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1. Ferrari A, Bisogno G, Macaluso A, Casanova M, D'Angelo P, Pierani P, Zanetti I, Alaggio R, Cecchetto G, Carli M: Soft-tissue sarcomas in children and adolescents with neurofibromatosis type 1. Cancer; 2007 Apr 1;109(7):1406-12
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Soft-tissue sarcomas in children and adolescents with neurofibromatosis type 1.
  • BACKGROUND: Patients affected by neurofibromatosis type 1 (NF1) are at higher risk of developing soft-tissue sarcomas (STS) than the general population.
  • METHODS: The study included 37 patients with neurogenic sarcomas (36 malignant peripheral nerve sheath tumors [MPNST], 1 triton tumor) and 6 cases of rhabdomyosarcoma (RMS).
  • The prevalence of NF1 observed during the study period was 43% in the MPNST population and 1% in the RMS group.
  • Two of 16 patients with evaluable disease responded to chemotherapy.
  • All 6 RMS patients were </=3 years old and had embryonal subtype, 5 of 6 arising in the genitourinary tract or pelvis (paravesical); 4 were alive in first remission at the time of the analysis, 1 was alive in second remission after a local recurrence, and 1 died of disease.
  • CONCLUSIONS: The occurrence of STS in pediatric patients with NF1 syndrome in Italy is discussed, confirming that NF1 patients have a high risk of developing STS, and particularly MPNST, often with an aggressive clinical presentation and poor outcome.
  • [MeSH-minor] Adolescent. Child. Child, Preschool. Combined Modality Therapy. Female. Humans. Infant. Male. Neoplasms, Second Primary / complications. Neoplasms, Second Primary / diagnosis. Neoplasms, Second Primary / therapy. Nerve Sheath Neoplasms / complications. Nerve Sheath Neoplasms / diagnosis. Nerve Sheath Neoplasms / therapy. Neurofibrosarcoma / complications. Neurofibrosarcoma / diagnosis. Neurofibrosarcoma / therapy. Peripheral Nervous System Neoplasms / complications. Peripheral Nervous System Neoplasms / diagnosis. Peripheral Nervous System Neoplasms / therapy. Prognosis. Prospective Studies. Rhabdomyosarcoma / complications. Rhabdomyosarcoma / diagnosis. Rhabdomyosarcoma / therapy. Risk Factors. Survival Rate

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  • [Copyright] (c) 2007 American Cancer Society.
  • (PMID = 17330850.001).
  • [ISSN] 0008-543X
  • [Journal-full-title] Cancer
  • [ISO-abbreviation] Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Multicenter Study; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
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2. El-Haddad AM, Ibrahim MF, El-Wakil MA, El-Bolkainy TN, Farahat IG: Pediatric Non Metastatic Non Rhabdomyosarcoma Soft Tissue Sarcomas (NRSTSS): Five Years Experience from NCI-Egypt. J Egypt Natl Canc Inst; 2008 Dec;20(4):395-402

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Pediatric Non Metastatic Non Rhabdomyosarcoma Soft Tissue Sarcomas (NRSTSS): Five Years Experience from NCI-Egypt.
  • PURPOSE: Evaluation of demographic, pathological, and clinical patterns in addition to treatment outcome of pediatric NRSTS patients treated at the NCI, Egypt.
  • PROCEDURE: 21 pediatric patients of NRSTS between 2001 and 2006 were included.
  • Clinical and pathological diagnosis and subtyping verification were done.
  • Patients' cohort formed of 3 treatment groups. (1) Patients who underwent complete surgical resection with no adjuvant therapies. (2) Patients who received chemotherapy and complete surgical resection, and group (3) Patients with localized unrersectable tumors for whom systemic chemotherapy only was given.
  • Demographic, clinicopathological variables, and treatment modalities were statistically evaluated and compared with the outcome.
  • RESULTS: Tumors of unknown histiogenesis followed by MPNST and myxofibrosarcoma were the most frequent tumor subtypes.
  • Low tumor grade was in favor of better outcome.
  • With a median follow up of 2-years; respectively 100% and 81.1% of patients who had complete surgical resection of a localized disease with or without chemotherapy entered in CR (p=0.01).
  • CONCLUSIONS: Complete surgical resection with or without chemotherapy is the mainstay of therapy for localized NRSTS.
  • Tumor grade and surgical resection of NRSTS are 2 important predictors of prognosis.
  • KEY WORDS: Nonrhabdomyosarcoma - Soft tissue sarcoma - Pediatric.

