, in combination with MAP (mitomycin-C, doxorubicin, cisplatin) chemotherapy in patients with advanced sarcoma.
  • Sarcoma histologies were gastrointestinal stromal tumors (GIST, two patients), leiomyosarcoma (two patients), liposarcoma (one patient), and malignant peripheral nerve sheath tumor (1 patient).
  • Immunohistochemistry of the metastatic lesions prior to treatment showed that five out of six patients were positive for p53, while two patients also had mdm-2 overexpression.
  • ONYX-015 viral DNA was detected by quantitative PCR in the plasma of 5/6 patients on day 5 of the first cycle, and up to day 12 (7 days after the last viral dose) in one patient who had extended sampling for viral kinetics performed, suggesting viral replication in sarcoma tissue.
  • One patient with p53 mutation and MDM-2 amplification achieved a partial response to treatment that lasted 11 months.
  • In conclusion, intratumoral administration of ONYX-015 in combination with MAP chemotherapy is well tolerated with no significant toxicity due to ONYX-015 being encountered.
  • Detection of viral DNA in post treatment tumor specimens by ISH and detection of the ONYX-015 genome in the peripheral blood by quantitative PCR, up to 7 days after the last viral dose provide evidence for adenoviral replication.
  • [MeSH-major] Adenoviridae. Antineoplastic Agents / administration & dosage. Genetic Therapy / methods. Sarcoma / therapy
  • [MeSH-minor] Adult. Aged. Antibodies, Viral / blood. Antineoplastic Combined Chemotherapy Protocols / administration & dosage. Cisplatin / administration & dosage. Combined Modality Therapy. DNA, Viral / analysis. DNA, Viral / blood. Doxorubicin / administration & dosage. Female. Humans. In Situ Hybridization. Injections, Intralesional. Male. Middle Aged. Mitomycin / administration & dosage. Viral Vaccines. Virus Replication

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  • (PMID = 15647767.001).
  • [ISSN] 0969-7128
  • [Journal-full-title] Gene therapy
  • [ISO-abbreviation] Gene Ther.
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / CA 84388; United States / NCI NIH HHS / CA / U01CA 69912
  • [Publication-type] Clinical Trial; Clinical Trial, Phase I; Clinical Trial, Phase II; Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antibodies, Viral; 0 / Antineoplastic Agents; 0 / DNA, Viral; 0 / ONYX015; 0 / Viral Vaccines; 50SG953SK6 / Mitomycin; 80168379AG / Doxorubicin; Q20Q21Q62J / Cisplatin; MAP protocol
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41. Mattingly RR, Kraniak JM, Dilworth JT, Mathieu P, Bealmear B, Nowak JE, Benjamins JA, Tainsky MA, Reiners JJ Jr: The mitogen-activated protein kinase/extracellular signal-regulated kinase kinase inhibitor PD184352 (CI-1040) selectively induces apoptosis in malignant schwannoma cell lines. J Pharmacol Exp Ther; 2006 Jan;316(1):456-65
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  • [Title] The mitogen-activated protein kinase/extracellular signal-regulated kinase kinase inhibitor PD184352 (CI-1040) selectively induces apoptosis in malignant schwannoma cell lines.
  • Type 1 neurofibromatosis (NF1) is a common autosomal dominant disorder that results in neuroectodermal tumors.
  • The NF1 tumor-suppressor gene encodes neurofibromin, which includes a GTPase-activating domain for Ras inactivation.
  • Affinity purification showed N-Ras to be the predominant activated isoform of Ras in two independent neurofibrosarcoma cell lines from NF1 patients (lines ST88-14 and NF90-8).
  • These NF1 cells also demonstrated increased constitutive activity of the extracellular signal-regulated kinases 1 and 2 (ERK1,2) mitogen-activated protein (MAP) kinases compared with a sporadic malignant schwannoma cell line that maintains neurofibromin expression (STS-26T).
  • Thus, MAP kinase kinase (MEK) inhibitors may be a rational approach to NF1 therapy.
  • Hence, although PD184352 effectively killed neurofibrosarcoma cells, its effects on normal Schwann cells may limit its usefulness in the clinic.
  • [MeSH-major] Apoptosis / drug effects. Benzamides / pharmacology. Enzyme Inhibitors / pharmacology. Mitogen-Activated Protein Kinase Kinases / antagonists & inhibitors. Neurilemmoma / pathology
  • [MeSH-minor] Blotting, Western. Butadienes / pharmacology. Cell Line, Tumor. Cell Proliferation / drug effects. Flavonoids / pharmacology. Flow Cytometry. Gene Expression Regulation, Neoplastic / drug effects. Genes, p53 / genetics. Genes, ras / genetics. Humans. Mitogen-Activated Protein Kinase 7 / metabolism. Neurofibromatosis 1 / pathology. Nitriles / pharmacology. Phosphorylation. Reverse Transcriptase Polymerase Chain Reaction. Transfection


42. Ilgner J, Rojas W, Biesterfeld S, Schürmann K, Zimny M, Westhofen M: [Low-grade malignant peripheral nerve sheath tumor of the neck soft tissues]. Laryngorhinootologie; 2001 Jan;80(1):39-42
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  • [Title] [Low-grade malignant peripheral nerve sheath tumor of the neck soft tissues].
  • [Transliterated title] Niedrig-maligner peripherer Nervenscheidentumor der Halsweichteile.
  • BACKGROUND: Malignant Peripheral Nerve Sheath Tumours (MPNST) either grow sporadically, after radiation or chemotherapy respectively.
  • Because of the multiform histologic picture they are often difficult to differentiate from other soft tissue tumours.
  • PATIENT: We present the case of a sporadic MPNST which developed from the vagus nerve of a 39-year-old patient following radiation of the neck 7 years before.
  • RESULTS AND CONCLUSIONS: Sporadic MPNST of the head and neck are comparatively rare.
  • With regard to the strong association with Neurofibromatosis I and the difficult differential diagnosis to other soft tissue tumours the emphasis should be put on excluding further manifestations of Neurofibromatosis I and of secondary tumours.
  • [MeSH-major] Cranial Nerve Neoplasms / diagnosis. Head and Neck Neoplasms / diagnosis. Neoplasms, Radiation-Induced / diagnosis. Nerve Sheath Neoplasms / diagnosis. Soft Tissue Neoplasms / diagnosis. Vagus Nerve Diseases / diagnosis
  • [MeSH-minor] Adult. Cell Transformation, Neoplastic / pathology. Diagnosis, Differential. Female. Humans. Neurofibromatosis 1 / diagnosis. Neurofibromatosis 1 / pathology. Neurofibromatosis 1 / surgery. Vagus Nerve / pathology

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  • (PMID = 11272246.001).
  • [ISSN] 0935-8943
  • [Journal-full-title] Laryngo- rhino- otologie
  • [ISO-abbreviation] Laryngorhinootologie
  • [Language] ger
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Germany
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43. Holtkamp N, Malzer E, Zietsch J, Okuducu AF, Mucha J, Mawrin C, Mautner VF, Schildhaus HU, von Deimling A: EGFR and erbB2 in malignant peripheral nerve sheath tumors and implications for targeted therapy. Neuro Oncol; 2008 Dec;10(6):946-57
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  • [Title] EGFR and erbB2 in malignant peripheral nerve sheath tumors and implications for targeted therapy.
  • Malignant peripheral nerve sheath tumors (MPNSTs) are sarcomas with poor prognosis and limited treatment options.
  • Evidence for a role of epidermal growth factor receptor (EGFR) and receptor tyrosine kinase erbB2 in MPNSTs led us to systematically study these potential therapeutic targets in a larger tumor panel (n = 37).
  • Multiplex ligation-dependent probe amplification and fluorescence in situ hybridization analysis revealed increased EGFR dosage in 28% of MPNSTs.
  • ERBB2 and three tumor suppressor genes (PTEN [phosphatase and tensin homolog deleted on chromosome 10], CDKN2A [cyclin-dependent kinase inhibitor 2A], and TP53 [tumor protein p53]) were frequently lost or reduced.
  • Comparison of corresponding neurofibromas and MPNSTs revealed an increase in genetic lesions in MPNSTs.
  • However, at the protein level, expression of EGFR and erbB2 was frequently detected in MPNSTs.
  • The EGFR ligands transforming growth factor alpha and EGF were more strongly expressed in MPNSTs than in neurofibromas.
  • The effects of the drugs erlotinib and trastuzumab, which target EGFR and erbB2, were determined on MPNST cell lines.
  • Summarized, our data indicate that EGFR and erbB2 are potential targets in treatment of MPNST patients.
  • [MeSH-major] Antineoplastic Agents / administration & dosage. Drug Delivery Systems. Nerve Sheath Neoplasms / genetics. Receptor, Epidermal Growth Factor / genetics. Receptor, ErbB-2 / genetics
  • [MeSH-minor] Antibodies, Monoclonal / pharmacology. Antibodies, Monoclonal, Humanized. Blotting, Western. Cell Line, Tumor. Cell Proliferation / drug effects. Drug Synergism. Epidermal Growth Factor / genetics. Epidermal Growth Factor / metabolism. Erlotinib Hydrochloride. Gene Dosage. Genes, p16. Genes, p53. Humans. Immunohistochemistry. PTEN Phosphohydrolase / genetics. Polymorphism, Single-Stranded Conformational. Quinazolines / pharmacology. Reverse Transcriptase Polymerase Chain Reaction. Transforming Growth Factor alpha / genetics. Transforming Growth Factor alpha / metabolism. Trastuzumab


44. Khorgami Z, Nasiri S, Rezakhanlu F, Sodagari N: Malignant schwannoma of anterior abdominal wall: report of a case. J Clin Med Res; 2009 Oct;1(4):233-6

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  • [Title] Malignant schwannoma of anterior abdominal wall: report of a case.
  • Malignant schwannoma of the anterior abdominal wall nerves is extremely rare.
  • Malignant peripheral nerve sheath tumors (MPNST) represent approximately 10% of all soft tissue sarcomas and it is found in 4% of patients with neurofibromatosis 1.
  • We present a case of malignant schwannoma in a 28-year-old female patient with neurofibromatosis 1.
  • She presented with a painful mass in the right upper quadrant of her abdomen.
  • The tumor location was in the abdominal wall in explorative laparatomy and malignant schwannoma was diagnosed in pathologic assessment.
  • The tumor recurred in 3 months and computed tomography showed two masses in the right side of abdominopelvic cavity.
  • In spite of administering chemotherapy after second surgery,the tumor recurred and magnetic resonance imaging finding showed a huge heterogeneously enhancing mass with adhesion to the inner side of the abdominal wall.
  • Tumor location and rapid recurrence was unique in our patient.
  • KEYWORDS: Malignant peripheral nerve sheath tumor; Malignant schwannoma; Abdominal wall.

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  • (PMID = 22461875.001).
  • [ISSN] 1918-3003
  • [Journal-full-title] Journal of clinical medicine research
  • [ISO-abbreviation] J Clin Med Res
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Canada
  • [Other-IDs] NLM/ PMC3299187
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45. Satoh M, Komori K, Shin M, Takada T, Honda M, Fujioka H, Tsujimoto M: [Retroperitoneal malignant peripheral nerve sheath tumor (MPNST) complicated with von Recklinghausen's disease: a case report]. Hinyokika Kiyo; 2004 Jun;50(6):417-20
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  • [Title] [Retroperitoneal malignant peripheral nerve sheath tumor (MPNST) complicated with von Recklinghausen's disease: a case report].
  • A retroperitoneal malignant peripheral nerve sheath tumor (MPNST) in a patient with von Recklinghausen's disease is reported.
  • A 55-year-old woman was admitted with a left side abdominal mass.
  • Computed tomography (CT) and magnetic resonance imaging (MRI) revealed a 9 x 9 cm retroperitoneal mass.
  • Two other tumors were also found.
  • The retroperitoneal tumor was resected en bloc.
  • The tumor was a solid yellow mass.
  • Macroscopically it has a pseudocapsule of fibrous tissue, weighed 1,120 g and measured 9 x 9 x 15 cm.
  • The histopathological diagnosis was malignant peripheral nerve sheath tumor (MPNST).
  • Since the responsiveness of these tumors to chemotherapy and radiation therapy is poor, we did not administer adjuvant therapy.
  • [MeSH-major] Nerve Sheath Neoplasms / etiology. Neurofibromatosis 1 / complications. Retroperitoneal Neoplasms / etiology

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  • (PMID = 15293741.001).
  • [ISSN] 0018-1994
  • [Journal-full-title] Hinyokika kiyo. Acta urologica Japonica
  • [ISO-abbreviation] Hinyokika Kiyo
  • [Language] jpn
  • [Publication-type] Case Reports; English Abstract; Journal Article; Review
  • [Publication-country] Japan
  • [Number-of-references] 7
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46. Charfeddine I, Mnejja M, Hammami B, Hasnaoui M, Hadj KA, Frikha I, Makni S, Boudawara T, Chakroun A, Ghorbel A: [Neurofibromatosis type 1 revealed by malignant peripheral nerve sheath tumor]. Rev Laryngol Otol Rhinol (Bord); 2009;130(4-5):327-30
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Neurofibromatosis type 1 revealed by malignant peripheral nerve sheath tumor].
  • [Transliterated title] Tumeur maligne des gaines nerveuses périphériques révélant une neurofibromatose type 1.
  • Neurofibromatosis type 1 or Von Recklinghausen's disease is an affection with variable clinical expression.
  • Malignant transformation is rare and dangerous.
  • OBJECTIVE: The aim of this work is to study diagnostic criterias as well as treatment of this malignant transformation.
  • The histological exam concluded to a low grade malignant peripheral nerve sheath tumour Unfortunately despite a larger reoperation including resection of the manubrium, the limit of this excision were not safe.
  • Early recurrence was observed, although treated by surgery, chemotherapy and radiotherapy, the patient died by mediastinal invasion.
  • [MeSH-major] Nerve Sheath Neoplasms / pathology. Neurofibromatosis 1 / diagnosis. Soft Tissue Neoplasms / pathology


47. Vander Salm TJ: Unusual primary tumors of the heart. Semin Thorac Cardiovasc Surg; 2000 Apr;12(2):89-100
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Unusual primary tumors of the heart.
  • Primary tumors of the heart, with the exception of atrial myxomas, occur rarely; tumors metastatic to or directly invasive of the heart are far more common.
  • About 75% of primary tumors are benign, and 75% of these are atrial myxomas.
  • The benign tumors include rhabdomyomas, fibromas, papillary fibroelastomas, hemangiomas, pericardial cysts, lipomas, hamartomas, teratomas, mesotheliomas, and paragangliomas or pheochromocytomas.
  • The last 3 may also be malignant.
  • The malignant tumors consist of various sarcomas: myxosarcoma, liposarcoma, angiosarcoma, fibrosarcoma, leiomyosarcoma, osteosarcoma, synovial sarcoma, rhabdomyosarcoma, undifferentiated sarcoma, reticulum cell sarcoma, neurofibrosarcoma, and malignant fibrous histiocytoma.
  • Cardiac tumors produce a large variety of symptoms through any of 4 mechanisms.
  • Bits of tumor can embolize, causing systemic deficits when the tumors are on the left side of the heart.
  • Finally, the tumors may cause systemic or constitutional symptoms.
  • Some tumors, of course, produce no symptoms and become evident as incidental findings.
  • The most useful diagnostic tool is the echocardiogram, which in almost all cases precisely locates the tumor and defines its extent.
  • The echocardiographic appearance may also allow quite accurate prediction of the tumor type and whether it is malignant or benign.
  • Magnetic resonance imaging serves as the next most important test where the density of T1 and T2 images may allow tumor cell type identification.
  • With few exceptions, these tumors require operative excision.
  • Most benign tumors can be resected completely; a few, because of their large size, cannot be, and only tumor debulking may be possible.
  • Many of the malignant tumors cannot be resected completely, either because of the extent of local spread and invasion or because of the frequent distant metastases.
  • The long-term results for resected benign tumors are excellent; the long-term results for sarcomas are very poor, and there are few survivors.
  • For patients with unresectable sarcomas, radiation and chemotherapy may be used, but without great expectation of successful results.
  • [MeSH-major] Heart Neoplasms / diagnosis. Heart Neoplasms / surgery
  • [MeSH-minor] Fibroma / diagnosis. Fibroma / surgery. Hamartoma / diagnosis. Hamartoma / surgery. Heart Septum / pathology. Heart Transplantation. Hemangioma / diagnosis. Hemangioma / surgery. Humans. Hypertrophy. Mesothelioma / diagnosis. Mesothelioma / surgery. Pheochromocytoma / diagnosis. Pheochromocytoma / surgery. Prognosis. Rhabdomyoma / diagnosis. Rhabdomyoma / surgery. Teratoma / diagnosis. Teratoma / surgery

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  • (PMID = 10807431.001).
  • [ISSN] 1043-0679
  • [Journal-full-title] Seminars in thoracic and cardiovascular surgery
  • [ISO-abbreviation] Semin. Thorac. Cardiovasc. Surg.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] UNITED STATES
  • [Number-of-references] 69
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48. Gudena V, Verma N, Post G, Kizziah M, Fenning R, Montero AJ: Metastatic chest wall malignant schwannoma responding to sorafenib: case report and literature review. Cancer Biol Ther; 2008 Jun;7(6):810-3
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  • [Title] Metastatic chest wall malignant schwannoma responding to sorafenib: case report and literature review.
  • Malignant schwannomas or malignant peripheral nerve sheath tumors (MPNST) represent approximately 10% of all soft tissue sarcomas.
  • Metastatic disease from chest wall MPNST is very rare.
  • We present a case of a major clinical response to the tyrosine kinase inhibitor (TKI) sorafenib in a patient with metastatic MPNST.
  • A 42-year-old female with a prior history of neurofibromas developed MPNST, which later metastasized to the lungs and brain.
  • MPNST show high levels of Ras activity and hence these tumors are promising targets for TKIs.
  • Studies are ongoing and the results are eagerly awaited regarding the responses to these medications and whether they can positively impact on the natural history of this disease.
  • [MeSH-major] Antineoplastic Agents / therapeutic use. Benzenesulfonates / therapeutic use. Nerve Sheath Neoplasms / diagnosis. Nerve Sheath Neoplasms / drug therapy. Neurilemmoma / diagnosis. Neurilemmoma / drug therapy. Pyridines / therapeutic use. Thoracic Wall / pathology
  • [MeSH-minor] Adult. Brain Neoplasms / drug therapy. Brain Neoplasms / secondary. Female. Humans. Lung Neoplasms / drug therapy. Lung Neoplasms / secondary. Magnetic Resonance Imaging. Neoplasm Metastasis. Niacinamide / analogs & derivatives. Phenylurea Compounds. Protein Kinase Inhibitors / therapeutic use. Radiography, Thoracic. Treatment Outcome

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  • (PMID = 18376142.001).
  • [ISSN] 1555-8576
  • [Journal-full-title] Cancer biology & therapy
  • [ISO-abbreviation] Cancer Biol. Ther.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Benzenesulfonates; 0 / Phenylurea Compounds; 0 / Protein Kinase Inhibitors; 0 / Pyridines; 25X51I8RD4 / Niacinamide; 9ZOQ3TZI87 / sorafenib
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49. Dilworth JT, Wojtkowiak JW, Mathieu P, Tainsky MA, Reiners JJ Jr, Mattingly RR, Hancock CN: Suppression of proliferation of two independent NF1 malignant peripheral nerve sheath tumor cell lines by the pan-ErbB inhibitor CI-1033. Cancer Biol Ther; 2008 Dec;7(12):1938-46
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Suppression of proliferation of two independent NF1 malignant peripheral nerve sheath tumor cell lines by the pan-ErbB inhibitor CI-1033.
  • Neurofibromatosis Type 1 (NF1) is characterized by the abnormal proliferation of neuroectodermal tissues and the development of certain tumors, particularly neurofibromas, which may progress into malignant peripheral nerve sheath tumors (MPNSTs).
  • Effective pharmacological therapy for the treatment of NF1 tumors is currently unavailable and the prognosis for patients with MPNSTs is poor.
  • Loss of neurofibromin correlates with increased expression of the epidermal growth factor receptor (EGFR) and ErbB2 tyrosine kinases and these kinases have been shown to promote NF1 tumor-associated pathologies in vivo.
  • We show here that while NF1 MPNST cells have higher EGFR expression levels and are more sensitive to EGF when compared to a non-NF1 MPNST cell line, the ability of the EGFR inhibitor gefitinib to selectively inhibit NF1 MPNST cell proliferation is marginal.
  • We also show that NF1 MPNST proliferation correlates with activated ErbB2 and can be suppressed by nanomolar concentrations of the pan-ErbB inhibitor CI-1033 (canertinib).
  • Consequently, targeting both EGFR and ErbB2 may prove an effective strategy for suppressing NF1 MPNST tumor growth in vivo.

