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1. Dhingra KK, Mandal S, Roy S, Khurana N: Malignant peripheral nerve sheath tumor of the breast: case report. World J Surg Oncol; 2007;5:142
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  • [Title] Malignant peripheral nerve sheath tumor of the breast: case report.
  • BACKGROUND: Malignant peripheral nerve sheath tumor is a rare soft tissue sarcoma of ectomesenchymal origin.
  • It is the malignant counterpart of benign soft tissue tumors like neurofibromas and schwannomas and may often follow them.
  • Common sites include deeper soft tissues, usually in the proximity of a nerve trunk.
  • Breast is an extremely rare location of this lesion and presentation as a breast lump in the absence of pain or previous benign neural tumor is even rarer.
  • Histopathology revealed a malignant spindle cell tumor which was confirmed to be malignant peripheral nerve sheath tumor on the basis of immunopositivity for vimentin, neurone specific enolase and S-100.
  • The differential diagnosis of malignant peripheral nerve sheath tumor should be considered by the clinician as well as the pathologists in the work-up of a breast neoplasm as treatment and prognosis of this rare malignancy is different.
  • [MeSH-major] Breast Neoplasms / pathology. Nerve Sheath Neoplasms / pathology. Peripheral Nervous System Neoplasms / pathology
  • [MeSH-minor] Adult. Biomarkers, Tumor / metabolism. Biopsy, Fine-Needle. Diagnosis, Differential. Female. Humans. Vimentin / metabolism

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  • (PMID = 18154670.001).
  • [ISSN] 1477-7819
  • [Journal-full-title] World journal of surgical oncology
  • [ISO-abbreviation] World J Surg Oncol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Vimentin
  • [Other-IDs] NLM/ PMC2246134
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2. Kresse SH, Skårn M, Ohnstad HO, Namløs HM, Bjerkehagen B, Myklebost O, Meza-Zepeda LA: DNA copy number changes in high-grade malignant peripheral nerve sheath tumors by array CGH. Mol Cancer; 2008;7:48
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  • [Title] DNA copy number changes in high-grade malignant peripheral nerve sheath tumors by array CGH.
  • BACKGROUND: Malignant peripheral nerve sheath tumors (MPNSTs) are rare and highly aggressive soft tissue tumors showing complex chromosomal aberrations.
  • In order to identify recurrent chromosomal regions of gain and loss, and thereby novel gene targets of potential importance for MPNST development and/or progression, we have analyzed DNA copy number changes in seven high-grade MPNSTs using microarray-based comparative genomic hybridization (array CGH).
  • CONCLUSION: Our study shows the potential of using DNA copy number changes obtained by array CGH to predict the prognosis of MPNST patients.
  • [MeSH-major] DNA, Neoplasm / analysis. Gene Dosage. Gene Expression Profiling / methods. Gene Expression Regulation, Neoplastic. Nerve Sheath Neoplasms / genetics. Oligonucleotide Array Sequence Analysis. Soft Tissue Neoplasms / genetics
  • [MeSH-minor] Adenovirus E1A Proteins / genetics. Adult. Aged. Amino Acid Oxidoreductases / genetics. Antigens, Neoplasm / genetics. Chromosomes, Human, Pair 17. Chromosomes, Human, Pair 8. DNA Topoisomerases, Type II / genetics. DNA-Binding Proteins / genetics. Female. Gene Expression Regulation, Enzymologic. Humans. Inhibitor of Apoptosis Proteins. Male. Microtubule-Associated Proteins / genetics. Middle Aged. Neoplasm Proteins / genetics. Prognosis. Proto-Oncogene Proteins / genetics. Reverse Transcriptase Polymerase Chain Reaction