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  • (PMID = 20571598.001).
  • [ISSN] 1110-0362
  • [Journal-full-title] Journal of the Egyptian National Cancer Institute
  • [ISO-abbreviation] J Egypt Natl Canc Inst
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Egypt
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3. Kawai A, Kondo T, Suehara Y, Kikuta K, Hirohashi S: Global protein-expression analysis of bone and soft tissue sarcomas. Clin Orthop Relat Res; 2008 Sep;466(9):2099-106
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  • [Title] Global protein-expression analysis of bone and soft tissue sarcomas.
  • Analysis of global protein expression, an approach known as expression proteomics, can offer important clues for understanding tumor biology that cannot be obtained by other approaches (e.g., genome or transcriptome analysis).
  • Using two-dimensional difference gel electrophoresis (2D-DIGE) and mass spectrometry, we performed global protein expression studies of bone and soft tissue sarcomas to develop novel diagnostic and therapeutic biomarkers and allow molecular classification of the tumors.
  • Among 1500 protein variants identified in the two-dimensional gel, 67 proteins correctly distinguished the eight subtypes of 99 histologically classified soft tissue sarcomas.
  • Hierarchical clustering demonstrated leiomyosarcoma and MFH shared a similar protein expression profile, and clear cell sarcoma, synovial sarcoma, and MPNST could be grouped according to their protein expression patterns.
  • We identified 10 protein spots associated with the chemosensitivity of osteosarcoma to preoperative chemotherapy.
  • These 10 spots could be new diagnostic and prognostic markers for osteosarcoma and new therapeutic targets for the disease.
  • Proteomic analysis using 2D-DIGE provides novel information on the biology of bone and soft tissue sarcomas that could be used to diagnosis and treat these tumors.

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  • (PMID = 18535868.001).
  • [ISSN] 1528-1132
  • [Journal-full-title] Clinical orthopaedics and related research
  • [ISO-abbreviation] Clin. Orthop. Relat. Res.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / KCTD12 protein, human; 0 / Proteins
  • [Other-IDs] NLM/ PMC2493021
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4. Casanova M, Meazza C, Gronchi A, Fiore M, Zaffignani E, Podda M, Collini P, Gandola L, Ferrari A: Soft-tissue sarcomas of the extremities in patients of pediatric age. J Child Orthop; 2007 Sep;1(3):195-203

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Soft-tissue sarcomas of the extremities in patients of pediatric age.
  • PURPOSE: The extremity site is a peculiar location for soft-tissue sarcomas (STS) of children and adolescents.
  • METHODS: The study series included 52 patients with rhabdomyosarcoma (RMS)(65% of which were of the alveolar subtype), nine with extraosseous Ewing sarcoma and 143 with non-rhabdomyosarcoma soft-tissue sarcomas (NRSTS), 38% of which were synovial sarcoma.
  • Patients were treated with a multimodality approach including surgery, chemotherapy, and radiotherapy.
  • RESULTS: For the RMS patients, the 5-year event-free survival (EFS) rate was 37.1%, with distant metastases being the main cause of treatment failure.
  • For the NRSTS cases, the 5-year EFS was 72.6%: tumor size and local invasiveness, tumor grade, malignant peripheral nerve sheath tumor (MPNST) histology, and distant metastases were the main prognostic factors.
  • DISCUSSION: While the limbs are the most common sites of NRSTS and are often characterized by a more favorable prognosis than for axial tumors, the clinical features of extremity RMS often differ from those of RMS of other sites, with a higher incidence of unfavorable prognostic factors (e.g., alveolar subtype) and consequently unsatisfactory treatment results.
  • The treatment of these patients is complex and necessarily multidisciplinary, and it demands not only adequate experience of treating children and adolescents in clinical trials, but also particular skills in the field of orthopedic surgery.