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  • [Cites] Neurochem Res. 2007 Jul;32(7):1129-41 [17404841.001]
  • [Cites] Ann Surg Oncol. 2007 Feb;14(2):942-53 [17103252.001]
  • [Cites] J Pharmacol Exp Ther. 2008 Jul;326(1):1-11 [18367665.001]
  • [Cites] Nat Clin Pract Oncol. 2008 Sep;5(9):512-20 [18594499.001]
  • [Cites] Int J Oncol. 1998 Dec;13(6):1281-4 [9824645.001]
  • [Cites] Nature. 1992 Apr 23;356(6371):713-5 [1570015.001]
  • [Cites] J Biol Chem. 1996 Aug 23;271(34):20608-16 [8702807.001]
  • [Cites] J Biol Chem. 1996 Nov 1;271(44):27456-61 [8910327.001]
  • [Cites] Mol Cell Biol. 1997 Feb;17(2):862-72 [9001241.001]
  • [Cites] Neuron. 1997 Jun;18(6):847-55 [9208852.001]
  • [Cites] J Neurosci. 1997 Nov 1;17(21):8293-9 [9334404.001]
  • [Cites] J Am Acad Dermatol. 1998 Jun;38(6 Pt 1):950-4 [9632003.001]
  • [Cites] Mol Cell Biol. 1998 Sep;18(9):5371-9 [9710621.001]
  • [Cites] Surg Neurol. 1999 Feb;51(2):211-8 [10029430.001]
  • [Cites] J Neurosci. 1999 May 15;19(10):3847-59 [10234017.001]
  • [Cites] Am J Med Genet. 1999 Mar 26;89(1):1-6 [10469430.001]
  • [Cites] Mol Cell Biol. 1999 Oct;19(10):6845-57 [10490623.001]
  • [Cites] Semin Oncol. 2004 Dec;31(6):734-43 [15599851.001]
  • [Cites] Cancer Cell. 2005 Jan;7(1):65-75 [15652750.001]
  • [Cites] Anticancer Res. 2005 May-Jun;25(3A):1699-702 [16033085.001]
  • [Cites] Oncogene. 2005 Aug 25;24(36):5589-605 [15897877.001]
  • [Cites] Onkologie. 2005 Oct;28(10):482-8 [16160394.001]
  • [Cites] Cancer Nurs. 2005 Nov-Dec;28(6):481-6 [16330971.001]
  • [Cites] J Pharmacol Exp Ther. 2006 Jan;316(1):456-65 [16239399.001]
  • [Cites] J Clin Invest. 2000 May;105(9):1233-41 [10791998.001]
  • [Cites] Toxicol Appl Pharmacol. 2000 Jun 1;165(2):115-26 [10828207.001]
  • [Cites] Biochim Biophys Acta. 2000 Jul 31;1471(1):M13-9 [10967421.001]
  • [Cites] Bioessays. 2000 Nov;22(11):987-96 [11056475.001]
  • [Cites] Traffic. 2001 Jan;2(1):12-8 [11208164.001]
  • [Cites] Oncogene. 2001 Jan 4;20(1):97-105 [11244508.001]
  • [Cites] Cell Signal. 2001 Jul;13(7):499-505 [11516625.001]
  • [Cites] Toxicol Appl Pharmacol. 2001 Nov 1;176(3):162-8 [11714248.001]
  • [Cites] Int J Cancer. 2001 Dec 15;94(6):774-82 [11745477.001]
  • [Cites] J Med Genet. 2002 May;39(5):311-4 [12011145.001]
  • [Cites] Semin Oncol. 2002 Jun;29(3 Suppl 11):11-21 [12138393.001]
  • [Cites] Cancer Res. 2002 Aug 1;62(15):4507-13 [12154062.001]
  • [Cites] J Clin Oncol. 2002 Sep 15;20(18 Suppl):1S-13S [12235219.001]
  • [Cites] J Pharmacol Exp Ther. 2002 Oct;303(1):74-81 [12235235.001]
  • [Cites] Cancer Res. 2002 Oct 15;62(20):5749-54 [12384534.001]
  • [Cites] Neurol Clin. 2002 Aug;20(3):841-65 [12432832.001]
  • [Cites] Genes Dev. 2003 Feb 15;17(4):449-54 [12600938.001]
  • [Cites] Glia. 2003 Aug;43(2):104-18 [12838503.001]
  • [Cites] J Neurosci. 2003 Aug 13;23(19):7269-80 [12917360.001]
  • [Cites] J Clin Invest. 2003 Dec;112(12):1851-61 [14679180.001]
  • [Cites] Curr Opin Neurol. 2004 Apr;17(2):101-5 [15021234.001]
  • [Cites] Otol Neurotol. 2004 Mar;25(2):155-9 [15021776.001]
  • [Cites] Science. 1987 Jul 10;237(4811):178-82 [2885917.001]
  • [Cites] Glia. 2006 Apr 15;53(6):593-600 [16432850.001]
  • [Cites] Oncogene. 2006 Apr 13;25(16):2297-303 [16288202.001]
  • [Cites] Am J Pathol. 2006 May;168(5):1686-96 [16651634.001]
  • [Cites] Hum Mol Genet. 2006 Aug 15;15(16):2421-37 [16835260.001]
  • [Cites] Genes Chromosomes Cancer. 2006 Oct;45(10):893-904 [16830335.001]
  • [Cites] Biochem Pharmacol. 2006 Nov 30;72(11):1485-92 [16797490.001]
  • [Cites] Mol Ther. 2007 Feb;15(2):279-86 [17235305.001]
  • [Cites] Mol Cancer Ther. 2008 May;7(5):1237-45 [18483311.001]
  • (PMID = 18927496.001).
  • [ISSN] 1555-8576
  • [Journal-full-title] Cancer biology & therapy
  • [ISO-abbreviation] Cancer Biol. Ther.
  • [Language] ENG
  • [Grant] United States / NIEHS NIH HHS / ES / T32 ES012163; United States / NIEHS NIH HHS / ES / P30 ES06639; United States / NIEHS NIH HHS / ES / P30 ES006639; United States / NCI NIH HHS / CA / P30 CA022453; United States / NCI NIH HHS / CA / P30 CA22453
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Morpholines; 0 / Quinazolines; C78W1K5ASF / Canertinib; EC 2.7.10.1 / Protein-Tyrosine Kinases; EC 2.7.10.1 / Receptor, Epidermal Growth Factor; EC 2.7.10.1 / Receptor, ErbB-2; S65743JHBS / gefitinib
  • [Other-IDs] NLM/ NIHMS525367; NLM/ PMC3923431
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50. Ambrosini G, Cheema HS, Seelman S, Teed A, Sambol EB, Singer S, Schwartz GK: Sorafenib inhibits growth and mitogen-activated protein kinase signaling in malignant peripheral nerve sheath cells. Mol Cancer Ther; 2008 Apr;7(4):890-6
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Sorafenib inhibits growth and mitogen-activated protein kinase signaling in malignant peripheral nerve sheath cells.
  • Malignant peripheral nerve sheath tumors (MPNST) are soft-tissue tumors with a very poor prognosis and largely resistant to chemotherapy.
  • MPNSTs are characterized by activation of the Ras pathway by loss of tumor suppressor neurofibromatosis type 1.
  • In view of this, MPNST may be susceptible to inhibition of the activated Ras/Raf/mitogen-activated protein kinase pathway by the B-Raf inhibitor sorafenib.
  • MPNST (MPNST and ST8814) and dedifferentiated liposarcoma (LS141 and DDLS) human tumor cell lines were characterized for Ras activation and B-Raf expression.
  • Tumor cells were treated with sorafenib and examined for growth inhibition, inhibition of phospho-MEK, phospho-ERK, cell cycle arrest, and changes in cyclin D1 and pRb expression.
  • MPNSTs were sensitive to sorafenib at nanomolar concentrations.
  • Small interfering RNA-mediated depletion of B-Raf in MPNSTs also induced a G(1) cell cycle arrest in these cells, with a marked inhibition of cyclin D1 expression and Rb phosphorylation, whereas depletion of C-Raf did not affect either.
  • With growth inhibition at the low nanomolar range, sorafenib, by inhibiting the mitogen-activated protein kinase pathway, may prove to be a novel therapy for patients with MPNST.

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  • [Cites] Am J Pathol. 1999 Dec;155(6):1885-91 [10595919.001]
  • [Cites] Mol Cancer Ther. 2007 Mar;6(3):1070-8 [17363500.001]
  • [Cites] Cancer Res. 2002 Mar 1;62(5):1573-7 [11894862.001]
  • [Cites] Nature. 2002 Jun 27;417(6892):949-54 [12068308.001]
  • [Cites] N Engl J Med. 2002 Aug 15;347(7):472-80 [12181401.001]
  • [Cites] Curr Pharm Des. 2002;8(25):2255-7 [12369853.001]
  • [Cites] Leukemia. 2003 Jul;17(7):1263-93 [12835716.001]
  • [Cites] Cancer Cell. 2003 Aug;4(2):95-8 [12957284.001]
  • [Cites] Cancer Res. 2003 Sep 1;63(17):5198-202 [14500344.001]
  • [Cites] Clin Cancer Res. 2004 Jun 1;10(11):3763-71 [15173083.001]
  • [Cites] Cancer Res. 2004 Oct 1;64(19):7099-109 [15466206.001]
  • [Cites] Cancer Cell. 2004 Oct;6(4):313-9 [15488754.001]
  • [Cites] Cell. 1992 Apr 17;69(2):265-73 [1568246.001]
  • [Cites] Nature. 1992 Apr 23;356(6371):713-5 [1570015.001]
  • [Cites] J Biol Chem. 1996 Aug 23;271(34):20608-16 [8702807.001]
  • [Cites] Development. 1998 Dec;125(24):4999-5008 [9811584.001]
  • [Cites] Cancer Chemother Pharmacol. 2004 Sep;54 Suppl 1:S69-77 [15316751.001]
  • [Cites] Cancer Res. 2005 Jan 1;65(1):236-45 [15665300.001]
  • [Cites] Mol Cancer Ther. 2005 Apr;4(4):677-85 [15827342.001]
  • [Cites] N Engl J Med. 2005 Aug 18;353(7):701-11 [16107623.001]
  • [Cites] Nature. 2006 Jan 19;439(7074):358-62 [16273091.001]
  • [Cites] J Biol Chem. 2006 Apr 7;281(14):9238-50 [16452469.001]
  • [Cites] Clin Cancer Res. 2006 Sep 15;12(18):5533-42 [17000690.001]
  • [Cites] Trends Mol Med. 2002 Jan;8(1):17-23 [11796262.001]
  • (PMID = 18413802.001).
  • [ISSN] 1535-7163
  • [Journal-full-title] Molecular cancer therapeutics
  • [ISO-abbreviation] Mol. Cancer Ther.
  • [Language] ENG
  • [Grant] United States / NCI NIH HHS / CA / P01 CA047179-16; United States / NCI NIH HHS / CA / T32 CA009501-18; United States / NCI NIH HHS / CA / T32 CA009501; United States / NCI NIH HHS / CA / T32 CA009501-17; United States / NCI NIH HHS / CA / P01 CA047179-17; United States / NCI NIH HHS / CA / CA09501; United States / NCI NIH HHS / CA / P01 CA047179
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Benzenesulfonates; 0 / Phenylurea Compounds; 0 / Pyridines; 0 / Retinoblastoma Protein; 136601-57-5 / Cyclin D1; 25X51I8RD4 / Niacinamide; 9ZOQ3TZI87 / sorafenib; EC 2.7.11.1 / BRAF protein, human; EC 2.7.11.1 / Proto-Oncogene Proteins B-raf; EC 2.7.11.1 / Proto-Oncogene Proteins c-raf; EC 2.7.11.22 / CDK4 protein, human; EC 2.7.11.22 / Cyclin-Dependent Kinase 4; EC 2.7.11.24 / Extracellular Signal-Regulated MAP Kinases; EC 2.7.11.24 / Mitogen-Activated Protein Kinases; EC 2.7.12.2 / Mitogen-Activated Protein Kinase Kinases; EC 3.6.5.2 / ras Proteins
  • [Other-IDs] NLM/ NIHMS349129; NLM/ PMC3267321
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51. Shimizu J, Arano Y, Murata T, Ishikawa N, Yachi T, Nomura T, Minato H: A case of intrathoracic giant malignant peripheral nerve sheath tumor in neurofibromatosis type I (von Recklinghausen's disease). Ann Thorac Cardiovasc Surg; 2008 Feb;14(1):42-7
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] A case of intrathoracic giant malignant peripheral nerve sheath tumor in neurofibromatosis type I (von Recklinghausen's disease).
  • She underwent surgery to alleviate respiratory and circulatory disorders caused by compression of the right lung and inferior vena cava due to the giant tumor.
  • Intraoperatively, the tumor was found to have originated from the 5th intercostal nerve.
  • The resected tumor was 20x17x15 cm in size and 2,300 g in weight.
  • It was histologically diagnosed as a malignant peripheral nerve sheath tumor.
  • Seven months after surgery, however, a recurrent tumor was found in the right thoracic cavity.
  • She died of rapid growth of recurrent tumor 3 months thereafter.
  • This tumor often complicates neurofibromatosis I and has a high frequency of local recurrence and distant metastasis, resulting in poor prognosis.
  • Neither an optimal extent of resection needed for complete resection of this tumor nor an optimal regimen of chemotherapy, radiotherapy, or other therapy for the tumor has yet been established.
  • [MeSH-major] Nerve Sheath Neoplasms / surgery. Neurofibromatosis 1 / complications. Thoracic Neoplasms / surgery
  • [MeSH-minor] Adult. Diagnosis, Differential. Fatal Outcome. Humans. Magnetic Resonance Imaging. Neoplasm Recurrence, Local. Tomography, X-Ray Computed

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  • (PMID = 18292741.001).
  • [ISSN] 1341-1098
  • [Journal-full-title] Annals of thoracic and cardiovascular surgery : official journal of the Association of Thoracic and Cardiovascular Surgeons of Asia
  • [ISO-abbreviation] Ann Thorac Cardiovasc Surg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Japan
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52. Mandić A, Djurdjević S, Popov M, Krnojelać D, Kukić B: Retroperitoneal malignant schwannoma and peritoneal malignant mesothelioma: a case report. J BUON; 2004 Jan-Mar;9(1):91-4

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Retroperitoneal malignant schwannoma and peritoneal malignant mesothelioma: a case report.
  • Malignant schwannoma and peritoneal malignant mesothelioma (MM) are very rare tumors.
  • Schwannoma or neurilemmoma-benign or malignant-do not arise from the nerves, but from the supporting Schwann cells.
  • Malignant peripheral nerve sheath tumors (MPNSTs) commonly are large in size.
  • A malignant schwannoma was diagnosed in a 52-yearold woman, which was surgically treated.
  • The patient was operated on for second time and MM was diagnosed as second primary tumor, along with recurrence of the malignant schwannoma.
  • The patient received postoperative adjuvant external beam radiotherapy and chemotherapy.
  • Despite combined-modality treatment the disease progressed and the patient was operated on for third time 2.5 years after the first operation with partial tumor resection.
  • She died 3 years after the first diagnosis.
  • Early diagnosis of these two types of tumors is very difficult because of unspecific clinical symptoms.
  • Singlemodality therapy of these tumors has shown poor results.

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  • (PMID = 17385835.001).
  • [ISSN] 1107-0625
  • [Journal-full-title] Journal of B.U.ON. : official journal of the Balkan Union of Oncology
  • [ISO-abbreviation] J BUON
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Greece
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53. Coffin CM, Cassity J, Viskochil D, Randall RL, Albritton K: Non-neurogenic sarcomas in four children and young adults with neurofibromatosis type 1. Am J Med Genet A; 2004 May 15;127A(1):40-3
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  • [Title] Non-neurogenic sarcomas in four children and young adults with neurofibromatosis type 1.
  • It is well known that children and young adults with neurofibromatosis type 1 (NF1) have a higher risk for non-neurogenic sarcomas than the general population, in addition to an increased risk for malignant peripheral nerve sheath tumor.
  • When non-neurogenic sarcomas occur in early childhood, a subsequent malignant peripheral nerve sheath tumor can occur as a second malignant neoplasm, especially after alkylating agent chemotherapy and irradiation.
  • This report includes the clinicopathologic features of non-neurogenic sarcomas and secondary malignant peripheral nerve sheath tumor in the context of four cases of NF1.
  • The purpose is to emphasize that early diagnosis of NF1 and recognition of potential manifestations of non-neurogenic sarcomas are important for clinical care of these patients and their families.
  • [MeSH-major] Neoplasms, Second Primary / diagnosis. Neurofibromatosis 1 / diagnosis. Sarcoma / diagnosis
  • [MeSH-minor] Adolescent. Adult. Child, Preschool. Female. Humans. Male. Nerve Sheath Neoplasms / genetics. Nerve Sheath Neoplasms / secondary. Phyllodes Tumor / diagnosis. Precursor Cell Lymphoblastic Leukemia-Lymphoma / genetics. Prognosis. Rhabdomyosarcoma, Embryonal / diagnosis. Rhabdomyosarcoma, Embryonal / genetics

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  • [Copyright] Copyright 2004 Wiley-Liss, Inc.
  • (PMID = 15103715.001).
  • [ISSN] 1552-4825
  • [Journal-full-title] American journal of medical genetics. Part A
  • [ISO-abbreviation] Am. J. Med. Genet. A
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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54. García-Alvarez García F, Gil-Albarova J, Castiella T, García-Alvarez Alvarez F: [A case of multiple malignant schwannoma as the only disease in a 40-year-old patient: is this a new type of neurofibromatosis?]. Neurologia; 2000 Feb;15(2):81-4
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  • [Title] [A case of multiple malignant schwannoma as the only disease in a 40-year-old patient: is this a new type of neurofibromatosis?].
  • [Transliterated title] A propósito de un caso de schwannoma maligno múltiple como única patología en un paciente de 40 años: un nuevo tipo de neurofibromatosis?
  • We present the case of a 40-year-old man that presented a fast growing tumour on the external side of the left elbow.
  • The tumour was extirpated by means of marginal exerectomy.
  • The microscopic study corresponded to epithelioid malignant schwannoma, and the patient received radiotherapy and adjuvant chemotherapy.
  • During the following years two local recidives and two new malignant schwannomas, one in the left sciatic common trunk and another paraspinal one, appeared and were extirpated.
  • Radiotherapy and chemotherapy were again administered.
  • In the last year, four new tumours have appeared: in the supraclavicular space, right posterior costofrenic, left costovertebral, and in the inferior abdominal wall, none of them has been extirpated or has hystologic diagnosis at the moment, however radiologic findings suggest malignant schwannomas.
  • In this moment there is no neurologic deficiency except for the secondary ones to surgical procedures, and no neurofibromatosis types I to VII signs have been observed.
  • So, the possibility of a new neurofibromatosis type is appointed.
  • [MeSH-major] Neurilemmoma / pathology. Neurofibromatosis 1 / diagnosis. Peripheral Nervous System Neoplasms / pathology
  • [MeSH-minor] Adult. Diagnosis, Differential. Humans. Magnetic Resonance Imaging. Male