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  • (PMID = 18522746.001).
  • [ISSN] 1476-4598
  • [Journal-full-title] Molecular cancer
  • [ISO-abbreviation] Mol. Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Adenovirus E1A Proteins; 0 / Antigens, Neoplasm; 0 / BIRC5 protein, human; 0 / DNA, Neoplasm; 0 / DNA-Binding Proteins; 0 / ETV4 protein, human; 0 / Inhibitor of Apoptosis Proteins; 0 / Microtubule-Associated Proteins; 0 / Neoplasm Proteins; 0 / Proto-Oncogene Proteins; EC 1.4.- / Amino Acid Oxidoreductases; EC 1.4.3.- / LOXL2 protein, human; EC 5.99.1.3 / DNA Topoisomerases, Type II; EC 5.99.1.3 / DNA topoisomerase II alpha
  • [Other-IDs] NLM/ PMC2442610
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3. Aoki M, Nabeshima K, Koga K, Hamasaki M, Suzumiya J, Tamura K, Iwasaki H: Imatinib mesylate inhibits cell invasion of malignant peripheral nerve sheath tumor induced by platelet-derived growth factor-BB. Lab Invest; 2007 Aug;87(8):767-79
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  • [Title] Imatinib mesylate inhibits cell invasion of malignant peripheral nerve sheath tumor induced by platelet-derived growth factor-BB.
  • Malignant peripheral nerve sheath tumor (MPNST) is rare, highly aggressive, resistant to radiochemotherapy, and associated with poor prognosis.
  • This study was designed to identify motogenic factor(s) involved in MPNST cell invasion and inhibitor(s) of such invasive activity.
  • We profiled the invasion-inducing activities of eight motogenic growth factors on two human MPNST cell lines, FU-SFT8611 and 9817, using in vitro Matrigel invasion assays.
  • Platelet-derived growth factor-BB (PDGF-BB) was identified as the most effective MPNST cell invasion-inducing factor.
  • Epidermal growth factor (EGF) and hepatocyte growth factor (HGF) also stimulated invasion in one MPNST cell line.
  • Expressions of PDGF-BB and EGF receptors (PDGFR-beta and EGFR) mRNAs were detected more frequently and their proteins were expressed at higher levels in MPNST tissues than benign peripheral nerve sheath tumors (schwannomas and neurofibromas).
  • In both MPNST cell lines, PDGF-BB induced tyrosine phosphorylation of PDGFR-beta but not of PDGFR-alpha, and specific PDGFR-beta inhibition by small interfering RNA to the receptor inhibited PDGF-BB-stimulated MPNST cell invasion, suggesting the predominant role of PDGFR-beta.
  • No mutations were present in exons 12 and 18 of PDGFR-beta in both MPNST cell lines and 10 human MPNST tissues examined.
  • Our results indicated that PDGF-BB enhanced the invasive activity of MPNST cells through PDGFR phosphorylation and that imatinib inhibited such activity.
  • The results provide the ground for further assessment of the therapeutic potential of imatinib in suppressing the invasion and growth of MPNST.
  • [MeSH-major] Antineoplastic Agents / pharmacology. Nerve Sheath Neoplasms / metabolism. Piperazines / pharmacology. Platelet-Derived Growth Factor / pharmacology. Pyrimidines / pharmacology
  • [MeSH-minor] Adult. Benzamides. Cell Line, Tumor. Female. Humans. Imatinib Mesylate. Intercellular Signaling Peptides and Proteins / pharmacology. Intercellular Signaling Peptides and Proteins / physiology. Male. Middle Aged. Mutation. Neoplasm Invasiveness. Phosphorylation. Proto-Oncogene Proteins c-sis. RNA, Messenger / metabolism. Receptor, Platelet-Derived Growth Factor beta / antagonists & inhibitors. Receptor, Platelet-Derived Growth Factor beta / genetics. Receptor, Platelet-Derived Growth Factor beta / metabolism. Receptors, Growth Factor / genetics. Receptors, Growth Factor / metabolism