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  • (PMID = 19308495.001).
  • [ISSN] 1863-2521
  • [Journal-full-title] Journal of children's orthopaedics
  • [ISO-abbreviation] J Child Orthop
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Other-IDs] NLM/ PMC2656726
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5. 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


6. 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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  • [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.
  • 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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7. 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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  • [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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  • (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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8. 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.
  • 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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9. 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].
  • 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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10. 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.
  • 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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11. 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

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  • [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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12. 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

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  • [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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13. 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.
  • 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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14. Komdeur R, Plaat BE, van der Graaf WT, Hoekstra HJ, Hollema H, van den Berg E, Zwart N, Scheper RJ, Molenaar WM: Expression of multidrug resistance proteins, P-gp, MRP1 and LRP, in soft tissue sarcomas analysed according to their histological type and grade. Eur J Cancer; 2003 May;39(7):909-16
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Expression of multidrug resistance proteins, P-gp, MRP1 and LRP, in soft tissue sarcomas analysed according to their histological type and grade.
  • The biological behaviour of different histological types and grades of soft tissue sarcomas (STS) varies.
  • This might result in a differing sensitivity to cytotoxic drugs.
  • Cross-resistance to functionally and structurally distinct natural-product drugs, known as multidrug resistance (MDR), is associated with the overexpression of P-glycoprotein (P-gp), multidrug resistance-associated protein 1 (MRP1) and lung resistance-related protein (LRP).
  • The purpose of this study was to evaluate the expression of P-gp, MRP1 and LRP in STS according to their histological type and grade.
  • In 141 chemotherapy-naive STS patients, the expression of the three MDR proteins was detected by immunohistochemistry.
  • Nine histological types were documented.
  • P-gp expression was most pronounced in malignant fibrous histiocytoma (MFH), but was low in leiomyosarcomas.
  • MRP1 was expressed in most malignant peripheral nerve sheath tumours (MPNST).
  • In conclusion, P-gp, MRP1 and LRP are expressed in the majority of STS, but this expression varies according to the histological type.
  • [MeSH-major] Multidrug Resistance-Associated Proteins / metabolism. Neoplasm Proteins / metabolism. P-Glycoproteins / metabolism. Sarcoma / drug therapy. Soft Tissue Neoplasms / drug therapy. Vault Ribonucleoprotein Particles / metabolism
  • [MeSH-minor] Adolescent. Adult. Aged. Aged, 80 and over. Child. Child, Preschool. Drug Resistance, Multiple. Drug Resistance, Neoplasm. Female. Humans. Immunohistochemistry / methods. Infant. Male. Middle Aged