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  • (PMID = 10769537.001).
  • [ISSN] 0213-4853
  • [Journal-full-title] Neurología (Barcelona, Spain)
  • [ISO-abbreviation] Neurologia
  • [Language] spa
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] SPAIN
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55. Bhola P, Banerjee S, Mukherjee J, Balasubramanium A, Arun V, Karim Z, Burrell K, Croul S, Gutmann DH, Guha A: Preclinical in vivo evaluation of rapamycin in human malignant peripheral nerve sheath explant xenograft. Int J Cancer; 2010 Jan 15;126(2):563-71
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  • [Title] Preclinical in vivo evaluation of rapamycin in human malignant peripheral nerve sheath explant xenograft.
  • Neurofibromatosis type 1 (NF1) patients are prone to the development of malignant tumors, the most common being Malignant Peripheral Nerve Sheath Tumor (MPNST).
  • NF1-MPNST patients have an overall poor survival due to systemic metastasis.
  • Currently, the management of MPNSTs includes surgery and radiation; however, conventional chemotherapy is not very effective, underscoring the need for effective biologically-targeted therapies.
  • Recently, the NF1 gene product, neurofibromin, was shown to negatively regulate the phosphoinositide-3-kinase (PI3K)/Protein Kinase-B (Akt)/mammalian Target Of Rapamycin (mTOR) pathway, with loss of neurofibromin expression in established human MPNST cell lines associated with high levels of mTOR activity.
  • We developed and characterized a human NF1-MPNST explant grown subcutaneously in NOD-SCID mice, to evaluate the effect of the mTOR inhibitor rapamycin.
  • We demonstrate that rapamycin significantly inhibited human NF1-MPNST mTOR pathway activation and explant growth in vivo at doses as low as 1.0 mg/kg/day, without systemic toxicities.
  • While rapamycin was effective at reducing NF1-MPNST proliferation and angiogenesis, with decreased CyclinD1 and VEGF respectively, there was no increase in tumor apoptosis.
  • This study demonstrates the therapeutic potential and limitations of rapamycin in NF1-associated, and likely sporadic, MPNSTs.
  • [MeSH-major] Neurofibromatosis 1 / drug therapy. Peripheral Nervous System Neoplasms / drug therapy. Sirolimus / pharmacology. Xenograft Model Antitumor Assays
  • [MeSH-minor] Animals. Antibiotics, Antineoplastic / pharmacology. Apoptosis / drug effects. Blotting, Western. Cyclin D1 / metabolism. Dose-Response Relationship, Drug. Drug Evaluation, Preclinical. Humans. Immunohistochemistry. Male. Mice. Mice, Inbred NOD. Mice, SCID. Protein Kinases / metabolism. Proto-Oncogene Proteins c-akt / metabolism. Ribosomal Protein S6 Kinases / metabolism. Signal Transduction / drug effects. TOR Serine-Threonine Kinases. Tumor Burden / drug effects. Vascular Endothelial Growth Factor A / metabolism. Young Adult

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  • (PMID = 19634141.001).
  • [ISSN] 1097-0215
  • [Journal-full-title] International journal of cancer
  • [ISO-abbreviation] Int. J. Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antibiotics, Antineoplastic; 0 / Vascular Endothelial Growth Factor A; 136601-57-5 / Cyclin D1; EC 2.7.- / Protein Kinases; EC 2.7.1.1 / MTOR protein, human; EC 2.7.1.1 / TOR Serine-Threonine Kinases; EC 2.7.1.1 / mTOR protein, mouse; EC 2.7.11.1 / Proto-Oncogene Proteins c-akt; EC 2.7.11.1 / Ribosomal Protein S6 Kinases; W36ZG6FT64 / Sirolimus
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56. Torres Lobatón A, Cruz Ortiz H, Rojo Herrera G, Avila Medrano L: [Sarcomas of the vulva. Report of 2 cases]. Ginecol Obstet Mex; 2000 Oct;68:429-34
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  • [Transliterated title] Sarcomas de la vulva. Informe de dos casos.
  • The first case was a teenager of 14 years-old with a low grade leiomyosarcoma surgically treated.
  • Along a 22 years follow-up the disease has had four local recurrences of more than 5 cm each one: two after surgery and two after surgery plus chemotherapy and surgery plus radiotherapy respectively.
  • She is alive disease evidence after two years from the last combined treatment.
  • The second one, was a 26 years-old patient with a malignant schwannoma of 12 cm in diameter treated with combined radical surgery, radiotherapy, and chemotherapy.
  • We emphasized that these tumors are very rare and the fact that the first patient is the youngest and with more years of follow up according the bibliography consulted.
  • Treatment of vulvar sarcomas is radical local excision followed mainly by radiotherapy with infiltrating margins.
  • The value of postoperative adjuvant chemotherapy is uncertain.
  • According to the natural history and behavior of vulvar sarcomas, we conclude that the elective treatment of these tumors should be carry out in institutions of high level.
  • [MeSH-major] Leiomyosarcoma. Neurilemmoma. Vulvar Neoplasms
  • [MeSH-minor] Abdominal Neoplasms / drug therapy. Abdominal Neoplasms / radiotherapy. Abdominal Neoplasms / secondary. Adolescent. Adult. Antineoplastic Agents, Alkylating / therapeutic use. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Bartholin's Glands. Chemotherapy, Adjuvant. Combined Modality Therapy. Cyclophosphamide / administration & dosage. Cystectomy. Cysts / diagnosis. Dacarbazine / administration & dosage. Dacarbazine / therapeutic use. Diagnosis, Differential. Disease-Free Survival. Doxorubicin / administration & dosage. Female. Follow-Up Studies. Humans. Neoplasm Recurrence, Local. Pelvic Neoplasms / drug therapy. Pelvic Neoplasms / radiotherapy. Pelvic Neoplasms / secondary. Radioisotope Teletherapy. Retrospective Studies. Treatment Outcome. Urinary Bladder Neoplasms / secondary. Urinary Bladder Neoplasms / surgery

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  • (PMID = 11138405.001).
  • [ISSN] 0300-9041
  • [Journal-full-title] Ginecología y obstetricia de México
  • [ISO-abbreviation] Ginecol Obstet Mex
  • [Language] spa
  • [Publication-type] Case Reports; English Abstract; Journal Article; Review
  • [Publication-country] Mexico
  • [Chemical-registry-number] 0 / Antineoplastic Agents, Alkylating; 7GR28W0FJI / Dacarbazine; 80168379AG / Doxorubicin; 8N3DW7272P / Cyclophosphamide
  • [Number-of-references] 12
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57. Steib JP, Bouchaïb J, Walter A, Schuller S, Charles YP: Could an osteoinductor result in degeneration of a neurofibroma in NF1? Eur Spine J; 2010 Jul;19 Suppl 2:S220-5
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  • [Title] Could an osteoinductor result in degeneration of a neurofibroma in NF1?
  • Five months later, he developed a huge posterior tumour on his back.
  • The biopsy diagnosed a neurofibrosarcoma.
  • The growth of the tumour was extremely rapid.
  • NF1 is characterised by neurofibromas that have a possibility of malign degeneration and conversion to a sarcoma.
  • However, the chronology, rapidity of evolution and the exceptional volume of the tumour made us wonder whether the BMP had a part of responsibility as osteoinductor in the malignant degeneration, in this particular case, of neurofibromatosis.
  • [MeSH-major] Bone Morphogenetic Proteins / adverse effects. Neurofibrosarcoma / chemically induced. Neurofibrosarcoma / pathology. Postoperative Complications / drug therapy. Spinal Neoplasms / chemically induced. Spinal Neoplasms / pathology

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  • [Cites] J Pediatr Surg. 2003 Mar;38(3):343-6; discussion 343-6 [12632346.001]
  • [Cites] Spine J. 2002 Mar-Apr;2(2):151-5 [14588275.001]
  • [Cites] Arch Pediatr. 2004 Jun;11(6):553-4 [15158830.001]
  • [Cites] J Orthop Sci. 2004;9(3):334-40 [15168194.001]
  • [Cites] Hum Genet. 1990 Aug;85(3):337-42 [2118474.001]
  • [Cites] J Spinal Disord. 1988;1(1):39-49 [2980062.001]
  • [Cites] Rev Chir Orthop Reparatrice Appar Mot. 1996;82(4):313-20 [8952911.001]
  • [Cites] Spine (Phila Pa 1976). 1998 Dec 15;23(24):2738-45 [9879099.001]
  • [Cites] Eur Spine J. 2005 Jun;14(5):427-39 [15712001.001]
  • [Cites] Spine J. 2005 Jul-Aug;5(4):461-6 [15996618.001]
  • [Cites] Can J Neurol Sci. 2005 May;32(2):225-31 [16018159.001]
  • [Cites] Cancer. 2007 Apr 1;109(7):1406-12 [17330850.001]
  • [Cites] Neurosurg Focus. 2007;22(6):E3 [17613220.001]
  • [Cites] Orthop Clin North Am. 2007 Oct;38(4):553-62, vii [17945135.001]
  • [Cites] Rev Neurol (Paris). 2008 Jan;164(1):82-6 [18342062.001]
  • [Cites] Mol Genet Metab. 2008 May;94(1):105-11 [18289904.001]
  • [Cites] Spine (Phila Pa 1976). 2008 Nov 1;33(23):E881-6 [18978582.001]
  • [Cites] J Bone Joint Surg Am. 2008 Dec;90(12):2735-44 [19047720.001]
  • [Cites] Bone. 2009 Feb;44(2):233-42 [19038372.001]
  • [ErratumIn] Eur Spine J. 3010 Aug;19(8):1394. Jean-Paul, Steib [corrected to Steib, Jean-Paul]; Julia, Bouchaïb [corrected to Bouchaïb, Julia]; Axel, Walter [corrected to Walter, Axel]; Sébastien, Schuller [corrected to Schuller, Sébastien]; Philippe, Charles [corrected to Charles, Yann Philippe]
  • (PMID = 20449613.001).
  • [ISSN] 1432-0932
  • [Journal-full-title] European spine journal : official publication of the European Spine Society, the European Spinal Deformity Society, and the European Section of the Cervical Spine Research Society
  • [ISO-abbreviation] Eur Spine J
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Bone Morphogenetic Proteins
  • [Other-IDs] NLM/ PMC2899640
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58. Terzic A, Bode B, Gratz KW, Stoeckli SJ: Prognostic factors for the malignant triton tumor of the head and neck. Head Neck; 2009 May;31(5):679-88
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  • [Title] Prognostic factors for the malignant triton tumor of the head and neck.
  • BACKGROUND: Malignant triton tumors are rare neoplasias consisting of a malignant peripheral nerve sheath tumor with additional rhabdomyoblastic differentiation.
  • These tumors are highly aggressive and prognosis is poor.
  • METHODS: From 1993 to 2005, 7 patients with a malignant triton tumor of the head and neck were treated at our institution.
  • RESULTS: Patients with primary tumors involving the nose and paranasal sinuses have better, patients involving the neck a poor prognosis.
  • Additional radiation or chemotherapy show little effect.
  • CONCLUSION: Location of the primary tumor is a key factor for prognosis.
  • Complete surgical removal is the only treatment associated with survival.
  • [MeSH-major] Head and Neck Neoplasms / mortality. Nerve Sheath Neoplasms / mortality

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  • (PMID = 19283843.001).
  • [ISSN] 1097-0347
  • [Journal-full-title] Head & neck
  • [ISO-abbreviation] Head Neck
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 47
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59. Jarkowski A 3rd: Possible contribution of aprepitant to ifosfamide-induced neurotoxicity. Am J Health Syst Pharm; 2008 Dec 1;65(23):2229-31
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  • SUMMARY: A 24-year-old white man diagnosed with a malignant peripheral nerve sheath tumor initially in the late 1990s was admitted to the hospital for treatment of a recurrence of the tumor in the supra-clavicular region.
  • With the fifth cycle of therapy, the patient suffered severe nausea and vomiting that required his readmission to the hospital.
  • With the initiation of the sixth cycle of chemotherapy, aprepitant was added to the existing antiemetic regimen of ondansetron and dexamethasone.
  • Since his symptoms resolved by morning, it was determined that the patient did not require treatment with methylene blue.
  • With the initiation of the seventh cycle of chemotherapy, aprepitant was again added to the standard antiemetic regimen of a corticosteroid and serotonin receptor antagonist.
  • The patient tolerated chemotherapy well without any signs or symptoms of neurotoxicity and was discharged four days later.
  • CONCLUSION: A 24-year-old patient treated with ifosfamide, carboplatin, and etoposide for a malignant peripheral nerve sheath tumor developed ifosfamide-induced neurotoxicity after the addition of aprepitant to a standard antiemetic regimen consisting of ondansetron and dexamethasone.
  • [MeSH-major] Antiemetics / adverse effects. Antineoplastic Combined Chemotherapy Protocols / adverse effects. Ifosfamide / adverse effects. Morpholines / adverse effects. Nerve Sheath Neoplasms / secondary. Neurotoxicity Syndromes / etiology
  • [MeSH-minor] Adult. Carboplatin / administration & dosage. Dexamethasone / administration & dosage. Drug Therapy, Combination. Etoposide / administration & dosage. Humans. Male. Nausea / prevention & control

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  • (PMID = 19020190.001).
  • [ISSN] 1535-2900
  • [Journal-full-title] American journal of health-system pharmacy : AJHP : official journal of the American Society of Health-System Pharmacists
  • [ISO-abbreviation] Am J Health Syst Pharm
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antiemetics; 0 / Morpholines; 1NF15YR6UY / aprepitant; 6PLQ3CP4P3 / Etoposide; 7S5I7G3JQL / Dexamethasone; BG3F62OND5 / Carboplatin; UM20QQM95Y / Ifosfamide
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60. Gallo A, Suriano M, Simonelli M, Ralli G, de Vincentiis M: Recurrent malignant schwannoma of the parapharyngeal space in neurofibromatosis type 1. Ear Nose Throat J; 2003 Nov;82(11):862-5
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  • [Title] Recurrent malignant schwannoma of the parapharyngeal space in neurofibromatosis type 1.
  • Malignant schwannoma is an aggressive tumor that carries a poor prognosis despite wide excision, chemotherapy, and radiotherapy.
  • Malignant schwannoma of the parapharyngeal space is an uncommon finding; to our knowledge, only four cases have been described in the literature during the past 30 years, and only one of them involved a patient who had clinical evidence of neurofibromatosis type 1.
  • In this article, we describe a new case of malignant schwannoma of the parapharyngeal space in a patient who had clinical evidence of neurofibromatosis type 1.
  • Following resection of the tumor and a total parotidectomy, the diagnosis was made on the basis of histology and immunohistochemistry.
  • The patient underwent postoperative chemotherapy with carboplatin and UP16.
  • However, 5 months following surgery, the tumor recurred and metastasized.
  • The IVA2 regimen slowed tumor growth, but 13 months after the initiation of therapy, the patient died of neoplastic cachexia.
  • Although chemotherapy is generally ineffective in most cases of malignant schwannoma, we did experience some positive results with the IVA2 protocol.
  • Therefore, we recommend that this combination be considered as a first-line adjuvant therapy following surgery or as a first-line therapy for patients with inoperable tumors.
  • [MeSH-major] Neurilemmoma / complications. Neurilemmoma / pathology. Neurofibromatosis 1 / complications. Pharyngeal Neoplasms / complications. Pharyngeal Neoplasms / pathology
  • [MeSH-minor] Adult. Fatal Outcome. Humans. Magnetic Resonance Imaging. Male. Neoplasm Recurrence, Local


61. Kochbati L, Boussen H, Benna F, Belhaj Ali Z, Gammoudi A, Bouaouina N, Besbes M, Ghilen L, Rahal K, Maalej M: [Second malignancies following Hodgkin's disease treatment in Tunisia. Retrospective study of 26 cases observed at the institute Salah-Azaïz]. Cancer Radiother; 2003 Oct;7(5):302-7
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  • [Title] [Second malignancies following Hodgkin's disease treatment in Tunisia. Retrospective study of 26 cases observed at the institute Salah-Azaïz].
  • [Transliterated title] Tumeurs secondaires après traitement pour maladie de Hodgkin en Tunisie. Etude rétrospective à propos de 26 cas observés à l'institut Salah-Azaïz.
  • METHODS AND PATIENTS: We consider as second cancer all tumours other than HD observed in patients after treatment for HD.
  • RESULTS: Twenty-five patients among 614 treated for HD between 1975 and 1991 developed 26 secondary tumours (4.2%).
  • Mean age at the diagnosis of HD was 32.5 years (12-56).
  • The first treatment was combined chemotherapy and radiotherapy in 22 cases and only chemotherapy in three cases (stage IV).
  • Mean dose was 41.3 Gy (2 Gy/fraction in 21 and 3.3 in one).
  • Chemotherapy was MOPP (13), MOPP and vinblastine (four), MOPP-ABVD (five), ABVD (two) and vinblastine only in one.
  • Mean delay of second tumours was 114.5 months (40-276).
  • There was five acute myeloid leukaemia, two digestive non-Hodgkin lymphomas, five nodal high-grade lymphomas, three breast cancers (one in man associated with thyroid cancer), five lung cancers (three non-small cell and two of small cell type), two gastric tumours and one rectal cancer, one synovialosarcoma of the knee and one malignant Schwannoma of the neck.
  • CONCLUSION: Second cancer risk after treatment for HD is not low.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / adverse effects. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Hodgkin Disease / drug therapy. Hodgkin Disease / radiotherapy. Neoplasms, Second Primary / epidemiology. Neoplasms, Second Primary / etiology

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  • (PMID = 14522350.001).
  • [ISSN] 1278-3218
  • [Journal-full-title] Cancer radiothérapie : journal de la Société française de radiothérapie oncologique
  • [ISO-abbreviation] Cancer Radiother
  • [Language] fre
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] France
  • [Chemical-registry-number] 11056-06-7 / Bleomycin; 35S93Y190K / Procarbazine; 50D9XSG0VR / Mechlorethamine; 5J49Q6B70F / Vincristine; 5V9KLZ54CY / Vinblastine; 7GR28W0FJI / Dacarbazine; 80168379AG / Doxorubicin; VB0R961HZT / Prednisone; ABVD protocol; MOPP protocol
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62. Neragi-Miandoab S, Kim J, Vlahakes GJ: Malignant tumours of the heart: a review of tumour type, diagnosis and therapy. Clin Oncol (R Coll Radiol); 2007 Dec;19(10):748-56
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  • [Title] Malignant tumours of the heart: a review of tumour type, diagnosis and therapy.
  • In this overview, current published studies concerning malignant neoplasms of the heart are reviewed, together with some insights into their aetiology, diagnosis and management.
  • Sarcomas are the most common cardiac tumours and include myxosarcoma, liposarcoma, angiosarcoma, fibrosarcoma, leiomyosarcoma, osteosarcoma, synovial sarcoma, rhabdomyosarcoma, neurofibrosarcoma, malignant fibrous histiocytoma and undifferentiated sarcoma.
  • The classic symptoms of cardiac tumours are intracardiac obstruction, signs of systemic embolisation, and systemic or constitutional symptoms.
  • However, serious complications including stroke, myocardial infarction and even sudden death from arrhythmia may be the first signs of a tumour.
  • Computed tomography and magnetic resonance imaging studies have improved the diagnostic approach in recent decades.
  • Successful treatment for benign cardiac tumours is usually achieved by surgical resection.
  • Unfortunately, resection of the tumour is not always feasible.
  • The prognosis after surgery is usually excellent in the case of benign tumours, but the prognosis of malignant tumours remains dismal.
  • Therefore, a high level of suspicion is required for early diagnosis.
  • Surgery is the cornerstone of therapy.
  • However, a multi-treatment approach, including chemotherapy, radiation as well as evolving approaches such as gene therapy, might provide a better palliative and curative result.
  • [MeSH-minor] Antineoplastic Agents / therapeutic use. Cardiovascular Surgical Procedures. Echocardiography, Three-Dimensional. Humans. Magnetic Resonance Imaging. Tomography, X-Ray Computed

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  • (PMID = 17693068.001).
  • [ISSN] 0936-6555
  • [Journal-full-title] Clinical oncology (Royal College of Radiologists (Great Britain))
  • [ISO-abbreviation] Clin Oncol (R Coll Radiol)
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antineoplastic Agents
  • [Number-of-references] 77
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63. Dartnell J, Pilling J, Ferner R, Cane P, Lang-Lazdunski L: Malignant triton tumor of the brachial plexus invading the left thoracic inlet: a rare differential diagnosis of pancoast tumor. J Thorac Oncol; 2009 Jan;4(1):135-7