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  • (PMID = 17558420.001).
  • [ISSN] 0023-6837
  • [Journal-full-title] Laboratory investigation; a journal of technical methods and pathology
  • [ISO-abbreviation] Lab. Invest.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Benzamides; 0 / Intercellular Signaling Peptides and Proteins; 0 / Piperazines; 0 / Platelet-Derived Growth Factor; 0 / Proto-Oncogene Proteins c-sis; 0 / Pyrimidines; 0 / RNA, Messenger; 0 / Receptors, Growth Factor; 0 / platelet-derived growth factor BB; 8A1O1M485B / Imatinib Mesylate; EC 2.7.10.1 / Receptor, Platelet-Derived Growth Factor beta
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4. Karube K, Nabeshima K, Ishiguro M, Harada M, Iwasaki H: cDNA microarray analysis of cancer associated gene expression profiles in malignant peripheral nerve sheath tumours. J Clin Pathol; 2006 Feb;59(2):160-5
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  • [Title] cDNA microarray analysis of cancer associated gene expression profiles in malignant peripheral nerve sheath tumours.
  • BACKGROUND: Malignant peripheral nerve sheath tumour (MPNST) is a highly aggressive malignancy that arises within peripheral nerves, and is associated with poor prognosis.
  • Little is known about the underlying biology of MPNST, especially the mechanisms involved in cell proliferation, invasion, or escape from apoptosis.
  • AIMS: To identify genes differentially expressed in MPNST compared with benign tumours, such as neurofibromas and schwannomas, by means of cDNA microarray analysis.
  • METHODS: Six MPNST cases and five benign cases (three schwannomas and two neurofibromas) were analysed.
  • RESULTS: Six genes (keratin 18, survivin, tenascin C, adenosine deaminase, collagen type VIa3, and collagen type VIIa1) were significantly upregulated in MPNST, whereas one gene, insulin-like growth factor binding protein 6, was downregulated in MPNST.
  • Immunohistochemistry confirmed upregulation of survivin in MPNST at the protein level in six of eight cases compared with benign tumours.
  • Tenascin C was also expressed at the invasive front and tumorous stroma in all MPNST cases.
  • MPNST cells expressed tenascin C in four of nine cases.
  • CONCLUSIONS: Survivin and tenascin C may be associated with the malignant potential of MPNST and could be considered as potential therapeutic targets.
  • [MeSH-major] Gene Expression Regulation, Neoplastic. Neoplasm Proteins / biosynthesis. Nerve Sheath Neoplasms / metabolism. Peripheral Nervous System Neoplasms / metabolism
  • [MeSH-minor] Adult. Aged. Aged, 80 and over. Female. Gene Expression Profiling / methods. Humans. Immunoenzyme Techniques. Inhibitor of Apoptosis Proteins. Male. Microtubule-Associated Proteins / biosynthesis. Microtubule-Associated Proteins / genetics. Middle Aged. Neurilemmoma / genetics. Neurilemmoma / metabolism. Neurilemmoma / pathology. Neurofibroma / genetics. Neurofibroma / metabolism. Neurofibroma / pathology. Oligonucleotide Array Sequence Analysis / methods. Reverse Transcriptase Polymerase Chain Reaction / methods. Tenascin / biosynthesis. Tenascin / genetics. Up-Regulation

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  • (PMID = 16443732.001).
  • [ISSN] 0021-9746
  • [Journal-full-title] Journal of clinical pathology
  • [ISO-abbreviation] J. Clin. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / BIRC5 protein, human; 0 / Inhibitor of Apoptosis Proteins; 0 / Microtubule-Associated Proteins; 0 / Neoplasm Proteins; 0 / Tenascin
  • [Other-IDs] NLM/ PMC1860323
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5. Izycka-Swieszewska E, Drogoszewska B, Filipowicz J, Szurowska E, Kaminski M, Jaskiewicz K: Epithelioid malignant peripheral nerve sheath tumor involving maxillary sinus. Neuropathology; 2005 Dec;25(4):341-5
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  • [Title] Epithelioid malignant peripheral nerve sheath tumor involving maxillary sinus.
  • We present a case of epithelioid malignant peripheral nerve sheath tumor (MPNST) located in the maxillary sinus region in a young man.
  • Histologically, the neoplasm had a predominant epithelioid component.
  • An appropriate immunohistochemical panel was essential for the final diagnosis of this epithelioid malignant tumor, with the location rather unusual for MPNST.
  • [MeSH-major] Maxillary Sinus Neoplasms / pathology. Nerve Sheath Neoplasms / pathology
  • [MeSH-minor] Adult. Carcinoma / pathology. Diagnosis, Differential. Fatal Outcome. Humans. Immunohistochemistry. Male