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  • (PMID = 12706359.001).
  • [ISSN] 0959-8049
  • [Journal-full-title] European journal of cancer (Oxford, England : 1990)
  • [ISO-abbreviation] Eur. J. Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / ABCC4 protein, human; 0 / Multidrug Resistance-Associated Proteins; 0 / Neoplasm Proteins; 0 / P-Glycoproteins; 0 / Vault Ribonucleoprotein Particles; 0 / major vault protein
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15. 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].
  • 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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16. 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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  • [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.
  • 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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  • (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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17. 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.
  • 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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  • (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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18. 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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  • [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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  • (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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19. 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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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [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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20. 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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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • 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.
  • 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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21. Stark AM, Buhl R, Hugo HH, Mehdorn HM: Malignant peripheral nerve sheath tumours--report of 8 cases and review of the literature. Acta Neurochir (Wien); 2001;143(4):357-63; discussion 363-4
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  • [Title] Malignant peripheral nerve sheath tumours--report of 8 cases and review of the literature.
  • BACKGROUND: Though Malignant peripheral nerve sheath tumours (MPNST) are a rare entity accounting for 5-10% of soft-tissue sarcomas they are an important differential diagnosis to benign tumours of the peripheral nervous system regarding treatment and prognosis.
  • METHOD: We present our experience with eight patients who underwent surgery for MPNST at the Department of Neurosurgery between 10/1990 and 9/1999.
  • Two patients suffered from Neurofibromatosis type 1.
  • All of these developed local recurrence with a mean disease free survival time of 10.6 months.
  • During follow up, three patients developed distant metastases located in the lung, liver and subcutaneous tissue.
  • Five out of eight patients died during follow-up with a mean survival time of 11.6 months after diagnosis.
  • INTERPRETATION: MPNST is a rare and fatal diagnosis in neurosurgery with high risk of local recurrence and occurence of distant metastases.
  • Though mulitimodal therapy including surgical resection and adjuvant radiotherapy including brachytherapy is available, the prognosis remains dismal.
  • Modern clinical studies and the development of effective chemotherapy is needed in order to gain control of the disease.
  • [MeSH-major] Head and Neck Neoplasms / mortality. Head and Neck Neoplasms / surgery. Neoplasm Recurrence, Local / mortality. Nerve Sheath Neoplasms / mortality. Nerve Sheath Neoplasms / surgery. Spinal Cord Neoplasms / mortality. Spinal Cord Neoplasms / surgery. Thoracic Neoplasms / mortality. Thoracic Neoplasms / surgery

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  • (PMID = 11437289.001).
  • [ISSN] 0001-6268
  • [Journal-full-title] Acta neurochirurgica
  • [ISO-abbreviation] Acta Neurochir (Wien)
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] Austria
  • [Number-of-references] 5
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22. 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

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [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).
  • 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.
  • 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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23. 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

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [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.
  • 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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24. Longhi A, Errani C, Magagnoli G, Alberghini M, Gambarotti M, Mercuri M, Ferrari S: High grade malignant peripheral nerve sheath tumors: outcome of 62 patients with localized disease and review of the literature. J Chemother; 2010 Dec;22(6):413-8
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] High grade malignant peripheral nerve sheath tumors: outcome of 62 patients with localized disease and review of the literature.
  • Malignant peripheral nerve sheath tumours (MPNST) are rare sarcomas with one of the poorest prognoses of all the soft tissue sarcomas.
  • Information about adjuvant treatment is scarce and not homogeneous for this diagnosis.
  • We analyzed retrospectively the outcome of patients with localized high grade MPNST admitted to our institute from 1969 to 2008.
  • Of 62 evaluable patients, 23 were females and 39 males, median age 39 years (17-71), 22/62 had neurofibromatosis type I.
  • A total of 22/62 are alive; 26 patients had surgery alone, 18 received radiation therapy, 12 received radiation therapy and chemotherapy, and 6 received only adjuvant chemotherapy.
  • A positive trend for adjuvant radiation, but not for chemotherapy was observed according to univariate analysis only for disease-free survival and overall survival.
  • New drugs employed successfully in advanced mpNSt should be employed also in the adjuvant setting.
  • [MeSH-major] Nerve Sheath Neoplasms / diagnosis. Nerve Sheath Neoplasms / therapy. Sarcoma / diagnosis. Sarcoma / therapy
  • [MeSH-minor] Adolescent. Adult. Aged. Chemotherapy, Adjuvant. Disease-Free Survival. Female. Humans. Male. Middle Aged. Retrospective Studies. Survival Rate. Treatment Outcome. Young Adult