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  • [Title] Malignant triton tumor of the brachial plexus invading the left thoracic inlet: a rare differential diagnosis of pancoast tumor.
  • Malignant triton tumor is a divergent malignant peripheral nerve sheath tumor with rhabdomyoblastic differentiation.
  • We report a case of malignant triton tumor arising in the brachial plexus of a 28-year-old women with neurofibromatosis type 1.
  • Fluorodeoxyglucose-positron emission tomography-computed tomography before excision demonstrated a tumor with a maximum standard uptake value of 21 at 4 hours postinjection.
  • The patient underwent complete excision of the tumor through median sternotomy and left supraclavicular approach.
  • Adjuvant radiotherapy and chemotherapy were planned but the patient died of metastatic disease within 3 months of surgical resection.
  • [MeSH-major] Brachial Plexus / pathology. Neurilemmoma / diagnosis. Pancoast Syndrome / diagnosis. Peripheral Nervous System Neoplasms / diagnosis. Thoracic Neoplasms / diagnosis
  • [MeSH-minor] Adult. Diagnosis, Differential. Fatal Outcome. Female. Fluorodeoxyglucose F18. Humans. Magnetic Resonance Imaging. Neoplasm Invasiveness. Neurofibromatosis 1 / complications. Positron-Emission Tomography. Radiopharmaceuticals. Tomography, X-Ray Computed

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  • (PMID = 19096322.001).
  • [ISSN] 1556-1380
  • [Journal-full-title] Journal of thoracic oncology : official publication of the International Association for the Study of Lung Cancer
  • [ISO-abbreviation] J Thorac Oncol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Radiopharmaceuticals; 0Z5B2CJX4D / Fluorodeoxyglucose F18
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64. Hatori M, Hosaka M, Watanabe M, Moriya T, Sasano H, Kokubun S: Osteosarcoma in a patient with neurofibromatosis type 1: a case report and review of the literature. Tohoku J Exp Med; 2006 Apr;208(4):343-8
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  • [Title] Osteosarcoma in a patient with neurofibromatosis type 1: a case report and review of the literature.
  • Neurofibromatosis type 1 (NF1) or von Recklinghausen's disease is a genetic disease generally characterized by café-au-lait spots and neurofibromas.
  • Malignant tumors of the nervous system, such as malignant schwannomas, gliomas, or astrocytomas, have been well known to coexist with neurofibromatosis.
  • However, occurrence of malignant tumors unrelated to the nervous system is rare.
  • We report an unusual case of a 29-year-old NF1 female suffering from malignant peripheral nerve sheath tumor (MPNST) that eventually developed osteosarcoma in the proximal femur.
  • Osteosarcoma is the most common high-grade malignant bone tumor in which the neoplastic cells produce osteoid.
  • At 23 and 24 years old, she underwent excision of MPNST in the left posterior thigh.
  • The patient underwent postoperative chemotherapy.
  • Computed tomography demonstrated cortical bone destruction in the left proximal femur where MPNST occurred.
  • Magnetic resonance imaging revealed extraskeletal growth of the tumor.
  • The removed tumor was composed of highly anaplastic cells.
  • Lace-like irregular osteoid formation was observed among the tumor cells.
  • MPNST component was totally absent.
  • The tumor was diagnosed as osteoblastic type osteosarcoma.
  • The correlation between the histogenesis of osteosarcoma and the genetic abnormality in NF1 patients has not been elucidated, but the finding of osteosarcomatous transformation in this case suggests the divergent cellular differentiation to mesenchymal malignant tumors of neuroectodermal tissue in NF1 patients.
  • [MeSH-minor] Adult. Cell Transformation, Neoplastic. Combined Modality Therapy. Fatal Outcome. Female. Femur / pathology. Femur / radiography. Humans. Magnetic Resonance Imaging. Nerve Sheath Neoplasms / pathology. Nerve Sheath Neoplasms / therapy


65. Sierszeń W, Stankiewicz C: [Malignant schwannoma of infraorbital nerve]. Otolaryngol Pol; 2003;57(4):573-6
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  • [Title] [Malignant schwannoma of infraorbital nerve].
  • Schwannoma of the head and neck may develop from many nerves, i.e. cranial or spinal.
  • The authors described malignant schwannoma of infraorbital nerve.
  • Patient was treated by combined method: in first step chemotherapy, in second surgical excision with 2 year disease-free survival.
  • [MeSH-major] Neurilemmoma / pathology. Oculomotor Nerve / pathology. Peripheral Nervous System Neoplasms / pathology

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  • (PMID = 14587398.001).
  • [ISSN] 0030-6657
  • [Journal-full-title] Otolaryngologia polska = The Polish otolaryngology
  • [ISO-abbreviation] Otolaryngol Pol
  • [Language] pol
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Poland
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66. Ferrari A, Bisogno G, Carli M: Management of childhood malignant peripheral nerve sheath tumor. Paediatr Drugs; 2007;9(4):239-48
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  • [Title] Management of childhood malignant peripheral nerve sheath tumor.
  • Malignant peripheral nerve sheath tumor (MPNST) is rare, but is one of the most frequent non-rhabdomyosarcoma soft-tissue sarcomas in the pediatric population.
  • These tumors occur most frequently at axial sites and are characterized by local aggressiveness and a propensity to metastasize.
  • They are often associated with neurofibromatosis type 1 (NF-1): the lifetime risk of patients with NF-1 developing MPNST has been estimated at 8-13%, compared with 0.001% in the general population.
  • Because of the rarity of this tumor, little information is available on its clinical management, particularly in the pediatric age group.
  • In a recent report on the clinical findings and treatment outcomes from a large number of children and adolescents with MPNST in an Italian and German series, less satisfactory overall outcomes than those for other pediatric sarcomas were described.
  • Therefore, the approach to the treatment of patients with MPNST should be aggressive and risk adapted, and is necessarily complex.
  • Patients should be referred to selected institutions with adequate experience in treating soft-tissue sarcomas, and with the multidisciplinary skills for enrolling patients in clinical trials.
  • Surgical resection represents the mainstay of treatment, while the role of adjuvant treatment is not yet clear.
  • Although lack of local control is the major cause of treatment failure, MPNST may give rise to distant metastases.
  • These tumors are usually considered as having uncertain chemosensitivity, but recent evidence suggests that there may be a role for chemotherapy in patients with a high-grade histology.
  • For the near future, our hopes lie in the development of novel tailored therapies directed specifically against the molecular targets of the neoplastic cells: soft-tissue sarcomas seem particularly promising candidates for targeted therapy.
  • [MeSH-major] Antineoplastic Agents / therapeutic use. Nerve Sheath Neoplasms / therapy
  • [MeSH-minor] Adolescent. Child. Child, Preschool. Drug Delivery Systems. Humans. Infant. Infant, Newborn. Neoplasm Staging. Neurofibromatosis 1 / complications. Prognosis. Treatment Outcome

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  • [Cites] Cancer. 1981 May 15;47(10):2503-9 [6791802.001]
  • [Cites] Cancer. 1986 Mar 15;57(6):1225-9 [3080222.001]
  • [Cites] J Clin Oncol. 2001 Mar 1;19(5):1238-47 [11230464.001]
  • [Cites] Am J Hum Genet. 2003 May;72(5):1288-92 [12660952.001]
  • [Cites] Cancer. 1973 Jan;31(1):184-90 [4683036.001]
  • [Cites] Lab Invest. 2001 Jun;81(6):833-44 [11406645.001]
  • [Cites] Nature. 1992 Apr 23;356(6371):713-5 [1570015.001]
  • [Cites] Am J Med Genet. 1997 May 16;70(2):138-43 [9128932.001]
  • [Cites] J Med Genet. 2002 May;39(5):311-4 [12011145.001]
  • [Cites] J Clin Invest. 2000 May;105(9):1233-41 [10791998.001]
  • [Cites] Cancer. 1993 Feb 15;71(4):1247-53 [8435801.001]
  • [Cites] Expert Rev Anticancer Ther. 2005 Apr;5(2):307-18 [15877527.001]
  • [Cites] Cancer. 2007 Apr 1;109(7):1406-12 [17330850.001]
  • [Cites] J Child Neurol. 2002 Aug;17(8):548-54; discussion 571-2, 646-51 [12403552.001]
  • [Cites] JAMA. 1997 Jul 2;278(1):51-7 [9207339.001]
  • [Cites] Cancer. 1988 Jan 15;61(2):209-20 [3275486.001]
  • [Cites] J Neurol Neurosurg Psychiatry. 2000 Mar;68(3):353-7 [10675220.001]
  • [Cites] Lancet. 2002 Jun 29;359(9325):2235-41 [12103287.001]
  • [Cites] N Engl J Med. 1986 Apr 17;314(16):1010-5 [3083258.001]
  • [Cites] Genes Chromosomes Cancer. 1994 Aug;10(4):250-5 [7522538.001]
  • [Cites] Cancer. 2006 Sep 1;107(5):1065-74 [16881077.001]
  • [Cites] J Clin Oncol. 1999 Dec;17(12):3697-705 [10577841.001]
  • [Cites] J Clin Oncol. 2005 Jun 20;23(18):4031-8 [15767644.001]
  • [Cites] Cancer. 1963 Aug;16:1015-27 [14050005.001]
  • [Cites] Cancer. 1993 Nov 1;72(9):2746-54 [8402499.001]
  • [Cites] Lancet. 1997 Dec 6;350(9092):1647-54 [9400508.001]
  • [Cites] Med Pediatr Oncol. 1998 Apr;30(4):201-9 [9473754.001]
  • [Cites] J Pediatr Hematol Oncol. 1999 Nov-Dec;21(6):509-13 [10598662.001]
  • [Cites] Mod Pathol. 1995 Sep;8(7):705-10 [8539226.001]
  • [Cites] Pediatr Blood Cancer. 2005 Aug;45(2):128-34 [15852434.001]
  • [Cites] Med Pediatr Oncol. 1994;22(2):78-83 [8259105.001]
  • [Cites] J Clin Oncol. 2005 Nov 20;23(33):8422-30 [16293873.001]
  • [Cites] J Clin Oncol. 2002 Aug 1;20(15):3225-35 [12149295.001]
  • [Cites] Ann Surg Oncol. 1995 Nov;2(6):524-9 [8591083.001]
  • [Cites] Clin Orthop Relat Res. 2004 Sep;(426):69-73 [15346054.001]
  • [Cites] J Clin Oncol. 1997 Jan;15(1):350-62 [8996162.001]
  • [Cites] Am J Surg Pathol. 1992 Jul;16(7):694-707 [1530109.001]
  • [Cites] Pediatr Neurol. 2005 Apr;32(4):221-8 [15797177.001]
  • [Cites] Cancer. 1990 Sep 15;66(6):1253-65 [2119249.001]
  • [Cites] Clin Cancer Res. 2003 Sep 15;9(11):4132-8 [14519636.001]
  • [Cites] Cell. 1990 Aug 10;62(3):599-608 [2116237.001]
  • [Cites] J Clin Oncol. 2005 Jun 20;23(18):4021-30 [15767645.001]
  • [Cites] Int J Radiat Oncol Biol Phys. 1998 Sep 1;42(2):351-60 [9788415.001]
  • [Cites] Eur J Surg Oncol. 1995 Feb;21(1):78-82 [7851559.001]
  • [Cites] J Clin Oncol. 1999 Apr;17(4):1219 [10561182.001]
  • [Cites] Genes Chromosomes Cancer. 1999 Dec;26(4):376-80 [10534774.001]
  • [Cites] Cancer. 1986 May 15;57(10):2006-21 [3082508.001]
  • [Cites] J Clin Oncol. 1998 Jan;16(1):197-203 [9440743.001]
  • [Cites] Cancer. 1987 Jan 1;59(1):1-5 [3791140.001]
  • [Cites] Nat Rev Cancer. 2005 Jul;5(7):557-64 [16069817.001]
  • [Cites] J Clin Oncol. 2006 Jan 20;24(3):507-16 [16421428.001]
  • [Cites] Hum Mutat. 1994;4(2):83-101 [7981724.001]
  • [Cites] Science. 1990 Jul 13;249(4965):181-6 [2134734.001]
  • [Cites] Genes Chromosomes Cancer. 1991 Jan;3(1):62-70 [1906341.001]
  • [Cites] Cancer Cell. 2005 Jan;7(1):65-75 [15652750.001]
  • (PMID = 17705563.001).
  • [ISSN] 1174-5878
  • [Journal-full-title] Paediatric drugs
  • [ISO-abbreviation] Paediatr Drugs
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] Switzerland
  • [Chemical-registry-number] 0 / Antineoplastic Agents
  • [Number-of-references] 59
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67. Jeyaretna DS, Rabkin SD, Martuza RL: Oncolytic herpes simplex virus therapy for peripheral nerve tumors. Neurosurg Focus; 2007;22(6):E4
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  • [Title] Oncolytic herpes simplex virus therapy for peripheral nerve tumors.
  • Oncolytic viruses are one of many emerging cancer therapies.
  • The surgical management of peripheral nerve tumors carries an inherent risk of damaging the nerves involved and so the search for novel therapies with reduced risk of morbidity continues.
  • In this review the authors discuss the use of oncolytic herpes simplex virus (HSV) in the treatment of peripheral nerve tumors.
  • Herpes simplex virus has a number of characteristics that make it a useful oncolytic vector, including its large, sequenced genome that can accommodate multiple transgenes, its lack of insertional mutagenesis, its ability to infect a wide array of cell types in various species, and the availability of well-established antiviral therapies to treat it.
  • The efficacy of oncolytic HSV therapy against schwannomas and malignant peripheral nerve sheath tumors has been studied in multiple experimental models both in vitro and in vivo.
  • The virus utilizes cell pathways unique to tumors to enhance its oncolytic efficacy, preferentially and effectively targeting and destroying peripheral nerve tumor cells without harming normal cells.
  • This effect is augmented by transgenes expressing antiangiogenic factors, such as dominant-negative fibroblast growth factor receptor and platelet factor 4, and displays synergy with chemotherapy.
  • In addition, new animal models have been developed to test the efficacy of oncolytic HSV therapy in peripheral nerve tumors.
  • [MeSH-major] Oncolytic Virotherapy / methods. Peripheral Nervous System Neoplasms / therapy. Simplexvirus / genetics
  • [MeSH-minor] Animals. Genetic Vectors / therapeutic use. Humans. Oncolytic Viruses / genetics

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  • (PMID = 17613221.001).
  • [ISSN] 1092-0684
  • [Journal-full-title] Neurosurgical focus
  • [ISO-abbreviation] Neurosurg Focus
  • [Language] eng
  • [Grant] United States / NINDS NIH HHS / NS / R01 NS032677
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, U.S. Gov't, Non-P.H.S.; Review
  • [Publication-country] United States
  • [Number-of-references] 37
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68. Munteanu M, Pîrşcoveanu M, Gugilă I, Munteanu MC, Munteanu AC, Ionescu M: [Rare case of retroperitoneal malignant Schwannoma]. Chirurgia (Bucur); 2004 Sep-Oct;99(5):345-50
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  • [Title] [Rare case of retroperitoneal malignant Schwannoma].
  • [Transliterated title] Caz rar de Schwannom malign retroperitoneal.
  • Malignant Schwannoma, recently renamed malignant peripheral nerve sheat tumor retroperitoneally localized, represents 0.01 of retroperitoneal tumours.
  • A 41-year old woman, without pathological record--and without cutaneous neurofibromatosis--hospitalized for increased volume of the abdomen, without symptomatology, is diagnosed after the imaging and biological tests--without CT and RMN--with retroperitoneal tumour.
  • The unusual size of tumour--6000 gr.
  • Two years after the operation, during which the patient was under chemotherapy, on a routine control we found a relapse at a distance from the initial tumour (primitive tumour ?
  • For the time being, after almost five years from the first operation, there are no clinical, biological and imaging changes.
  • CONCLUSIONS: the retroperitoneal space is quite enough for the development of large tumour masses, without symptomatology.
  • The present case combines most characteristics of retroperitoneal neoplasms: large or very large size, quasi-absent symptomatology, difficulty in preoperative diagnosis, surgical tactics and techniques--quite often, the total extirpation of tumour mass led to the sacrifice of other organs within the limits of a justified risk--and unforeseeable evolution with relapses having the same characteristics.
  • [MeSH-major] Neoplasm Recurrence, Local / therapy. Neurilemmoma / therapy. Retroperitoneal Neoplasms / therapy
  • [MeSH-minor] Adult. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Chemotherapy, Adjuvant. Female. Humans. Pancreatectomy. Radiotherapy, Adjuvant. Splenectomy. Treatment Outcome

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  • (PMID = 15675290.001).
  • [ISSN] 1221-9118
  • [Journal-full-title] Chirurgia (Bucharest, Romania : 1990)
  • [ISO-abbreviation] Chirurgia (Bucur)
  • [Language] hun
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Romania
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69. Etienne-Mastroianni B, Falchero L, Chalabreysse L, Loire R, Ranchère D, Souquet PJ, Cordier JF: Primary sarcomas of the lung: a clinicopathologic study of 12 cases. Lung Cancer; 2002 Dec;38(3):283-9
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  • PURPOSE: To study patients with primary sarcomas of the lung diagnosed in our pathology department in order to define their clinical characteristics, treatment, and prognosis.
  • PATIENTS: The study group consisted of 12 patients, with a mean age of 53 years.
  • Imaging findings consisted of: eight single peripheral opacities, three single parahilar opacities, and one lobar actelectasis.
  • The histologic diagnoses confirmed in all cases by detailed immunohistochemical study were leiomyosarcoma (7), monophasic synovial sarcoma (2), one case each of malignant peripheral nerve sheath tumor (MPNST), epithelioid sarcoma, and malignant fibrous histiocytoma.
  • Four patients received chemotherapy and two patients had radiation therapy postoperatively.
  • Treatment and prognosis do not differ from other soft tissue sarcomas.
  • [MeSH-minor] Adolescent. Adult. Aged. Aged, 80 and over. Female. Humans. Male. Middle Aged. Neoplasm Staging. Retrospective Studies. Survival Rate. Treatment Outcome

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  • (PMID = 12445750.001).
  • [ISSN] 0169-5002
  • [Journal-full-title] Lung cancer (Amsterdam, Netherlands)
  • [ISO-abbreviation] Lung Cancer
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Ireland
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70. Rückert RI, Fleige B, Rogalla P, Woodruff JM: Schwannoma with angiosarcoma. Report of a case and comparison with other types of nerve tumors with angiosarcoma. Cancer; 2000 Oct 1;89(7):1577-85
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  • [Title] Schwannoma with angiosarcoma. Report of a case and comparison with other types of nerve tumors with angiosarcoma.
  • BACKGROUND: Schwannoma with angiosarcomatous change is a rare tumor, the clinical characteristics of which have not been analyzed.
  • METHODS: A patient with schwannoma with angiosarcoma arising in the midneck and clinically mimicking a carotid body paraganglioma is described with a literature review of all previously reported cases and a comparison of their clinical features with those of schwannoma with conventional malignant transformation and cases of neurofibroma and malignant peripheral nerve sheath tumor (MPNST) with angiosarcoma.
  • Schwannoma with angiosarcoma affects older adults, mainly men.
  • Three tumors arose from the vagus nerve in the neck.
  • Three of the four angiosarcomas were epithelioid in type.
  • Treatment in all cases was surgical resection followed by radiation and chemotherapy in one case and by radiation alone in another.
  • One patient died with residual local angiosarcoma 5 months after the diagnosis.
  • CONCLUSIONS: Schwannoma with angiosarcoma should be included in the differential diagnosis of presumed carotid body paragangliomas.
  • Like angiosarcoma alone and schwannoma with conventional malignant transformation, but unlike cases of neurofibroma and MPNST with angiosarcoma, the patients are older adults, and there is a male prevalence.
  • Schwannoma with angiosarcoma is capable of local spread with a fatal outcome and of distant metastasis, but follow-up strongly suggests that these patients have a better prognosis than patients with neurofibroma or MPNST with angiosarcoma.
  • Recommended treatment is attempted complete surgical resection followed by radiation therapy and chemotherapy, if it can be tolerated by the patient.
  • [MeSH-major] Carotid Body Tumor / pathology. Hemangiosarcoma / pathology. Nerve Sheath Neoplasms / pathology. Neurilemmoma / pathology. Paraganglioma / pathology
  • [MeSH-minor] Cell Transformation, Neoplastic. Diagnosis, Differential. Humans. Male. Middle Aged. Prognosis