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  • (PMID = 16382783.001).
  • [ISSN] 0919-6544
  • [Journal-full-title] Neuropathology : official journal of the Japanese Society of Neuropathology
  • [ISO-abbreviation] Neuropathology
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Australia
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6. 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.
  • A computed tomography scan revealed a giant tumor which displaced the bladder and segments of the intestine.
  • The histopathological diagnosis was MPNST.
  • [MeSH-major] Constipation / etiology. Nerve Sheath Neoplasms / complications. Neurofibromatosis 1 / complications. Urinary Retention / etiology
  • [MeSH-minor] Adult. Fatal Outcome. Humans. Male

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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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7. Santarius T, Chia HL, Xuereb JH, Kirollos RW: Sporadic malignant nerve sheath tumour of the oculomotor nerve. Acta Neurochir (Wien); 2007 Jun;149(6):617-22; discussion 622

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Sporadic malignant nerve sheath tumour of the oculomotor nerve.
  • Malignant peripheral nerve sheath tumours (MPNST) are exceedingly rare in an intracranial location.
  • In this report clinical and pathological evidence for the diagnosis of a MPNST arising from of the oclumotor nerve is presented.
  • [MeSH-major] Cranial Nerve Neoplasms / surgery. Nerve Sheath Neoplasms / surgery. Oculomotor Nerve Diseases / surgery
  • [MeSH-minor] Adult. Biomarkers, Tumor / analysis. Decompression, Surgical. Diagnosis, Differential. Diplopia / etiology. Female. Headache / etiology. Humans. Magnetic Resonance Angiography. Magnetic Resonance Imaging. Microsurgery. Neoplasm Recurrence, Local / diagnosis. Neoplasm Recurrence, Local / pathology. Neoplasm Recurrence, Local / surgery. Neoplasm, Residual / diagnosis. Neoplasm, Residual / surgery. Ophthalmoplegia / etiology. Postoperative Complications / diagnosis. Postoperative Complications / surgery. Radiosurgery. Reoperation