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  • (PMID = 21303750.001).
  • [ISSN] 1973-9478
  • [Journal-full-title] Journal of chemotherapy (Florence, Italy)
  • [ISO-abbreviation] J Chemother
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Italy
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25. Perrin RG, Guha A: Malignant peripheral nerve sheath tumors. Neurosurg Clin N Am; 2004 Apr;15(2):203-16
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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 tumors.
  • Suspicion of an MPNST based on clinical or radiologic alteration of a soft tissue mass in proximity to a peripheral nerve, especially in the context of NF I, should lead to referral to such a tertiary center.
  • Early diagnosis followed by oncologic surgery to obtain tumor-free margins provides the best chance for long-term cure.
  • Current adjuvant therapy with radiation and chemotherapy is suboptimal.
  • There have been major inroads toward the molecular biologic understanding of MPNSTs,with several biologic targets that are of potential therapeutic interest.
  • [MeSH-major] Neurofibromatosis 1 / pathology. Neurofibromatosis 1 / surgery. Peripheral Nervous System Neoplasms / pathology. Peripheral Nervous System Neoplasms / surgery

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  • (PMID = 15177319.001).
  • [ISSN] 1042-3680
  • [Journal-full-title] Neurosurgery clinics of North America
  • [ISO-abbreviation] Neurosurg. Clin. N. Am.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 125
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26. Siveke JT, Sen Gupta R, Rieckhoff KU, Braumann D, Goldmann T: [Progressive paralysis caused by radiation-induced cervical malignant peripheral nerve sheath tumor]. HNO; 2003 Oct;51(10):825-8
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Progressive paralysis caused by radiation-induced cervical malignant peripheral nerve sheath tumor].
  • HISTORY AND DIAGNOSIS: A 59-year old engineer was admitted to the hospital because of pain in his right collar region and the onset of incomplete paresis of the right arm.
  • Magnetic resonance tomography displayed an advanced tumour arising from the right paravertebral soft tissue.
  • Histological examination revealed a malignant peripheral nerve sheath tumor (MPNST).
  • Thirteen years before admission, the patient had a right-sided tumor-tonsillectomy of a squamous cell carcinoma and local radiation of a cystic squamous cell carcinoma in the ipsilateral cervical soft tissue.
  • CLINICAL COURSE AND THERAPY: In the following course, progressive neurological symptoms occurred including beginning paraplegia, right phrenic paralysis and a severe concomitant pain syndrome.
  • Due to the location and advanced tumor state, surgical treatment was not performed and palliative chemotherapy remained ineffective.
  • CONCLUSION: MPNST represents a rare entity which has been related to postoperative radiation.
  • Unusual neurological symptoms in anatomical regions of former radiation should therefore include neurogenic secondary malignancies in the differential diagnosis for early surgical intervention.
  • [MeSH-major] Carcinoma, Squamous Cell / radiotherapy. Head and Neck Neoplasms / diagnosis. Neoplasms, Multiple Primary / radiotherapy. Neoplasms, Radiation-Induced / diagnosis. Neoplasms, Second Primary / diagnosis. Nerve Sheath Neoplasms / diagnosis. Paraparesis / etiology. Radioisotope Teletherapy / adverse effects. Tonsillar Neoplasms / radiotherapy
  • [MeSH-minor] Arm / innervation. Combined Modality Therapy. Diagnosis, Differential. Humans. Magnetic Resonance Imaging. Male. Middle Aged. Radiotherapy, Adjuvant. Respiratory Paralysis / etiology. Tomography, X-Ray Computed

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  • [Cites] Cancer. 1981 May 15;47(10):2503-9 [6791802.001]
  • [Cites] Cancer. 1993 Feb 15;71(4):1247-53 [8435801.001]
  • [Cites] Cancer. 1983 Mar 15;51(6):1028-33 [6821867.001]
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  • [Cites] Int J Radiat Oncol Biol Phys. 1999 Nov 1;45(4):951-61 [10571202.001]
  • [Cites] Int J Radiat Oncol Biol Phys. 1998 Sep 1;42(2):351-60 [9788415.001]
  • [Cites] Cancer. 1986 May 15;57(10):2006-21 [3082508.001]
  • (PMID = 14523537.001).
  • [ISSN] 0017-6192
  • [Journal-full-title] HNO
  • [ISO-abbreviation] HNO
  • [Language] ger
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Germany
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27. 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.
  • 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


28. 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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  • [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 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.
  • 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.
  • 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


29. 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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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [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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