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  • (PMID = 11013374.001).
  • [ISSN] 0008-543X
  • [Journal-full-title] Cancer
  • [ISO-abbreviation] Cancer
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] UNITED STATES
  • [Number-of-references] 23
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71. Moharir M, London K, Howman-Giles R, North K: Utility of positron emission tomography for tumour surveillance in children with neurofibromatosis type 1. Eur J Nucl Med Mol Imaging; 2010 Jul;37(7):1309-17
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  • [Title] Utility of positron emission tomography for tumour surveillance in children with neurofibromatosis type 1.
  • PURPOSE: There is little consensus regarding optimal surveillance of optic pathway glioma (OPG) and plexiform neurofibroma (PNF) in childhood neurofibromatosis type 1 (NF1). (18)F-2-Fluoro-2-deoxy-D: -glucose (FDG) positron emission tomography and computed tomography (PET/CT) is employed in the surveillance of adult PNFs; but its utility has neither been specifically studied in children with PNFs nor in children with OPG.
  • FDG-avidity of tumours was semi-quantitatively analysed and graded by calculating the maximum standardised uptake value (SUV(max)) [grade 1: <3 (low), grade 2: >3-<4 (intermediate), grade 3: >4 (intense)].
  • FDG-avidity reduced from grade 3 to grade 1 in two symptomatic OPGs following chemotherapy and this was associated with clinical improvement.
  • PET/CT diagnosed symptomatic OPGs with a sensitivity of 0.625 [95% confidence interval (CI): 0.259-0.897] and specificity of 0.875 (95% CI: 0.466-0.993).
  • The two grade 3 PNFs were confirmed malignant peripheral nerve sheath tumours.
  • PET/CT diagnosed malignant transformation with a sensitivity of 1.0 (95% CI: 0.197-1.0) and specificity of 0.857 (95% CI: 0.561-0.974).
  • CONCLUSION: PET/CT may contribute useful information to the surveillance of OPG in childhood NF1-particularly to identify progressive, symptomatic tumours.
  • As in adults, PET/CT is useful for the detection of malignant transformation in PNFs in children with NF1.
  • [MeSH-major] Neurofibromatosis 1 / diagnostic imaging. Positron-Emission Tomography
  • [MeSH-minor] Adult. Child. Child, Preschool. Female. Fluorodeoxyglucose F18. Humans. Male. Neurofibroma, Plexiform / diagnostic imaging. Optic Nerve Glioma / diagnostic imaging. Retrospective Studies. Tomography, X-Ray Computed

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  • [Cites] Neurofibromatosis. 1988;1(3):172-8 [3152465.001]
  • [Cites] Neurology. 2004 Nov 23;63(10):1944-6 [15557519.001]
  • [Cites] Brain. 1988 Dec;111 ( Pt 6):1355-81 [3145091.001]
  • [Cites] J Neuropathol Exp Neurol. 2002 Mar;61(3):215-25; discussion 226-9 [11895036.001]
  • [Cites] J Child Neurol. 1997 Nov;12 (8):499-506 [9430315.001]
  • [Cites] J Neuropathol Exp Neurol. 2000 Sep;59(9):759-67 [11005256.001]
  • [Cites] Neurol Med Chir (Tokyo). 2006 Oct;46(10 ):500-3 [17062990.001]
  • [Cites] Ann Oncol. 2008 Feb;19(2):390-4 [17932395.001]
  • [Cites] Arch Dis Child. 1999 Sep;81(3):263-70 [10451403.001]
  • [Cites] Neurology. 2006 Oct 24;67(8):1509-12 [17060590.001]
  • [Cites] J Neurol Neurosurg Psychiatry. 2000 Mar;68(3):353-7 [10675220.001]
  • [Cites] Semin Nucl Med. 2007 Sep;37(5):391-8 [17707244.001]
  • [Cites] J Neurol Neurosurg Psychiatry. 1994 Dec;57(12):1479-83 [7798976.001]
  • [Cites] Radiology. 1993 Oct;189(1):221-5 [8372197.001]
  • [Cites] Eur J Nucl Med Mol Imaging. 2009 May;36(5):751-7 [19142634.001]
  • [Cites] Eur J Nucl Med Mol Imaging. 2006 Apr;33(4):428-32 [16404595.001]
  • [Cites] Mol Imaging Biol. 2007 May-Jun;9(3):106-9 [17318667.001]
  • [Cites] Nucl Med Commun. 2008 May;29(5):418-24 [18391724.001]
  • [Cites] Ann Neurol. 2007 Mar;61(3):189-98 [17387725.001]
  • [Cites] Neurology. 2002 May 28;58(10):1461-70 [12041525.001]
  • (PMID = 20179923.001).
  • [ISSN] 1619-7089
  • [Journal-full-title] European journal of nuclear medicine and molecular imaging
  • [ISO-abbreviation] Eur. J. Nucl. Med. Mol. Imaging
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0Z5B2CJX4D / Fluorodeoxyglucose F18
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72. Zhang PJ, Brooks JS, Goldblum JR, Yoder B, Seethala R, Pawel B, Gorman JH, Gorman RC, Huang JH, Acker M, Narula N: Primary cardiac sarcomas: a clinicopathologic analysis of a series with follow-up information in 17 patients and emphasis on long-term survival. Hum Pathol; 2008 Sep;39(9):1385-95
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  • [Title] Primary cardiac sarcomas: a clinicopathologic analysis of a series with follow-up information in 17 patients and emphasis on long-term survival.
  • Although cardiac sarcomas are rare in comparison to their soft tissue counterparts, they are the second most common type of primary cardiac neoplasm.
  • There were 6 angiosarcomas, 6 myxofibrosarcomas, 3 malignant peripheral nerve sheath tumors, 3 leiomyosarcomas, 2 synovial sarcomas, 1 epithelioid hemangioendothelioma, 1 chondrosarcoma, 1 osteosarcoma, and 4 poorly differentiated sarcomas.
  • Six high-grade and 1 low-grade tumors were also treated with adjuvant chemotherapy and/or radiation.
  • In 17 patients with follow-up data, 6 of 12 patients with high-grade tumor died (4 within 5 days of the initial surgery, 1 in 21 months, and 1 in 131 months), and 1 patient with moderate-grade tumor and all 4 patients with low-grade tumor were alive without evidence of disease at the end of follow-up.
  • Tumor grade appeared to be prognostically important in cardiac sarcoma.
  • [MeSH-minor] Adolescent. Adult. Aged. Child. Child, Preschool. Combined Modality Therapy. Female. Follow-Up Studies. Humans. Infant. Male. Middle Aged. Survival Analysis

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  • [Cites] Ann Thorac Surg. 2000 Jun;69(6):1949-51 [10892961.001]
  • [Cites] Z Kardiol. 2002 Apr;91(4):352-6 [12063709.001]
  • [Cites] J Heart Lung Transplant. 2002 Oct;21(10):1135-9 [12398881.001]
  • [Cites] Rev Esp Cardiol. 2003 Apr;56(4):408-11 [12689577.001]
  • [Cites] Chest. 2003 May;123(5):1766-8 [12740300.001]
  • [Cites] Thorac Cardiovasc Surg. 2004 Apr;52(2):77-81 [15103579.001]
  • [Cites] Ann Surg. 1980 Feb;191(2):127-38 [7362282.001]
  • [Cites] Hum Pathol. 1982 Jul;13(7):640-5 [7084941.001]
  • [Cites] Cancer. 1984 Feb 1;53(3):530-41 [6692258.001]
  • [Cites] Arch Pathol Lab Med. 1985 Oct;109(10):943-5 [3840005.001]
  • [Cites] Chest. 1987 Jul;92(1):177-9 [3595230.001]
  • [Cites] Chest. 1987 Nov;92(5):860-2 [3665601.001]
  • [Cites] Thorac Cardiovasc Surg. 1990 Aug;38 Suppl 2:183-91 [2237900.001]
  • [Cites] Thorac Cardiovasc Surg. 1990 Aug;38 Suppl 2:192-5 [2237901.001]
  • [Cites] Ann Thorac Surg. 1991 Jun;51(6):906-10 [2039319.001]
  • [Cites] Ann Thorac Surg. 1991 Jun;51(6):999-1001 [2039335.001]
  • [Cites] Ann Thorac Surg. 1991 Oct;52(4):886-95 [1929651.001]
  • [Cites] Cancer. 1992 Jan 15;69(2):387-95 [1728367.001]
  • [Cites] Am Heart J. 1992 Jan;123(1):232-4 [1729836.001]
  • [Cites] Aust N Z J Med. 1991 Dec;21(6):881-3 [1818549.001]
  • [Cites] Scand J Thorac Cardiovasc Surg. 1992;26(3):233-6 [1287840.001]
  • [Cites] J Cardiovasc Surg (Torino). 1993 Dec;34(6):529-33 [8300722.001]
  • [Cites] Semin Surg Oncol. 1994 Sep-Oct;10(5):374-82 [7997732.001]
  • [Cites] J Heart Lung Transplant. 1995 Mar-Apr;14(2):382-6 [7779860.001]
  • [Cites] Hum Mol Genet. 1995 Jun;4(6):1097-9 [7655467.001]
  • [Cites] Histopathology. 1997 Apr;30(4):349-52 [9147083.001]
  • [Cites] Jpn Circ J. 1997 Sep;61(9):795-7 [9293411.001]
  • [Cites] Ann Diagn Pathol. 1998 Jun;2(3):167-72 [9845736.001]
  • [Cites] Br J Cancer. 1998 Dec;78(12):1624-8 [9862574.001]
  • [Cites] J Surg Oncol. 1999 Mar;70(3):194-8 [10102352.001]
  • [Cites] Histopathology. 1999 Apr;34(4):295-304 [10231396.001]
  • [Cites] Eur J Cardiothorac Surg. 2006 Jun;29(6):925-32 [16675225.001]
  • [Cites] J Thorac Oncol. 2006 Feb;1(2):188-9 [17409853.001]
  • [Cites] Gen Thorac Cardiovasc Surg. 2007 Jan;55(1):19-22 [17444167.001]
  • [Cites] Oncologist. 2007 Sep;12(9):1134-42 [17914083.001]
  • (PMID = 18602663.001).
  • [ISSN] 1532-8392
  • [Journal-full-title] Human pathology
  • [ISO-abbreviation] Hum. Pathol.
  • [Language] eng
  • [Grant] United States / NHLBI NIH HHS / HL / R01 HL063954
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Other-IDs] NLM/ NIHMS522251; NLM/ PMC4081532
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73. Yoo NJ, Kim HS, Kim SY, Park WS, Park CH, Jeon H, Jung ES, Lee JY, Lee SH: Immunohistochemical analysis of Smac/DIABLO expression in human carcinomas and sarcomas. APMIS; 2003 Mar;111(3):382-8
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  • Alteration of apoptosis is essential for cancer development, and cancer cell death by radiation and chemotherapy is largely dependent upon apoptosis.
  • In this study, archival tissues of 100 carcinomas and 50 sarcomas from various origins were analyzed by immunohistochemistry for the expression of Smac/DIABLO.
  • Smac/DIABLO is expressed in 11 of 50 (22%) sarcomas, including 2 of 8 malignant schwannomas, 5 of 11 rhabdomyosarcomas, 2 of 7 malignant fibrous histiocytomas, 1 of 6 leiomyosarcomas, 0 of 8 angiosarcomas, 0 of 8 liposarcomas, and 1 of 2 Ewing's sarcomas.
  • These data demonstrated that Smac/DIABLO expression levels vary depending on the individual cancer types.

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  • (PMID = 12752217.001).
  • [ISSN] 0903-4641
  • [Journal-full-title] APMIS : acta pathologica, microbiologica, et immunologica Scandinavica
  • [ISO-abbreviation] APMIS
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Denmark
  • [Chemical-registry-number] 0 / Carrier Proteins; 0 / DIABLO protein, human; 0 / Intracellular Signaling Peptides and Proteins; 0 / Mitochondrial Proteins
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74. Oda Y, Saito T, Tateishi N, Ohishi Y, Tamiya S, Yamamoto H, Yokoyama R, Uchiumi T, Iwamoto Y, Kuwano M, Tsuneyoshi M: ATP-binding cassette superfamily transporter gene expression in human soft tissue sarcomas. Int J Cancer; 2005 May 10;114(6):854-62
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  • [Title] ATP-binding cassette superfamily transporter gene expression in human soft tissue sarcomas.
  • The phenomenon of multidrug resistance (MDR) in various malignant neoplasms has been reported as being caused by one or multiple expressions of ATP-binding cassette (ABC) superfamily protein, including P-glycoprotein/multidrug resistance (MDR) 1 and the MDR protein (MRP) family.
  • However, their expression levels and distribution within soft tissue sarcomas remain controversial.
  • In 86 cases of surgically resected soft tissue sarcoma, intrinsic mRNA levels of MDR1, MRP1, MRP2 and MRP3 were assessed using a quantitative reverse transcriptase-PCR (RT-PCR) method.
  • Moreover, immunohistochemical protein expressions of P-glycoprotein (P-gp), MRP1, MRP2, MRP3 and p53 protein were evaluated in concordant paraffin-embedded material.
  • Among the various histologic types, malignant peripheral nerve sheath tumor (MPNST) showed significantly high levels of MDR1 (p=0.017) and MRP3 (p=0.0384) mRNA expression, compared to the other tumor types.
  • P-gp expression was significantly correlated with large tumor size (> or =5 cm, p=0.041) and high AJCC stage (stages III and IV) (p=0.0365).
  • Our results suggest that MDR1/P-gp expression may have an important role to play in tumor progression in the cases of soft tissue sarcoma, and p53 may be one of the active regulators of the MDR1 transcript.
  • In addition, the high levels of both MDR1 and MRP3 mRNA expression in MPNST may help to explain the poor response of this tumor to anticancer-drugs.
  • [MeSH-major] ATP-Binding Cassette Transporters / biosynthesis. ATP-Binding Cassette Transporters / genetics. Drug Resistance, Multiple. Gene Expression Regulation, Neoplastic. Genes, p53. Sarcoma / drug therapy. Sarcoma / genetics
  • [MeSH-minor] Adolescent. Adult. Disease Progression. Drug Resistance, Neoplasm / genetics. Female. Gene Expression Profiling. Humans. Immunohistochemistry. Male. Reverse Transcriptase Polymerase Chain Reaction

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  • (PMID = 15609299.001).
  • [ISSN] 0020-7136
  • [Journal-full-title] International journal of cancer
  • [ISO-abbreviation] Int. J. Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / ATP-Binding Cassette Transporters
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75. Malerba M, Garofalo A: [A rare case of nerve-sheath sarcoma with rhabdomyoblastic differentiation (malignant triton tumor)]. Tumori; 2003 Jul-Aug;89(4 Suppl):246-50

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  • [Title] [A rare case of nerve-sheath sarcoma with rhabdomyoblastic differentiation (malignant triton tumor)].
  • [Transliterated title] Un raro caso di sarcoma delle guaine nervose a differenziazione rabdomioblastica (malignant Triton tumor).
  • Malignant peripheral nerve sheath tumors (MPNST) are spindle-cell sarcomas that appear in a setting of neurofibroma or schwannoma or are associated with peripheral nerves or demonstrate nerve sheath differentiation.
  • Malignant triton tumor (MTT) is a subtype of MPNST that also contain tissue with skeletal muscle differentiation (embryonal, plemorphic and botryoid rhabdomyosarcoma).
  • The estimated incidence of MPNSTs in patients with NF1 is 2-5% compared with 0.0001% in the general population and approximately 69% of the reported cases of MTT are associated with von Recklinghausen disease.
  • Seventeen months before, when the patient underwent surgery at the same Department for both a left-sided paravertebral inferior mediastinal neurofibroma and a right-sided axillary neurofibroma, diagnosis of von Recklinghausen disease (NF1) was made, according to the criteria established by the NIH Consensus Development.
  • A xifopubic laparotomy was performed: the tumor appeared to be localized, well-capsulated and strictly associated to the lumbar and sacral nervous radicles (L4, L5, S1) without evidence of invasion.
  • The tumor was completely resected with sparing of the psoas muscle and the lumbar plexus through a subperineural dissection technique.
  • Postoperative pathologic findings showed evidence for a trition tumor.
  • The popliteal mass was resected too and resulted to be a neurofibroma just like the tumors resected 17 months before when diagnosis of von Recklinghausen disease was made.
  • Sarcoma arising in anatomic site other than extremity and superficial trunk are often more difficult to control because of anatomic constraints, delayed disease presentation, proximity to neurovascular and osseous structures and toxicity for normal adjacent tissues that limits the use of adequate radiation doses.
  • Indeed, the anatomic site is an important prognostic factor in STS and the prognosis for retroperitoneal tumors is considerably worse than for extremity tumors.
  • In contrast to the benefit most patients with high grade soft tissue sarcomas of the extremities receive from adjuvant radiation and chemotherapy, these modalities have been of little value for retroperitoneal tumors.
  • Current chemotherapy for retroperitoneal sarcomas is ineffective.
  • Local adjuvant therapy such as intraperitoneal chemotherapy or experimental immunotherapy seems to be attractive in theory, but needs further investigations through prospective randomized multicentric trials.
  • In conclusion, to date aggressive surgical management remains the most effective modality for selected primary and recurrent retroperitoneal soft tissue sarcomas including MPNSTs and the subtype MTT.
  • Patients with incomplete resection and other risk factors such as younger age and high grade tumors may be suitable candidates for investigational adjuvant therapy.
  • [MeSH-major] Neurilemmoma / pathology. Neurofibromatosis 1 / pathology. Peripheral Nervous System Neoplasms / pathology. Retroperitoneal Neoplasms / pathology
  • [MeSH-minor] Adult. Axilla. Cell Differentiation. Humans. Knee. Male. Mediastinal Neoplasms / pathology. Mediastinal Neoplasms / surgery. Muscles / pathology. Soft Tissue Neoplasms / pathology. Soft Tissue Neoplasms / surgery. Spinal Nerve Roots / pathology. Spinal Nerve Roots / surgery

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  • (PMID = 12903608.001).
  • [ISSN] 0300-8916
  • [Journal-full-title] Tumori
  • [ISO-abbreviation] Tumori
  • [Language] ita
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] United States
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76. Karatzoglou P, Karagiannidis A, Kountouras J, Christofiridis CV, Karavalaki M, Zavos C, Gavalas E, Patsiaoura K, Vougiouklis N: Von Recklinghausen's disease associated with malignant peripheral nerve sheath thmor presenting with constipation and urinary retention: a case report and review of the literature. Anticancer Res; 2008 Sep-Oct;28(5B):3107-13
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  • [Title] Von Recklinghausen's disease associated with malignant peripheral nerve sheath thmor presenting with constipation and urinary retention: a case report and review of the literature.
  • A malignant peripheral nerve sheath tumor (MPNST) is a rare neoplasm arising from peripheral nerve sheaths.
  • We herein report the first case of MPNST originating from the left gluteal muscle region, diffusely extending into the adjacent small pelvis and perineum.
  • The patient had a family history of neurofibromatosis type 1.
  • A computed tomography scan revealed a giant tumor which displaced the bladder and segments of the intestine.
  • The histopathological diagnosis was MPNST.
  • The patient declined chemotherapy and radiation therapy and died 2 months later.
  • [MeSH-major] Constipation / etiology. Nerve Sheath Neoplasms / complications. Neurofibromatosis 1 / complications. Urinary Retention / etiology