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  • (PMID = 17514351.001).
  • [ISSN] 0942-0940
  • [Journal-full-title] Acta neurochirurgica
  • [ISO-abbreviation] Acta Neurochir (Wien)
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Austria
  • [Chemical-registry-number] 0 / Biomarkers, Tumor
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8. Subramanian S, Thayanithy V, West RB, Lee CH, Beck AH, Zhu S, Downs-Kelly E, Montgomery K, Goldblum JR, Hogendoorn PC, Corless CL, Oliveira AM, Dry SM, Nielsen TO, Rubin BP, Fletcher JA, Fletcher CD, van de Rijn M: Genome-wide transcriptome analyses reveal p53 inactivation mediated loss of miR-34a expression in malignant peripheral nerve sheath tumours. J Pathol; 2010 Jan;220(1):58-70
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  • [Title] Genome-wide transcriptome analyses reveal p53 inactivation mediated loss of miR-34a expression in malignant peripheral nerve sheath tumours.
  • Malignant peripheral nerve sheath tumours (MPNSTs) are aggressive soft tissue tumours that occur either sporadically or in patients with neurofibromatosis type 1.
  • The malignant transformation of the benign neurofibroma to MPNST is incompletely understood at the molecular level.
  • We have determined the gene expression signature for benign and malignant PNSTs and found that the major trend in malignant transformation from neurofibroma to MPNST consists of the loss of expression of a large number of genes, rather than widespread increase in gene expression.
  • Subsequent microRNA profiling of benign and malignant PNSTs indicated a relative down-regulation of miR-34a in most MPNSTs compared to neurofibromas.
  • In vitro studies using the cell lines MPNST-14 (NF1 mutant) and MPNST-724 (from a non-NF1 individual) show that exogenous expression of p53 or miR-34a promotes apoptotic cell death.
  • In addition, exogenous expression of p53 in MPNST cells induces miR-34a and other miRNAs.
  • Our data show that p53 inactivation and subsequent loss of expression of miR-34a may significantly contribute to the MPNST development.
  • Collectively, our findings suggest that deregulation of miRNAs has a potential role in the malignant transformation process in peripheral nerve sheath tumours.
  • [MeSH-major] Genes, p53. MicroRNAs / metabolism. Nerve Sheath Neoplasms / genetics. RNA, Neoplasm / metabolism
  • [MeSH-minor] Adult. Apoptosis / genetics. Cell Adhesion / genetics. Cell Proliferation. Cluster Analysis. Down-Regulation. Female. Gene Expression Profiling / methods. Gene Expression Regulation, Neoplastic. Gene Silencing. Humans. Male. Middle Aged. Neoplasm Proteins / genetics. Neoplasm Proteins / metabolism. Neurofibroma. Oligonucleotide Array Sequence Analysis / methods. Receptor, Epidermal Growth Factor / genetics. Receptor, Epidermal Growth Factor / metabolism. Reverse Transcriptase Polymerase Chain Reaction / methods. Signal Transduction / genetics. Tumor Cells, Cultured. Tumor Suppressor Protein p53 / metabolism

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  • (PMID = 19890883.001).
  • [ISSN] 1096-9896
  • [Journal-full-title] The Journal of pathology
  • [ISO-abbreviation] J. Pathol.
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / P50 CA127003; United States / NCI NIH HHS / CA / P50 CA127003-03
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, Non-P.H.S.
  • [Publication-country] England
  • [Chemical-registry-number] 0 / MIRN34 microRNA, human; 0 / MicroRNAs; 0 / Neoplasm Proteins; 0 / RNA, Neoplasm; 0 / Tumor Suppressor Protein p53; EC 2.7.10.1 / EGFR protein, human; EC 2.7.10.1 / Receptor, Epidermal Growth Factor
  • [Other-IDs] NLM/ NIHMS212333; NLM/ PMC4058327
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9. 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
  • [MeSH-minor] Adult. Doxorubicin / administration & dosage. Humans. Ifosfamide / administration & dosage. Male

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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] Spain
  • [Chemical-registry-number] 80168379AG / Doxorubicin; UM20QQM95Y / Ifosfamide
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10. Tucker T, Wolkenstein P, Revuz J, Zeller J, Friedman JM: Association between benign and malignant peripheral nerve sheath tumors in NF1. Neurology; 2005 Jul 26;65(2):205-11

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Association between benign and malignant peripheral nerve sheath tumors in NF1.
  • OBJECTIVE: People with neurofibromatosis type 1 (NF1) have a 10% lifetime risk of developing a malignant peripheral nerve sheath tumor (MPNST).
  • However, it is not known whether an individual's risk of developing an MPNST is associated with the burden of benign neurofibromas.
  • CONCLUSIONS: The observation that malignant peripheral nerve sheath tumors are strongly associated with internal plexiform neurofibromas suggests that patients with neurofibromatosis type 1 with these benign tumors warrant increased surveillance for malignancy.
  • [MeSH-major] Nerve Sheath Neoplasms / epidemiology. Neurofibroma, Plexiform / epidemiology. Neurofibromatosis 1 / epidemiology. Peripheral Nerves / pathology
  • [MeSH-minor] Adolescent. Adult. Comorbidity. Cross-Sectional Studies. Female. Follow-Up Studies. Humans. Life Expectancy. Logistic Models. Male. Middle Aged. Neoplasm Metastasis / pathology. Neoplasm Metastasis / physiopathology. Prevalence. Prognosis. Risk Factors. Survival Rate. Tomography, X-Ray Computed / adverse effects. Tomography, X-Ray Computed / standards. Ultrasonography / standards

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  • (PMID = 16043787.001).
  • [ISSN] 1526-632X
  • [Journal-full-title] Neurology
  • [ISO-abbreviation] Neurology
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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11. Bottillo I, Ahlquist T, Brekke H, Danielsen SA, van den Berg E, Mertens F, Lothe RA, Dallapiccola B: Germline and somatic NF1 mutations in sporadic and NF1-associated malignant peripheral nerve sheath tumours. J Pathol; 2009 Apr;217(5):693-701
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  • [Title] Germline and somatic NF1 mutations in sporadic and NF1-associated malignant peripheral nerve sheath tumours.
  • Malignant peripheral nerve sheath tumours (MPNSTs) are a malignancy occurring with increased frequency in patients with neurofibromatosis type 1 (NF1).
  • In addition, DNA from peripheral blood and cutaneous neurofibroma biopsies from, respectively, 14/25 and 7/25 of the NF1 patients were analysed.
  • The spectrum of germline NF1 mutations in neurofibromatosis patients with MPNST is different from the spectrum of somatic mutations seen in MPNSTs.
  • [MeSH-major] Germ-Line Mutation. Nerve Sheath Neoplasms / genetics. Neurofibromatosis 1 / genetics
  • [MeSH-minor] Adolescent. Adult. Aged. Child. Chromatography, High Pressure Liquid / methods. DNA, Neoplasm / genetics. Female. Genetic Predisposition to Disease. Humans. Male. Middle Aged. Neoplasm Proteins / genetics. Point Mutation. Proto-Oncogene Proteins / genetics. Proto-Oncogene Proteins B-raf / genetics. ras Proteins / genetics

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  • (PMID = 19142971.001).
  • [ISSN] 1096-9896
  • [Journal-full-title] The Journal of pathology
  • [ISO-abbreviation] J. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / DNA, Neoplasm; 0 / KRAS protein, human; 0 / Neoplasm Proteins; 0 / Proto-Oncogene Proteins; EC 2.7.11.1 / BRAF protein, human; EC 2.7.11.1 / Proto-Oncogene Proteins B-raf; EC 3.6.5.2 / ras Proteins
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12. Snyder LA, Linder KE, Neel JA: Malignant peripheral nerve sheath tumor in a hamster. J Am Assoc Lab Anim Sci; 2007 Nov;46(6):55-7
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  • [Title] Malignant peripheral nerve sheath tumor in a hamster.
  • An adult female golden hamster (Mesocricetus auratus) developed a firm subcutaneous mass on the lateral distal right forelimb that progressed to diffuse limb enlargement accompanied by extensive cutaneous ulceration and drainage and axillary lymph node metastasis.
  • On histology, the invasive neoplasm merged with a large subcutaneous nerve and was composed of spindle cells with a high mitotic index, and lymph node metastasis was confirmed.
  • Histologic morphology and positive immunohistochemical staining of neoplastic cells for vimentin, S100, and neuron-specific enolase were consistent with a malignant peripheral nerve sheath tumor.
  • Although relatively common in dogs, peripheral nerve sheath tumors had not been reported previously in hamsters.
  • [MeSH-major] Mesocricetus. Nerve Sheath Neoplasms / veterinary. Soft Tissue Neoplasms / veterinary