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  • (PMID = 19031965.001).
  • [ISSN] 0250-7005
  • [Journal-full-title] Anticancer research
  • [ISO-abbreviation] Anticancer Res.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Greece
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77. Hirokawa Y, Nheu T, Grimm K, Mautner V, Maeda S, Yoshida M, Komiyama K, Maruta H: Sichuan pepper extracts block the PAK1/cyclin D1 pathway and the growth of NF1-deficient cancer xenograft in mice. Cancer Biol Ther; 2006 Mar;5(3):305-9
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  • Thus, we have been exploring an alternative which is already in the market, and therefore immediately useful for the treatment of those desperate cancer/NF patients.
  • The Chinese pepper extract selectively inhibits the growth of NF1-deficient malignant peripheral nerve sheath tumor (MPNST) cells, without affecting the growth of normal fibroblasts, and suppresses the growth of NF1-deficient human breast cancer (MDA-MB-231) xenograft in mice.
  • Our data suggest that these peppercorn extracts would be potentially useful for the treatment of PAK1-dependent NF such as MPNST, in addition to a variety of PAK1-dependent cancers including breast cancers.
  • [MeSH-major] Antimitotic Agents / pharmacology. Cyclin D1 / metabolism. Neurofibromatosis 1 / drug therapy. Plant Extracts / pharmacology. Protein-Serine-Threonine Kinases / antagonists & inhibitors. Zanthoxylum / chemistry
  • [MeSH-minor] Animals. Cell Line, Tumor. Cell Proliferation / drug effects. Down-Regulation. Enzyme Inhibitors / pharmacology. Enzyme Inhibitors / therapeutic use. Female. Humans. Mice. Mice, Inbred BALB C. Neoplasm Transplantation. Nerve Sheath Neoplasms / pathology. Neurofibromin 1 / genetics. Phytotherapy. Signal Transduction. Transplantation, Heterologous. p21-Activated Kinases


78. Antoniello L, Cecchetto G, Carli M, Dall'Igna P, Bisogno G, Lo Piccolo R, Gigante C, Zanetti I, Guglielmi M: [Role of mutilating surgery in the treatment of non-chemosensitive pediatric soft tissue sarcomas. Experience of the Italian Cooperative Group Studies RMS-79 and RMS-88]. Pediatr Med Chir; 2003 Jul-Aug;25(4):255-60
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  • [Title] [Role of mutilating surgery in the treatment of non-chemosensitive pediatric soft tissue sarcomas. Experience of the Italian Cooperative Group Studies RMS-79 and RMS-88].
  • [Transliterated title] Ruolo della chirurgia mutilante nel trattamento dei sarcomi delle parti molli non chemiosensibili in età pediatrica. Esperienza degli studi Cooperativi Italiani RMS-79 ed RMS-88.
  • Aim of the study was to evaluate the role of mutilating surgery in the patients with non chemosensitive soft tissue sarcomas (STS) registered in the Italian Studies.
  • HISTOLOGY: fibrosarcoma 29, Malignant Perpheral Nerve Sheath Tumors (MPNST) 40, malignant fibrous histiocytoma 5, hemangiopericytoma 6, leiomyosarcoma 4, others 20, STS nos 10.
  • Twelve out of 114 pts (7%), 5/33 (14%) in the first study and 7/81 (8%) in the second, underwent mutilating surgery: 8 pts (of whom 3 were < 2 y of age) had a fibrosarcoma and 4 a MPNST.
  • The mutilating procedure was carried out at diagnosis in 6 cases (4 in RMS-79 and 2 in RMS-and 88) and achieved radicality in 5/6 cases.
  • It was performed after ineffective chemotherapy (CT) in 5 pts (1 in RMS-79 and 4 in RMS-88).
  • OUTCOME: At present 6/12 pts, 5 with fibrosarcoma and 1 with MPNST, are alive with no evidence of disease (NED), 4 of the first and 2 of the second study.
  • Of the 5 Gr. I patients, 4 are alive (NED) and 1 died of 2nd tumor; 1 Gr.
  • III pts 1 is alive NED and 4 died (3 of metastatic spread and 1 of 2nd tumor); the pt amputated after repeated local relapses (Gr.
  • CONCLUSIONS: In the RMS-79 study the mutilations were frequent and were performed at diagnosis in several cases; this trend decreased in the 2nd study in which chemotherapy was attempted in most of the patients.
  • Only fibrosarcomas and MPNST probably requires a more aggressive surgical behaviour.
  • [MeSH-major] Sarcoma / surgery. Soft Tissue Neoplasms / surgery

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  • (PMID = 15070267.001).
  • [ISSN] 0391-5387
  • [Journal-full-title] La Pediatria medica e chirurgica : Medical and surgical pediatrics
  • [ISO-abbreviation] Pediatr Med Chir
  • [Language] ita
  • [Publication-type] English Abstract; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Italy
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79. Jacobs S, Fox E, Krailo M, Hartley G, Navid F, Wexler L, Blaney SM, Goodwin A, Goodspeed W, Balis FM, Adamson PC, Widemann BC: Phase II trial of ixabepilone administered daily for five days in children and young adults with refractory solid tumors: a report from the children's oncology group. Clin Cancer Res; 2010 Jan 15;16(2):750-4
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  • [Title] Phase II trial of ixabepilone administered daily for five days in children and young adults with refractory solid tumors: a report from the children's oncology group.
  • PURPOSE: Ixabepilone is a microtubule-stabilizing agent with activity in adult solid tumors and in pediatric tumor xenograft models that are resistant to paclitaxel.
  • This study aimed to determine the response rate to ixabepilone in six solid tumor strata in children and young adults.
  • EXPERIMENTAL DESIGN: We conducted a phase II trial of ixabepilone (8 mg/m(2)/dose for 5 days every 21 days) using a two-stage design in taxane-naïve children and young adults with treatment-refractory, measurable rhabdomyosarcoma, Ewing sarcoma family tumors, osteosarcoma, synovial sarcoma, or malignant peripheral nerve sheath tumor, neuroblastoma, and Wilms tumor.
  • No partial or complete responses (response evaluation criteria in solid tumors) were observed.
  • Seven patients received >or=3 cycles, and two had prolonged stable disease (Wilms' tumor, 38 cycles; synovial sarcoma, 8 cycles).
  • CONCLUSIONS: Ixabepilone at 8 mg/m(2)/dose daily for 5 days was tolerable in children and adolescents, but did not show evidence of clinical activity in the childhood solid tumors studied.

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  • [Cites] J Natl Cancer Inst. 2000 Feb 2;92(3):205-16 [10655437.001]
  • [Cites] Clin Cancer Res. 2005 Oct 1;11(19 Pt 1):6950-8 [16203787.001]
  • [Cites] J Pediatr Hematol Oncol. 2001 Jun-Jul;23(5):277-81 [11464982.001]
  • [Cites] J Clin Oncol. 2003 May 1;21(9):1866-73 [12721265.001]
  • [Cites] Clin Cancer Res. 2004 Feb 15;10(4):1289-98 [14977827.001]
  • [Cites] J Clin Oncol. 2004 May 15;22(10):2015-25 [15143095.001]
  • [Cites] J Clin Oncol. 1993 Dec;11(12):2324-9 [7902425.001]
  • [Cites] J Clin Oncol. 1997 Apr;15(4):1538-43 [9193350.001]
  • [Cites] Clin Cancer Res. 1999 Apr;5(4):733-7 [10213206.001]
  • [Cites] J Clin Oncol. 2005 Apr 20;23(12):2726-34 [15837987.001]
  • [Cites] Cancer. 2006 Apr 15;106(8):1821-8 [16532433.001]
  • [Cites] J Clin Oncol. 2007 Aug 10;25(23):3421-7 [17606971.001]
  • [Cites] J Clin Oncol. 2007 Aug 10;25(23):3415-20 [17606972.001]
  • [Cites] J Clin Oncol. 2007 Aug 10;25(23):3399-406 [17606975.001]
  • [Cites] Ann Oncol. 2007 Sep;18(9):1548-53 [17761711.001]
  • [Cites] Pediatr Blood Cancer. 2007 Dec;49(7):928-40 [17066459.001]
  • [Cites] Oncologist. 2008 Dec;13(12):1207-23 [19088324.001]
  • [Cites] J Clin Oncol. 2009 Feb 1;27(4):550-6 [19075272.001]
  • [Cites] Clin Cancer Res. 2001 May;7(5):1429-37 [11350914.001]
  • (PMID = 20068084.001).
  • [ISSN] 1078-0432
  • [Journal-full-title] Clinical cancer research : an official journal of the American Association for Cancer Research
  • [ISO-abbreviation] Clin. Cancer Res.
  • [Language] ENG
  • [Grant] United States / NCI NIH HHS / CA / U10 CA098413; United States / NCI NIH HHS / CA / U10 CA 98543; United States / Intramural NIH HHS / / ; United States / NCI NIH HHS / CA / U10 CA098543-08; None / None / / U10 CA098543-08; United States / NCI NIH HHS / CA / U10 CA098543; United States / NCI NIH HHS / CA / U10 CA098413-08; None / None / / U10 CA098413-08
  • [Publication-type] Clinical Trial, Phase II; Journal Article; Research Support, N.I.H., Extramural; Research Support, N.I.H., Intramural
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Epothilones; K27005NP0A / ixabepilone
  • [Other-IDs] NLM/ NIHMS160304; NLM/ PMC3086796
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80. Wang KF, Wu B, Zhang Y: [Adult prostate sarcoma: a report of 6 cases with clinical analysis]. Zhonghua Nan Ke Xue; 2007 Jul;13(7):617-9
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  • OBJECTIVE: To investigate the diagnosis, treatment and prognosis of sarcoma of the adult prostate.
  • METHODS: We reported 6 cases of sarcoma of the adult prostate, of which 3 were leiomyosarcoma, 2 rhabdomyosarcoma and 1 malignant neurilemoma, 2 at Ghavimi Stage II, 3 at Stage III and 1 at Stage IV.
  • Five of them received operation, radiotherapy and / or chemotherapy and 1 underwent cystostomy only.
  • RESULTS: Immunohistochemical dyeing showed vimentin to be positive while PSA and PAP negative in all the 6 cases, actin (HHF35) positive in the cases of leiomyosarcoma and rhabdomyosarcoma, and S-100 and lysozyme positive in the case of malignant neurilemoma.
  • Early diagnosis and radical surgical resection may offer the best chance of survival, but with poor prognosis.
  • [MeSH-major] Prostatic Neoplasms / therapy. Sarcoma / therapy
  • [MeSH-minor] Actins / analysis. Adolescent. Adult. Combined Modality Therapy. Cystostomy. Drug Therapy. Fatal Outcome. Humans. Immunohistochemistry. Male. Neoplasm Staging. Prognosis. Radiotherapy. S100 Proteins / analysis. Vimentin / analysis

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  • (PMID = 17725305.001).
  • [ISSN] 1009-3591
  • [Journal-full-title] Zhonghua nan ke xue = National journal of andrology
  • [ISO-abbreviation] Zhonghua Nan Ke Xue
  • [Language] chi
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] China
  • [Chemical-registry-number] 0 / Actins; 0 / S100 Proteins; 0 / Vimentin
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81. Häcker FM, von Schweinitz D, Gambazzi F: The relevance of surgical therapy for bilateral and/or multiple pulmonary metastases in children. Eur J Pediatr Surg; 2007 Apr;17(2):84-9
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  • [Title] The relevance of surgical therapy for bilateral and/or multiple pulmonary metastases in children.
  • PURPOSE: Pulmonary surgery is frequently used for the treatment of metastases in children with various malignant diseases.
  • The benefit of an aggressive surgical treatment in children with bilateral and/or multiple pulmonary metastases is still discussed controversially.
  • The primary malignancies were osteosarcoma (n = 4), hepatoblastoma (n = 3), malignant peripheral nerve sheath tumor (n = 1), adrenocortical carcinoma (n = 1) and alveolar rhabdomyosarcoma (n = 1).
  • Preoperative induction chemotherapy with tumor regression and a subsequent decrease in the size and number of pulmonary metastases was mandatory for the surgery of metastases.
  • RESULTS: Standardized bilateral thoracotomy was performed in 4 patients in 1 operation (in 1 patient combined with a hemihepatectomy), and in 3 patients, in 2 operations on different days.
  • CONCLUSION: Complete surgical resection of pulmonary metastases after response to induction chemotherapy may increase survival in carefully selected children, even in cases with multiple and recurrent metastatic disease.

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  • (PMID = 17503299.001).
  • [ISSN] 0939-7248
  • [Journal-full-title] European journal of pediatric surgery : official journal of Austrian Association of Pediatric Surgery ... [et al] = Zeitschrift für Kinderchirurgie
  • [ISO-abbreviation] Eur J Pediatr Surg
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Germany
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82. Li Q, Gao C, Juzi JT, Hao X: Analysis of 82 cases of retroperitoneal schwannoma. ANZ J Surg; 2007 Apr;77(4):237-40
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  • [Title] Analysis of 82 cases of retroperitoneal schwannoma.
  • BACKGROUND: The aim of the study was to improve the diagnosis and treatment of retroperitoneal schwannoma by analysing clinical manifestations and postoperative course of this rare disease.
  • METHODS: A retrospective analysis of 82 patients with retroperitoneal schwannoma between January 1951 and September 2004 was carried out.
  • The interval between clinical manifestation and diagnosis ranged from 10 days to 2 years.
  • Only in 13 patients (15.9%) a correct preoperative diagnosis was made by either ultrasound-guided biopsy, computed tomography scanning or magnetic resonance imaging.
  • All patients received operative therapy.
  • Two patients (2.4%) had multiple schwannomas and two others had a simultaneous malignancy (adenocarcinoma of the ascending colon and squamous-cell carcinoma of the lung, respectively).
  • Most of the retroperitoneal schwannomas were close to the spine.
  • Pathological results showed 81 (98.8%) were benign schwannoma and 1 (1.2%) was a malignant one.
  • The tumour size ranged from 3 to 22 cm.
  • One benign schwannoma recurred 3 years after the operation.
  • The patient with malignant schwannoma died 18 months after the operation because of metastasized disease.
  • CONCLUSION: Most of the retroperitoneal schwannomas are benign.
  • It is difficult to make an accurate preoperative diagnosis.
  • However, with the preoperative assessment of ultrasound-guided fine-needle aspiration, computed tomography and magnetic resonance imaging, the accuracy of diagnosis could definitely be improved.
  • Treatment depends solely on surgery.
  • Malignant schwannomas are insensitive to chemotherapy and radiation, resulting in poor prognosis.
  • [MeSH-major] Neurilemmoma / diagnosis. Neurilemmoma / surgery. Retroperitoneal Neoplasms / diagnosis. Retroperitoneal Neoplasms / surgery
  • [MeSH-minor] Adolescent. Adult. Aged. Child. Child, Preschool. Diagnostic Imaging. Female. Humans. Infant. Male. Middle Aged. Neoplasm Recurrence, Local. Prognosis. Retrospective Studies. Survival Analysis. Treatment Outcome

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  • (PMID = 17388825.001).
  • [ISSN] 1445-1433
  • [Journal-full-title] ANZ journal of surgery
  • [ISO-abbreviation] ANZ J Surg
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Australia
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83. Landy H, Feun L, Markoe A, Patchen S, Bruce J, Marcus J, Levi A: Extended remission of a recurrent median nerve malignant peripheral nerve sheath tumor after multimodal treatment. Case report. J Neurosurg; 2005 Oct;103(4):760-3
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  • [Title] Extended remission of a recurrent median nerve malignant peripheral nerve sheath tumor after multimodal treatment. Case report.
  • Malignant peripheral nerve sheath tumors (MPNSTs) are difficult to control despite aggressive treatment.
  • In this report the authors describe the treatment and follow-up review of a patient with neurofibromatosis Type I who harbored a recurrent median nerve MPNST.
  • The man underwent preoperative intraarterial and intravenous chemotherapy followed by additional surgery for gross-total removal and postoperative radiotherapy.
  • Two courses of preoperative intraarterial cisplatin and intravenous Adriamycin produced significant tumor shrinkage.
  • Gross-total removal of the remaining tumor without amputation of the arm was followed by fractionated radiotherapy (total minimum tumor dose 6485 cGy, maximal dose 6575 cGy).
  • The patient is alive 9.5 years after treatment without evidence of tumor recurrence and with only focal median nerve functional deficits.
  • A review of the patient's treatment is warranted to provide a description of a regimen that may be useful in the treatment of similar patients in the future.
  • [MeSH-major] Neoplasm Recurrence, Local / surgery. Nerve Sheath Neoplasms / surgery. Neurofibromatosis 1 / surgery
  • [MeSH-minor] Adult. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Cisplatin / administration & dosage. Combined Modality Therapy. Doxorubicin / administration & dosage. Humans. Male. Treatment Outcome

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  • (PMID = 16266062.001).
  • [ISSN] 0022-3085
  • [Journal-full-title] Journal of neurosurgery
  • [ISO-abbreviation] J. Neurosurg.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 80168379AG / Doxorubicin; Q20Q21Q62J / Cisplatin
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84. Pirayesh A, Chee Y, Helliwell TR, Hershman MJ, Leinster SJ, Fordham MV, Poston GJ: The management of retroperitoneal soft tissue sarcoma: a single institution experience with a review of the literature. Eur J Surg Oncol; 2001 Aug;27(5):491-7
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  • [Title] The management of retroperitoneal soft tissue sarcoma: a single institution experience with a review of the literature.
  • AIM: Ten percent of soft tissue sarcomas (STS) arise in the retroperitoneal tissues.
  • The prognosis for patients with retroperitoneal sarcoma is poor with a 5-year survival rate between 12% and 70%.
  • Stage at presentation, high histological grade, unresectable primary tumour and incomplete resection are associated with a less favourable outcome.
  • Patient, tumour and treatment variables were analysed including use of adjuvant therapy and survival status.
  • Thirteen patients presented with tumours larger than 10 cm.
  • The tumours were seven liposarcomas, six leiomyosarcomas, three malignant fibrous histiocytomas, two rhabdomyosarcomas, two malignant schwannomas and two undifferentiated sarcomas.
  • Six primary tumours were completely excised, five patients received radiotherapy and five received chemotherapy.
  • Five patients received radiotherapy and five received chemotherapy.
  • The median survival for patients with primary tumours was 36 months, and 5-year survival was 44%.
  • Adjuvant therapy was not associated with higher survival rates.
  • Adjuvant radiotherapy and chemotherapy do not appear to be any proven benefit and the single most important prognostic factor is aggressive successful en bloc resection of the primary tumour.
  • [MeSH-major] Retroperitoneal Neoplasms / surgery. Soft Tissue Neoplasms / surgery
  • [MeSH-minor] Adult. Aged. Aged, 80 and over. Female. Follow-Up Studies. Histiocytoma, Benign Fibrous / surgery. Humans. Male. Middle Aged. Neurilemmoma / surgery. Prognosis. Retrospective Studies. Sarcoma / surgery. Survival Analysis. Tomography, X-Ray Computed. Treatment Outcome

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  • [Copyright] Copyright Harcourt Publishers Limited.
  • (PMID = 11504522.001).
  • [ISSN] 0748-7983
  • [Journal-full-title] European journal of surgical oncology : the journal of the European Society of Surgical Oncology and the British Association of Surgical Oncology
  • [ISO-abbreviation] Eur J Surg Oncol
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] England
  • [Number-of-references] 53
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85. Banerjee S, Byrd JN, Gianino SM, Harpstrite SE, Rodriguez FJ, Tuskan RG, Reilly KM, Piwnica-Worms DR, Gutmann DH: The neurofibromatosis type 1 tumor suppressor controls cell growth by regulating signal transducer and activator of transcription-3 activity in vitro and in vivo. Cancer Res; 2010 Feb 15;70(4):1356-66
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  • [Title] The neurofibromatosis type 1 tumor suppressor controls cell growth by regulating signal transducer and activator of transcription-3 activity in vitro and in vivo.
  • Neurofibromatosis type 1 (NF1) is a common cancer predisposition syndrome in which affected individuals develop benign and malignant nerve tumors.
  • The NF1 gene product neurofibromin negatively regulates Ras and mammalian target of rapamycin (mTOR) signaling, prompting clinical trials to evaluate the ability of Ras and mTOR pathway inhibitors to arrest NF1-associated tumor growth.
  • To discover other downstream targets of neurofibromin, we performed an unbiased cell-based high-throughput chemical library screen using NF1-deficient malignant peripheral nerve sheath tumor (MPNST) cells.
  • We further showed that signal transducer and activator of transcription-3 (STAT3), the target of cucurbitacin-I inhibition, was hyperactivated in NF1-deficient primary astrocytes and neural stem cells, mouse glioma cells, and human MPNST cells through Ser(727) phosphorylation, leading to increased cyclin D1 expression.
  • Finally, cucurbitacin-I inhibited the growth of NF1-deficient MPNST cells in vivo.
  • In summary, we used a chemical genetics approach to reveal STAT3 as a novel neurofibromin/mTOR pathway signaling molecule, define its action and regulation, and establish STAT3 as a tractable target for future NF1-associated cancer therapy studies.
  • [MeSH-minor] Animals. Animals, Newborn. Cells, Cultured. Chromones / pharmacology. Enzyme Inhibitors / pharmacology. Gene Expression Regulation / drug effects. Genes, Tumor Suppressor / physiology. Humans. Intracellular Signaling Peptides and Proteins / metabolism. Male. Mice. Mice, Nude. Mice, Transgenic. Morpholines / pharmacology. Protein-Serine-Threonine Kinases / metabolism. Signal Transduction / drug effects. Signal Transduction / genetics. TOR Serine-Threonine Kinases. Triterpenes / pharmacology. Xenograft Model Antitumor Assays