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  • (PMID = 17994674.001).
  • [ISSN] 1559-6109
  • [Journal-full-title] Journal of the American Association for Laboratory Animal Science : JAALAS
  • [ISO-abbreviation] J. Am. Assoc. Lab. Anim. Sci.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / S100 Proteins; 0 / Vimentin; EC 4.2.1.11 / Phosphopyruvate Hydratase
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13. Upadhyaya M, Kluwe L, Spurlock G, Monem B, Majounie E, Mantripragada K, Ruggieri M, Chuzhanova N, Evans DG, Ferner R, Thomas N, Guha A, Mautner V: Germline and somatic NF1 gene mutation spectrum in NF1-associated malignant peripheral nerve sheath tumors (MPNSTs). Hum Mutat; 2008 Jan;29(1):74-82
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Germline and somatic NF1 gene mutation spectrum in NF1-associated malignant peripheral nerve sheath tumors (MPNSTs).
  • About 10% of neurofibromatosis type 1 (NF1) patients develop malignant peripheral nerve sheath tumors (MPNSTs) and represent considerable patient morbidity and mortality.
  • Elucidation of the genetic mechanisms by which inherited and acquired NF1 disease gene variants lead to MPNST development is important.
  • The NF1 germline mutation spectrum was similar to that previously identified in adult NF1 patients without MPNST.
  • Somatic NF1 mutations were identified in tumor DNA from 31 out of 34 MPNSTs, of which 28 were large genomic deletions.
  • The high prevalence (>90%) of such deletions in MPNST contrast with the =or<20% found in benign neurofibromas and is indicative of the involvement of different mutational mechanisms in these tumors.
  • [MeSH-major] Germ-Line Mutation. Mutation. Nerve Sheath Neoplasms / genetics. Neurofibromin 1 / genetics. Peripheral Nervous System Neoplasms / genetics
  • [MeSH-minor] Adult. DNA Mutational Analysis. DNA, Neoplasm / metabolism. Humans. Loss of Heterozygosity. Lymphocytes / metabolism. Sequence Deletion. Tumor Suppressor Protein p53 / genetics. Tumor Suppressor Protein p53 / metabolism

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  • [Copyright] (c) 2007 Wiley-Liss, Inc.
  • (PMID = 17960768.001).
  • [ISSN] 1098-1004
  • [Journal-full-title] Human mutation
  • [ISO-abbreviation] Hum. Mutat.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / DNA, Neoplasm; 0 / Neurofibromin 1; 0 / Tumor Suppressor Protein p53
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14. Keizman D, Issakov J, Meller I, Maimon N, Ish-Shalom M, Sher O, Merimsky O: Expression and significance of EGFR in malignant peripheral nerve sheath tumor. J Neurooncol; 2009 Sep;94(3):383-8
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  • [Title] Expression and significance of EGFR in malignant peripheral nerve sheath tumor.
  • Malignant peripheral nerve sheath tumor (MPNST) is an aggressive sarcoma.
  • The study was aimed at investigating the expression and prognostic influence of EGFR in MPNST.
  • Forty-three percentage of 46 patients with MPNST overexpressed EGFR in the primary tumor, and had a higher prevalence of advanced-stage tumors (>or=IIc, 46% vs. 80%, P = 0.011).
  • EGFR appeared to play a role in MPNST progression.
  • Clinical trials of targeting EGFR in MPNST are warranted.
  • [MeSH-major] Nerve Sheath Neoplasms / metabolism. Receptor, Epidermal Growth Factor / metabolism
  • [MeSH-minor] Adult. Cohort Studies. Female. Humans. Logistic Models. Male. Middle Aged. Multivariate Analysis. Neoplasm Staging. Prognosis. Retrospective Studies. Young Adult