86. Ferraresi V, Ciccarese M, Cercato MC, Nuzzo C, Zeuli M, Di Filippo F, Giannarelli D, Cognetti F: Gemcitabine at fixed dose-rate in patients with advanced soft-tissue sarcomas: a mono-institutional phase II study. Cancer Chemother Pharmacol; 2008 Dec;63(1):149-55
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  • [Title] Gemcitabine at fixed dose-rate in patients with advanced soft-tissue sarcomas: a mono-institutional phase II study.
  • PURPOSE: To explore the clinical activity and toxicity of gemcitabine infused at the fixed dose of 10 mg/m(2)/min over 100 min in patients with soft tissue sarcomas (STSs).
  • PATIENTS AND METHODS: Fourteen patients with advanced locally unresectable and/or metastatic, pretreated STSs (seven leiomyosarcoma, three malignant schwannoma, one synovialsarcoma, one malignant fibrous histiocytoma, one endometrial stromal cell sarcoma, one undifferentiated) were treated with gemcitabine 10 mg/m(2)/min/week over 100 min given for 3 weeks out of 4.
  • The median number of previous medical treatments for advanced disease was 1 (range 1-2).
  • Treatment was well tolerated and the main causes of dose-reduction or omission/delay were hematological and liver toxicities.
  • One patient (7%; 95% confidence interval: 0.2-33.9%) with a metastatic uterine leiomyosarcoma obtained a partial response that lasted for 6.5 months.
  • Three patients (two leiomyosarcoma and one schwannoma) (21%) obtained a stabilization of disease.
  • The median time to progression was 3.1 months (range 1.0-9.5).
  • Nevertheless, an interesting tumor growth control rate was observed in specific histological variants (i.e., leiomyosarcoma), thus confirming data from recent controlled clinical trials.
  • [MeSH-major] Antimetabolites, Antineoplastic / therapeutic use. Deoxycytidine / analogs & derivatives. Sarcoma / drug therapy. Soft Tissue Neoplasms / drug therapy
  • [MeSH-minor] Adult. Aged. Combined Modality Therapy. Disease Progression. Dose-Response Relationship, Drug. Female. Gastrointestinal Diseases / chemically induced. Hematologic Diseases / chemically induced. Humans. Male. Middle Aged

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  • (PMID = 18351342.001).
  • [ISSN] 1432-0843
  • [Journal-full-title] Cancer chemotherapy and pharmacology
  • [ISO-abbreviation] Cancer Chemother. Pharmacol.
  • [Language] eng
  • [Publication-type] Clinical Trial, Phase II; Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Antimetabolites, Antineoplastic; 0W860991D6 / Deoxycytidine; B76N6SBZ8R / gemcitabine
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87. Oya N, Aoki J, Shinozaki T, Watanabe H, Takagishi K, Endo K: Preliminary study of proton magnetic resonance spectroscopy in bone and soft tissue tumors: an unassigned signal at 2.0-2.1 ppm may be a possible indicator of malignant neuroectodermal tumor. Radiat Med; 2000 May-Jun;18(3):193-8
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  • [Title] Preliminary study of proton magnetic resonance spectroscopy in bone and soft tissue tumors: an unassigned signal at 2.0-2.1 ppm may be a possible indicator of malignant neuroectodermal tumor.
  • PURPOSE: To evaluate the utility of proton magnetic resonance spectroscopy in bone and soft tissue tumors, especially whether or not the N-acetyl aspartate signal (NAA) could be recognized in neurogenic tumors.
  • MATERIALS AND METHODS: Forty-nine proton magnetic resonance spectroscopy studies were performed in 60 bone and soft tissue tumors.
  • RESULTS: An unassigned signal at about 2.0-2.1 ppm was recognized in six of 47 lesions: clear cell sarcoma (2/2), Ewing sarcoma (1/1), malignant fibrous histiocytoma (1/3), malignant schwannoma (1/1), and mucoepidermoid carcinoma (1/1).
  • Neuroblastoma (1/1), primitive neuroectodermal tumor (1/1), and malignant melanoma (1/1) after chemotherapy or radiotherapy did not show this signal.
  • This signal was not detected in neurofibroma (9/9), schwannoma (6/6), pheochromocytoma (2/2), or other mesenchymal tumors of non-neuroectodermal origin.
  • CONCLUSIONS: The assigned signal at about 2.0-2.1 ppm was detected in a small percentage of bone and soft tissue tumors and could be suggestive of an untreated malignant tumor of neuroectodermal origin.
  • [MeSH-major] Aspartic Acid / analogs & derivatives. Bone Neoplasms / diagnosis. Magnetic Resonance Spectroscopy. Neuroectodermal Tumors / diagnosis. Soft Tissue Neoplasms / diagnosis
  • [MeSH-minor] Adolescent. Adult. Aged. Aged, 80 and over. Child. Child, Preschool. Diagnosis, Differential. Female. Humans. Infant. Male. Middle Aged

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  • (PMID = 10972550.001).
  • [ISSN] 0288-2043
  • [Journal-full-title] Radiation medicine
  • [ISO-abbreviation] Radiat Med
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] JAPAN
  • [Chemical-registry-number] 30KYC7MIAI / Aspartic Acid; 997-55-7 / N-acetylaspartate
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88. Chen L, Mao Y, Chen H, Zhou LF: Diagnosis and management of intracranial malignant peripheral nerve sheath tumors. Neurosurgery; 2008 Apr;62(4):825-32; discussion 832

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  • [Title] Diagnosis and management of intracranial malignant peripheral nerve sheath tumors.
  • OBJECTIVE: Intracranial malignant peripheral nerve sheath tumors (MPNSTs) are rare and generally carry a poor prognosis.
  • We have analyzed our experience with MPNSTs and conducted a review of the literature in an attempt to identify a rational approach to the management of these tumors.
  • METHODS: Eight patients underwent surgical treatment for intracranial MPNSTs during a 10-year period from 1996 to 2005.
  • The general strategy was to perform complete resection whenever possible and to provide adjuvant radiotherapy for residual tumor.
  • Chemotherapy was not used in this group.
  • Total tumor resection was achieved in five patients.
  • At this time, two have been recurrence-free for 3.5 and 5 years, respectively, and the other three patients had a mean postoperative survival of 7 months.
  • There was one case of near total (>90%) and two cases of partial (<90%) tumor removal; the postoperative survival rate was 4, 4, and 2 months, respectively.
  • CONCLUSION: MPNSTs are fast-growing, invasive tumors with rather unsatisfactory outcomes.
  • Total surgical resection seems to be the most effective therapeutic method, and radiotherapy may play a role in local control.
  • [MeSH-major] Cranial Nerve Neoplasms / diagnosis. Cranial Nerve Neoplasms / surgery. Nerve Sheath Neoplasms / diagnosis. Nerve Sheath Neoplasms / surgery
  • [MeSH-minor] Adolescent. Adult. Child. Child, Preschool. Female. Humans. Male. Middle Aged. Treatment Outcome

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  • (PMID = 18496188.001).
  • [ISSN] 1524-4040
  • [Journal-full-title] Neurosurgery
  • [ISO-abbreviation] Neurosurgery
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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89. Rekhi B, Jambhekar NA, Puri A, Agrawal M, Chinoy RF: Clinicomorphologic features of a series of 10 cases of malignant triton tumors diagnosed over 10 years at a tertiary cancer hospital in Mumbai, India. Ann Diagn Pathol; 2008 Apr;12(2):90-7
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  • [Title] Clinicomorphologic features of a series of 10 cases of malignant triton tumors diagnosed over 10 years at a tertiary cancer hospital in Mumbai, India.
  • A rhabdomyoblastic differentiation in a malignant peripheral nerve sheath tumor is unusual and is termed as a malignant triton tumor.
  • Distinct rhabdomyoblastic cells were identified in the areas of malignant peripheral nerve sheath tumor.
  • Surgery with adequate margins constituted the treatment mainstay with adjuvant chemotherapy and/or radiotherapy in individual cases.
  • Malignant triton tumor is an uncommon tumor associated with an aggressive behavior.
  • Surgery with clear margins is the treatment mainstay.
  • [MeSH-major] Nerve Sheath Neoplasms / pathology. Rhabdomyosarcoma / pathology
  • [MeSH-minor] Adolescent. Adult. Biomarkers, Tumor / metabolism. Female. Humans. Immunohistochemistry. Male. Middle Aged. Neoplasm Recurrence, Local. S100 Proteins / analysis. Treatment Outcome

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  • (PMID = 18325468.001).
  • [ISSN] 1092-9134
  • [Journal-full-title] Annals of diagnostic pathology
  • [ISO-abbreviation] Ann Diagn Pathol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / S100 Proteins
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90. Wang Z, Gao J, Wang X, Zhou L, Hong B: [Clinical analysis of 7 cases of adult prostate sarcoma]. Zhonghua Nan Ke Xue; 2004 Sep;10(9):678-80
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  • OBJECTIVE: To investigate the diagnosis, treatment and prognosis of adult prostatic sarcoma.
  • METHODS: The records of 7 patients with prostate sarcoma were reviewed in the light of clinical manifestation, laboratory examination, therapeutic methods and histological subtypes.
  • RESULTS: Of the 7 cases, 3 were leiomyosarcoma, 3 rhabdomyosarcoma and the other malignant neurilemoma.
  • Most patients presented urinary obstruction, and the diagnosis of prostate sarcoma was established with ultrasound guided biopsy.
  • Two cases underwent total pelvic exenteration, followed by chemotherapy and/or radiotherapy and still alive 15 months after operation.
  • The other 3 received only chemotherapy and/or radiotherapy and died 7 months on average after diagnosis.
  • Early diagnosis and complete surgical resection offer patients the best chance for survival.
  • Long-term surveillance is necessary for the early detection of recurrence.
  • [MeSH-major] Prostatic Neoplasms / diagnosis. Prostatic Neoplasms / therapy. Sarcoma / diagnosis. Sarcoma / therapy

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  • (PMID = 15497710.001).
  • [ISSN] 1009-3591
  • [Journal-full-title] Zhonghua nan ke xue = National journal of andrology
  • [ISO-abbreviation] Zhonghua Nan Ke Xue
  • [Language] chi
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] China
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91. Leu KM, Ostruszka LJ, Shewach D, Zalupski M, Sondak V, Biermann JS, Lee JS, Couwlier C, Palazzolo K, Baker LH: Laboratory and clinical evidence of synergistic cytotoxicity of sequential treatment with gemcitabine followed by docetaxel in the treatment of sarcoma. J Clin Oncol; 2004 May 1;22(9):1706-12
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  • [Title] Laboratory and clinical evidence of synergistic cytotoxicity of sequential treatment with gemcitabine followed by docetaxel in the treatment of sarcoma.
  • Additionally, cell-culture studies were performed to assess the effect of the sequence of drug administration on colony formation.
  • Gemcitabine 675 mg/m(2) intravenously was administered over 90 minutes on days 1 and 8, and docetaxel 100 mg/m(2) intravenously was administered over 60 minutes on day 8 of a 21-day cycle.
  • Responses occurred in uterine, extremity, and retroperitoneal leiomyosarcoma, osteosarcomas, angiosarcomas, malignant fibrous histiocytomas, malignant peripheral-nerve sheath tumors, and Ewing's sarcoma.
  • In contrast, the administration of drugs simultaneously resulted in antagonism, and docetaxel followed by gemcitabine provided mixed results.
  • A multicenter, randomized clinical trial in soft tissue sarcoma comparing gemcitabine alone with this combination, is ongoing.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Bone Neoplasms / drug therapy. Deoxycytidine / analogs & derivatives. Sarcoma / drug therapy. Soft Tissue Neoplasms / drug therapy. Taxoids / therapeutic use
  • [MeSH-minor] Adult. Aged. Drug Administration Schedule. Drug Interactions. Female. Humans. Infusions, Intravenous. Male. Middle Aged. Retrospective Studies

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  • (PMID = 15117993.001).
  • [ISSN] 0732-183X
  • [Journal-full-title] Journal of clinical oncology : official journal of the American Society of Clinical Oncology
  • [ISO-abbreviation] J. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Taxoids; 0W860991D6 / Deoxycytidine; 15H5577CQD / docetaxel; B76N6SBZ8R / gemcitabine
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92. Pusceddu S, Bajetta E, Buzzoni R, Carcangiu ML, Platania M, Del Vecchio M, Ditto A: Primary uterine cervix melanoma resembling malignant peripheral nerve sheath tumor: a case report. Int J Gynecol Pathol; 2008 Oct;27(4):596-600
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  • [Title] Primary uterine cervix melanoma resembling malignant peripheral nerve sheath tumor: a case report.
  • A rare variant of malignant melanoma (MM) of the uterine cervix that mimics a malignant peripheral nerve sheath tumor (MPNST) is described.
  • Neoadjuvant chemotherapy and total abdominal hysterectomy and bilateral salpingo-ovariectomy plus pelvic lymphadenectomy were performed, and the diagnosis was MPNST, FIGO IIB.
  • A pathological review was obtained in our institution by a gynecological pathologist, who defined the primary neoplasm in the cervix as an MM, with a pattern of growth histologically simulating an MPNST, metastatic to the vagina.
  • To our knowledge, this is the first report in literature of MM of the uterine cervix resembling MPNST.
  • Despite its rarity, this variant of MM should be considered when a diagnosis of cervix MPNST is made.
  • The histological and immunohistochemical features of these different entities should be considered in the differential diagnosis.
  • [MeSH-major] Melanoma / pathology. Nerve Sheath Neoplasms / pathology. Uterine Cervical Neoplasms / pathology
  • [MeSH-minor] Adult. Diagnosis, Differential. Female. Histocytochemistry. Humans


93. Korf BR: Malignancy in neurofibromatosis type 1. Oncologist; 2000;5(6):477-85
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  • [Title] Malignancy in neurofibromatosis type 1.
  • Neurofibromatosis type 1 (NF1) represents a major risk factor for development of malignancy, particularly malignant peripheral nerve sheath tumors (MPNST), optic gliomas, other gliomas, and leukemias.
  • The oncologist will see NF1 patients referred for treatment of malignancy, and should be alert to the possibility of undiagnosed NF1 among patients with cancer.
  • Brain tumors tend to have a more indolent course in NF1 than in the general population, and hence are best managed conservatively.
  • MPNST, in contrast, do not respond to standard chemotherapy or radiation therapy.
  • The most effective treatment of MPNST appears to be early diagnosis and surgery, but early diagnosis is hampered by frequent occurrence within preexisting large tumors, making new growth or change difficult to detect.
  • New insights into pathogenesis now offer hope of development of specific methods of treatment with reduced toxicity and more precise molecular targeting.
  • There is an urgent need, however, to develop methods to measure tumor growth and monitor outcomes, develop preclinical drug screening systems, and further explore the pathogenesis of the disorder to determine whether mechanisms other than Ras regulation may be important in pathogenesis.
  • [MeSH-minor] Glioma / etiology. Humans. Leukemia / etiology. Neurilemmoma / etiology. Phenotype


94. Mosca F, Stracqualursi A, Lipari G, Latteri F, Palazzo F, Russo G: [Malignant schwannoma of the small intestine: a report of 2 cases]. G Chir; 2000 Apr;21(4):149-55
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  • [Title] [Malignant schwannoma of the small intestine: a report of 2 cases].
  • [Transliterated title] Lo schwannoma maligno dell'intestino tenue: descrizione di due casi.
  • The authors report two cases of small bowel malignant schwannoma.
  • Histologic differentiation from other stromal tumors may require electron microscopy, although the preparation of immunohistochemical reactions now allows to identify forms which were previously diagnosed in different terms to be precisely classified.
  • Diagnosis is often late and no preoperative test enables a correct clinical diagnosis to be made.
  • The primary treatment is surgical and the resection is the only real therapy.
  • Radiotherapy and chemotherapy are ineffective.
  • [MeSH-major] Ileal Neoplasms / diagnosis. Ileocecal Valve. Jejunal Neoplasms / diagnosis. Neurilemmoma / diagnosis

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  • (PMID = 10812769.001).
  • [ISSN] 0391-9005
  • [Journal-full-title] Il Giornale di chirurgia
  • [ISO-abbreviation] G Chir
  • [Language] ita
  • [Publication-type] Case Reports; English Abstract; Journal Article; Review
  • [Publication-country] ITALY
  • [Number-of-references] 19
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95. Li H, Zhang X, Fishbein L, Kweh F, Campbell-Thompson M, Perrin GQ, Muir D, Wallace M: Analysis of steroid hormone effects on xenografted human NF1 tumor schwann cells. Cancer Biol Ther; 2010 Oct 15;10(8):758-64
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  • [Title] Analysis of steroid hormone effects on xenografted human NF1 tumor schwann cells.
  • The neurofibroma, a common feature of neurofibromatosis type 1 (NF1), is a benign peripheral nerve sheath tumor that contains predominantly Schwann cells (SC).
  • This study examined the effects of estrogen and progesterone on proliferation and apoptosis in a panel of NF1 tumor xenografts.
  • SC-enriched cultures derived from three human NF1 tumor types (dermal neurofibroma, plexiform neurofibroma, and malignant peripheral nerve sheath tumor (MPNST)) were xenografted in sciatic nerves of ovariectomized scid /Nf1-/+ mice.
  • At the same time, mice were implanted with time-release pellets for systemic delivery of progesterone, estrogen or placebo.
  • Estrogen was found to increase the growth of all three MPNST xenografts.
  • Progesterone was associated with increased growth in two of the three MPNSTs, yet decreased growth of the other.
  • These findings indicate that human NF1 Schwann cells derived from some tumors show increased proliferation or decreased apoptosis in response to particular steroid hormones in a mouse xenograft model.
  • This suggests that anti-estrogen or anti-progesterone therapies may be worth considering for specific NF1 neurofibromas and MPNSTs.

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  • [Cites] JAMA. 1997 Jul 2;278(1):51-7 [9207339.001]
  • [Cites] Mol Cancer Res. 2009 Dec;7(12):2000-10 [19996302.001]
  • [Cites] J Clin Invest. 2000 May;105(9):1233-41 [10791998.001]
  • [Cites] J Biol Chem. 2000 Sep 29;275(39):30740-5 [10900196.001]
  • [Cites] Hum Mol Genet. 2000 Dec 12;9(20):3055-64 [11115850.001]
  • [Cites] Am J Pathol. 2001 Feb;158(2):501-13 [11159187.001]
  • [Cites] Oncogene. 2001 Jan 4;20(1):97-105 [11244508.001]
  • [Cites] Science. 2002 May 3;296(5569):920-2 [11988578.001]
  • [Cites] Arch Pathol Lab Med. 2002 Jul;126(7):816-22 [12088451.001]
  • [Cites] J Neuropathol Exp Neurol. 2002 Aug;61(8):702-9 [12152785.001]
  • [Cites] Cancer Chemother Pharmacol. 2011 Feb;67(2):285-91 [20383709.001]
  • [Cites] Cancer Res. 2003 Feb 15;63(4):752-5 [12591720.001]
  • [Cites] Mod Pathol. 2003 Apr;16(4):293-8 [12692193.001]
  • [Cites] J Clin Pathol. 2003 Oct;56(10):758-63 [14514779.001]
  • [Cites] Int J Cancer. 2004 Jan 1;108(1):8-14 [14618609.001]
  • [Cites] Acta Neuropathol. 2004 Feb;107(2):159-68 [14673600.001]
  • [Cites] Oncogene. 2004 Feb 5;23(5):1146-52 [14762442.001]
  • [Cites] Br J Cancer. 1990 Sep;62(3):405-9 [1698443.001]
  • [Cites] Acta Obstet Gynecol Scand. 1991;70(3):243-4 [1927303.001]
  • [Cites] Cancer Res. 1992 Aug 1;52(15):4261-4 [1322240.001]
  • [Cites] Brain Res Dev Brain Res. 1993 Apr 16;72(2):282-90 [8387406.001]
  • [Cites] Genes Dev. 1994 May 1;8(9):1019-29 [7926784.001]
  • [Cites] Clin Exp Metastasis. 1995 Jul;13(4):303-14 [7606893.001]
  • [Cites] Nat Genet. 1995 Sep;11(1):90-2 [7550323.001]
  • [Cites] J Steroid Biochem Mol Biol. 1996 Apr;58(1):77-82 [8809188.001]
  • [Cites] Am J Med Genet. 1996 Dec 2;66(1):7-10 [8957502.001]
  • [Cites] EMBO Mol Med. 2009 Jul;1(4):236-48 [20049725.001]
  • [Cites] Mod Pathol. 1998 Jul;11(7):612-7 [9688181.001]
  • [Cites] Eur J Cancer. 2004 Dec;40(18):2820-8 [15571966.001]
  • [Cites] Horm Behav. 2005 Mar;47(3):350-7 [15708765.001]
  • [Cites] Cancer Genet Cytogenet. 2005 Mar;157(2):181-6 [15721644.001]
  • [Cites] BMC Cancer. 2005 Feb 9;5:16 [15703081.001]
  • [Cites] J Neuropathol Exp Neurol. 2005 Dec;64(12):1080-8 [16319718.001]
  • [Cites] Exp Neurol. 2006 Jan;197(1):197-205 [16226751.001]
  • [Cites] Hum Mutat. 2006 Jul;27(7):716 [16786508.001]
  • [Cites] J Med Genet. 2007 Feb;44(2):81-8 [17105749.001]
  • [Cites] J Neurosci Res. 2007 May 1;85(6):1347-57 [17335073.001]
  • [Cites] Mol Carcinog. 2007 Jul;46(7):512-23 [17393410.001]
  • [Cites] Anticancer Res. 2007 Jul-Aug;27(4A):2085-90 [17649826.001]
  • [Cites] Lab Invest. 2007 Nov;87(11):1092-102 [17876295.001]
  • [Cites] Dev Dyn. 2008 Feb;237(2):513-24 [18213578.001]
  • [Cites] Clin Cancer Res. 2008 Feb 15;14(4):1015-24 [18281533.001]
  • [Cites] Clinics (Sao Paulo). 2008 Feb;63(1):39-42 [18297205.001]
  • [Cites] Am J Med Genet A. 2008 Jun 15;146A(12):1624-33 [18481270.001]
  • [Cites] Pediatr Res. 2008 Jul;64(1):40-3 [18360307.001]
  • [Cites] J Cutan Pathol. 2008 Nov;35(11):1014-9 [18547346.001]
  • [Cites] J Cell Biochem. 2009 Mar 1;106(4):553-62 [19160418.001]
  • [Cites] Neurogenetics. 2009 Jul;10(3):251-63 [19221814.001]
  • [Cites] Anticancer Res. 2009 Aug;29(8):3149-55 [19661328.001]
  • [Cites] Clin Cancer Res. 2009 Oct 15;15(20):6327-40 [19808870.001]
  • [Cites] Mol Carcinog. 2009 Nov;48(11):1005-17 [19479903.001]
  • [CommentIn] Cancer Biol Ther. 2010 Dec 1;10(11):1077-8 [20890125.001]
  • [CommentIn] Cancer Biol Ther. 2010 Oct 15;10(8):765-6 [20935464.001]
  • (PMID = 20699653.001).
  • [ISSN] 1555-8576
  • [Journal-full-title] Cancer biology & therapy
  • [ISO-abbreviation] Cancer Biol. Ther.
  • [Language] ENG
  • [Grant] United States / NINDS NIH HHS / NS / F30 NS043951; United States / NINDS NIH HHS / NS / 1F30NS43951
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, Non-P.H.S.
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Drug Implants; 0 / Estrogens; 0 / Ki-67 Antigen; 4G7DS2Q64Y / Progesterone
  • [Other-IDs] NLM/ PMC3093914
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96. Kitamura M, Wada N, Nagata S, Iizuka N, Jin YF, Tomoeda M, Yuki M, Naka N, Araki N, Yutani C, Tomita Y: Malignant peripheral nerve sheath tumor associated with neurofibromatosis type 1, with metastasis to the heart: a case report. Diagn Pathol; 2010;5:2
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  • [Title] Malignant peripheral nerve sheath tumor associated with neurofibromatosis type 1, with metastasis to the heart: a case report.
  • A rare case is presented of a 61-year-old man with a malignant peripheral nerve sheath tumor associated with neurofibromatosis type 1, with metastasis to the heart.
  • The primary tumor originated in the right thigh in 1982.
  • Since then, the patient has had repeated local recurrences in spite of repeated surgical treatment and adjuvant chemotherapy.
  • He has developed previous metastases of the lung and heart.
  • [MeSH-major] Heart Neoplasms / secondary. Nerve Sheath Neoplasms / secondary. Neurofibromatosis 1 / pathology. Soft Tissue Neoplasms / pathology
  • [MeSH-minor] Amputation. Autopsy. Biopsy. Chemotherapy, Adjuvant. Fatal Outcome. Humans. Lung Neoplasms / secondary. Lung Neoplasms / therapy. Magnetic Resonance Imaging. Male. Middle Aged. Neoplasm Recurrence, Local. Thigh. Treatment Outcome


97. Mrugala MM, Batchelor TT, Plotkin SR: Peripheral and cranial nerve sheath tumors. Curr Opin Neurol; 2005 Oct;18(5):604-10
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  • [Title] Peripheral and cranial nerve sheath tumors.
  • PURPOSE OF REVIEW: The intention of the authors is to provide the reader with an overview of the recent advances in the diagnosis and treatment of nerve sheath tumors.
  • Vestibular schwannomas, neurogenetic syndromes such as schwannomatosis and multiple isolated neurofibromas, and malignant peripheral nerve sheath tumors are covered in this review.
  • RECENT FINDINGS: Over the last year, literature focusing on different management strategies for patients with vestibular schwannomas dominated the field.
  • New insights into the biology of peripheral nerve tumor development and growth, including expression of vascular endothelial growth factor by vestibular schwannomas and the role of Notch signaling in malignant transformation of benign neurofibromas have been described.
  • Diagnostic criteria for schwannomatosis, a recently described condition, are being developed.
  • SUMMARY: Peripheral nerve tumors are classified according to the specific features of cellular differentiation.
  • The most common types include schwannoma and neurofibroma.
  • These tumors can occur sporadically or as manifestations of genetic syndromes such as neurofibromatosis types 1 and 2 or schwannomatosis.
  • The majority of peripheral nerve tumors are benign but malignant transformation does occur.
  • Metastatic tumors can also affect peripheral nerves.
  • Positron emission tomography is a useful technique in the presurgical differentiation between benign and malignant peripheral nerve sheath tumors.
  • Treatment is directed towards symptomatic control.
  • Surgery, radiation and, in rare instances, chemotherapy are the major treatment modalities employed.
  • [MeSH-major] Cranial Nerve Neoplasms. Nerve Sheath Neoplasms / diagnosis. Nerve Sheath Neoplasms / therapy. Peripheral Nervous System Neoplasms

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  • (PMID = 16155448.001).
  • [ISSN] 1350-7540
  • [Journal-full-title] Current opinion in neurology
  • [ISO-abbreviation] Curr. Opin. Neurol.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] England
  • [Number-of-references] 33
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98. Bisogno G, Sotti G, Nowicki Y, Ferrari A, Garaventa A, Zanetti I, Favre C, Schiavetti A, Tamaro P, Carli M: Soft tissue sarcoma as a second malignant neoplasm in the pediatric age group. Cancer; 2004 Apr 15;100(8):1758-65
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  • [Title] Soft tissue sarcoma as a second malignant neoplasm in the pediatric age group.
  • BACKGROUND: Survivors of childhood malignancies have an increased risk of developing second malignant neoplasms (SMN) due to their prior treatment and/or genetic susceptibility.
  • A small proportion of SMNs are soft tissue sarcomas (STS), whose prognosis is generally thought to be poor, though publications on such patients' treatment and outcome is limited.
  • The primary tumor was STS in five patients; Hodgkin disease in five patients; leukemia in four patients; retinoblastoma, neuroblastoma, and Wilms tumor in two patients each; and other tumor types in five patients.
  • SMNs occurred after a median of 8 years (range, 1.9-15.0 years) and included rhabdomyosarcoma (RMS) in 4 patients, malignant peripheral nerve sheath tumor in 4 patients, extraosseous Ewing family tumor (EFT) in 4 patients, leiomyosarcoma in 3 patients, fibrosarcoma in 2 patients, synovial sarcoma in 2 patients, and other tumor types in 6 patients.
  • Treatment generally was administered according to the guidelines for primary STS.
  • RESULTS: Seven non-RMS patients with STS underwent surgery alone, whereas 18 patients received chemotherapy and 8 patients received radiotherapy.
  • Fifteen patients were alive in complete remission of their SMN at the time of last follow-up.
  • Responses to chemotherapy and survival were satisfactory for patients with tumors such as RMS and EFT.
  • Complete tumor resection was correlated with a favorable prognosis in patients with other types of STS and in patients with postirradiation sarcoma.
  • Two patients developed a third malignancy.
  • CONCLUSIONS: Although prior treatment may hinder the management of these patients, pediatric STS second malignancies can be cured using the same strategies used for de novo pediatric sarcomas.
  • Long-term follow-up is mandatory given the risks of further malignancies and more severe, treatment-related side effects.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Neoplasms, Second Primary. Sarcoma / surgery. Soft Tissue Neoplasms / surgery
  • [MeSH-minor] Adolescent. Adult. Child. Child, Preschool. Combined Modality Therapy. Female. Follow-Up Studies. Humans. Male. Prognosis. Survival Analysis. Treatment Outcome

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  • [Copyright] Copyright 2004 American Cancer Society.
  • (PMID = 15073867.001).
  • [ISSN] 0008-543X
  • [Journal-full-title] Cancer
  • [ISO-abbreviation] Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
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99. López Alvarez F, Llorente Pendás JL, Coca Pelaz A, Fernández García MS, Cuello Bueno G, Suárez Nieto C: Malignant triton tumor of the infratemporal fossa. J Craniofac Surg; 2009 Jul;20(4):1282-6

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Malignant triton tumor of the infratemporal fossa.
  • Malignant triton tumor is a very aggressive type of sarcoma that comprises rhabdomyoblasts and malignant Schwann cells.
  • It is a different entity from malignant schwannoma, characterized by their aggressiveness and poor prognosis.
  • Head and neck location is frequent, and early diagnosis and complete resection followed by radiation therapy is important for long-term survival.
  • However, the therapeutic plan should be individualized, taking into account the location and size of the primary tumor.
  • The use of adjuvant chemotherapy and molecular therapies should be considered in the treatment of these tumors.
  • We report an unusual presentation of a malignant triton tumor located in the infratemporal fossa, describing its clinical and pathologic features, and we try to update the knowledge in the management of these tumors, including the use of molecular therapies.
  • [MeSH-major] Hamartoma / diagnosis. Skull Base Neoplasms / diagnosis
  • [MeSH-minor] Biopsy. Combined Modality Therapy. Diagnosis, Differential. Endoscopy. Fatal Outcome. Female. Humans. Tomography, X-Ray Computed. Young Adult

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  • (PMID = 19625850.001).
  • [ISSN] 1536-3732
  • [Journal-full-title] The Journal of craniofacial surgery
  • [ISO-abbreviation] J Craniofac Surg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
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100. Wojtkowiak JW, Fouad F, LaLonde DT, Kleinman MD, Gibbs RA, Reiners JJ Jr, Borch RF, Mattingly RR: Induction of apoptosis in neurofibromatosis type 1 malignant peripheral nerve sheath tumor cell lines by a combination of novel farnesyl transferase inhibitors and lovastatin. J Pharmacol Exp Ther; 2008 Jul;326(1):1-11
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  • [Title] Induction of apoptosis in neurofibromatosis type 1 malignant peripheral nerve sheath tumor cell lines by a combination of novel farnesyl transferase inhibitors and lovastatin.
  • Neurofibromatosis type 1 (NF1) is a genetic disorder that is driven by the loss of neurofibromin (Nf) protein function.
  • Hence, Ras is a potential therapeutic target for treating NF1.
  • We have tested the efficacy of two novel farnesyl transferase inhibitors (FTIs), 1 and 2, alone or in combination with lovastatin, on two NF1 malignant peripheral nerve sheath tumor (MPNST) cell lines, NF90-8 and ST88-14.
  • Single treatments of 1, 2, or lovastatin had no effect on Ras prenylation or MPNST cell proliferation.
  • However, low micromolar combinations of 1 or 2 with lovastatin (FTI/lovastatin) reduced Ras prenylation in both MPNST cell lines.
  • Furthermore, this FTI/lovastatin combination treatment reduced cell proliferation and induced an apoptotic response as shown by morphological analysis, procaspase-3/-7 activation, loss of mitochondrial membrane potential, and accumulation of cells with sub-G(1) DNA content.
  • Little to no detectable toxicity was observed in normal rat Schwann cells following FTI/lovastatin combination treatment.
  • These data support the hypothesis that combination FTI plus lovastatin therapy may be a potential treatment for NF1 MPNSTs.
  • [MeSH-major] Apoptosis / drug effects. Farnesyltranstransferase / antagonists & inhibitors. Lovastatin / administration & dosage. Nerve Sheath Neoplasms / drug therapy. Nerve Sheath Neoplasms / pathology. Neurofibromatosis 1 / drug therapy. Neurofibromatosis 1 / pathology
  • [MeSH-minor] Animals. Animals, Newborn. Cell Line. Cell Line, Transformed. Cell Line, Tumor. Drug Therapy, Combination. Enzyme Inhibitors / administration & dosage. Rats. Rats, Sprague-Dawley

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  • [Cites] J Biol Chem. 2000 Sep 29;275(39):30451-7 [10852915.001]
  • [Cites] J Biol Chem. 1997 May 30;272(22):14459-64 [9162087.001]
  • [Cites] Cell Signal. 2001 Jul;13(7):499-505 [11516625.001]
  • [Cites] Curr Med Chem. 2001 Oct;8(12):1437-65 [11562276.001]
  • [Cites] J Cell Biochem Suppl. 2001;Suppl 37:64-70 [11842430.001]
  • [Cites] Leukemia. 2002 Apr;16(4):508-19 [11960327.001]
  • [Cites] Neurology. 2002 May 28;58(10):1461-70 [12041525.001]
  • [Cites] J Pharmacol Exp Ther. 2002 Oct;303(1):74-81 [12235235.001]
  • [Cites] Neurol Clin. 2002 Aug;20(3):841-65 [12432832.001]
  • [Cites] Leukemia. 2003 Aug;17(8):1482-98 [12886235.001]
  • [Cites] Curr Opin Neurol. 2004 Apr;17(2):101-5 [15021234.001]
  • [Cites] Mol Pharmacol. 2004 Apr;65(4):1016-28 [15044632.001]
  • [Cites] Cell Growth Differ. 1990 Oct;1(10):499-502 [2278880.001]
  • [Cites] J Biol Chem. 1997 Jun 20;272(25):15591-4 [9188444.001]
  • [Cites] J Cell Biochem. 1997 Dec 1;67(3):399-408 [9361194.001]
  • [Cites] Proc Natl Acad Sci U S A. 1998 Apr 28;95(9):5139-44 [9560242.001]
  • [Cites] Am J Respir Crit Care Med. 1998 May;157(5 Pt 1):1616-22 [9603146.001]
  • [Cites] J Neurosci Res. 1998 Sep 15;53(6):747-56 [9753202.001]
  • [Cites] Clin Cancer Res. 1996 Mar;2(3):483-91 [9816194.001]
  • [Cites] Surg Neurol. 1999 Feb;51(2):211-8 [10029430.001]
  • [Cites] Carcinogenesis. 1999 Aug;20(8):1561-6 [10426807.001]
  • [Cites] Blood. 1999 Oct 1;94(7):2469-76 [10498620.001]
  • [Cites] Cell Cycle. 2004 Oct;3(10):1227-9 [15467451.001]
  • [Cites] Pediatr Neurol. 2005 Apr;32(4):221-8 [15797177.001]
  • [Cites] Neoplasia. 2005 Jul;7(7):638-45 [16026643.001]
  • [Cites] J Biol Chem. 2005 Sep 2;280(35):31101-8 [16006564.001]
  • [Cites] Br J Haematol. 2005 Sep;130(6):912-25 [16156861.001]
  • [Cites] Curr Biol. 2005 Nov 8;15(21):1961-7 [16271875.001]
  • [Cites] J Pharmacol Exp Ther. 2006 Jan;316(1):456-65 [16239399.001]
  • [Cites] J Lipid Res. 2006 Jan;47(1):15-31 [16278491.001]
  • [Cites] J Clin Oncol. 2006 Jan 20;24(3):507-16 [16421428.001]
  • [Cites] Cancer Res. 2006 Jan 15;66(2):1040-51 [16424040.001]
  • [Cites] Biochem Pharmacol. 2006 Nov 30;72(11):1485-92 [16797490.001]
  • [Cites] J Med Chem. 2007 Jul 12;50(14):3274-82 [17555307.001]
  • [Cites] Glia. 2007 Aug 15;55(11):1123-33 [17597122.001]
  • [Cites] Biochim Biophys Acta. 2007 Aug;1773(8):1177-95 [17428555.001]
  • [Cites] Cancer Commun. 1991 May;3(5):141-7 [2043425.001]
  • [Cites] Proc Natl Acad Sci U S A. 1991 Nov 1;88(21):9668-70 [1946384.001]
  • [Cites] Proc Natl Acad Sci U S A. 1991 Nov 15;88(22):9914-8 [1946460.001]
  • [Cites] Nature. 1992 Apr 23;356(6371):713-5 [1570015.001]
  • [Cites] J Neurosci Res. 1992 Oct;33(2):231-8 [1280693.001]
  • [Cites] J Biol Chem. 1993 Dec 25;268(36):27012-9 [8262937.001]
  • [Cites] Cancer Res. 1995 Aug 15;55(16):3569-75 [7627966.001]
  • [Cites] Mol Cell Biol. 1997 Feb;17(2):862-72 [9001241.001]
  • [Cites] Oncogene. 2001 May 24;20(23):2918-26 [11420704.001]
  • (PMID = 18367665.001).
  • [ISSN] 1521-0103
  • [Journal-full-title] The Journal of pharmacology and experimental therapeutics
  • [ISO-abbreviation] J. Pharmacol. Exp. Ther.
  • [Language] eng
  • [Grant] United States / NIEHS NIH HHS / ES / T32 ES012163; United States / NIEHS NIH HHS / ES / T32 ES012163-05; United States / NIEHS NIH HHS / ES / P30 ES06639; United States / NCI NIH HHS / CA / P30 CA022453-25; United States / NIEHS NIH HHS / ES / P30 ES006639; United States / NCI NIH HHS / CA / P30 CA022453; United States / NIEHS NIH HHS / ES / P30 ES006639-11; United States / NCI NIH HHS / CA / P30 CA22453
  • [Publication-type] Comparative Study; Journal Article; Research Support, N.I.H., Extramural; Research Support, U.S. Gov't, Non-P.H.S.
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Enzyme Inhibitors; 9LHU78OQFD / Lovastatin; EC 2.5.1.29 / Farnesyltranstransferase
  • [Other-IDs] NLM/ NIHMS256795; NLM/ PMC3768167
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