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  • [ErratumIn] J Neurooncol. 2009 Sep;94(3):389. Meimon, Natalie [corrected to Maimon, Natalie]
  • (PMID = 19330289.001).
  • [ISSN] 1573-7373
  • [Journal-full-title] Journal of neuro-oncology
  • [ISO-abbreviation] J. Neurooncol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] EC 2.7.10.1 / Receptor, Epidermal Growth Factor
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15. Rodriguez AO, Truskinovsky AM, Kasrazadeh M, Leiserowitz GS: Case report: Malignant peripheral nerve sheath tumor of the uterine cervix treated with radical vaginal trachelectomy. Gynecol Oncol; 2006 Jan;100(1):201-4
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Case report: Malignant peripheral nerve sheath tumor of the uterine cervix treated with radical vaginal trachelectomy.
  • BACKGROUND: Malignant peripheral nerve sheath tumors (MPNST) are rare tumors which occur primarily in major nerve trunks and most commonly in patients with neurofibromatosis.
  • CASE: We report a 22-year-old woman with MPNST of the uterine cervix which recurred after loop electrocautery excision and had positive margins on cone biopsy.
  • CONCLUSIONS: MPNST of the uterine cervix is a rare, but potentially aggressive neoplasm.
  • This is the first case of such a tumor treated successfully with preservation of the patients fertility.
  • [MeSH-major] Neoplasm Recurrence, Local / surgery. Nerve Sheath Neoplasms / surgery. Uterine Cervical Neoplasms / surgery
  • [MeSH-minor] Adult. Female. Fertility. Gynecologic Surgical Procedures. Humans


16. Zou C, Smith KD, Liu J, Lahat G, Myers S, Wang WL, Zhang W, McCutcheon IE, Slopis JM, Lazar AJ, Pollock RE, Lev D: Clinical, pathological, and molecular variables predictive of malignant peripheral nerve sheath tumor outcome. Ann Surg; 2009 Jun;249(6):1014-22
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Clinical, pathological, and molecular variables predictive of malignant peripheral nerve sheath tumor outcome.
  • OBJECTIVE: Improved staging systems for malignant peripheral nerve sheath tumor (MPNST) prognostication and management are needed.
  • Consequently, we sought to identify clinical, pathologic, and molecular predictors of outcome in patients with/without neurofibromatosis type 1 (NF-1) associated MPNST.
  • METHODS: MPNST patients treated from 1986 to 2006 (n = 140) were identified; 72 had NF-1 syndrome and 68 did not.
  • Paraffin-embedded neurofibroma or MPNST blocks were assembled in a tissue microarray; marker expression was evaluated immunohistochemically.
  • The 5 years DSS for localized tumor patients was 35% for NF-1 patients and 50% for sporadic patients.
  • MPNST >or=10 cm at diagnosis, partial resection, and metastasis development were significant negative predictors of DSS; completely resected tumors that lacked S-100 immunoreactivity had a nearly 5-fold increased risk of developing distant metastasis.
  • Ki67, vascular endothelial growth factor, p53, and pMEK were over-expressed in MPNST compared with benign neurofibromas.
  • Only tumor size and nuclear p53 expression were found to be independent prognosticators for MPNST DSS in a multivariable analysis.
  • CONCLUSIONS: MPSNT is a markedly metastatic and aggressive poor prognosis tumor.
  • Multiple clinical, pathologic, and molecular markers identified in this study, coupled with findings from previous series, should be considered for an improved MPNST staging system useful for prognostic assessment and management decisions.
  • [MeSH-major] Neoplasm Recurrence, Local / epidemiology. Nerve Sheath Neoplasms / metabolism. Nerve Sheath Neoplasms / pathology
  • [MeSH-minor] Adolescent. Adult. Aged. Aged, 80 and over. Biomarkers / metabolism. Child. Child, Preschool. Cohort Studies. Disease-Free Survival. Female. Humans. Infant. Male. Middle Aged. Neurofibromatosis 1 / complications. Retrospective Studies. Risk Factors. Survival Rate. Treatment Outcome. Young Adult

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  • (PMID = 19474676.001).
  • [ISSN] 1528-1140
  • [Journal-full-title] Annals of surgery
  • [ISO-abbreviation] Ann. Surg.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers
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17. Yildirim G, Gillenwater AM, Ordonez NG, Garden AS, El-Naggar AK: Concurrent epithelioid malignant peripheral nerve sheath tumor and papillary thyroid carcinoma in the treated field of Hodgkin's disease. Head Neck; 2008 May;30(5):675-9
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  • [Title] Concurrent epithelioid malignant peripheral nerve sheath tumor and papillary thyroid carcinoma in the treated field of Hodgkin's disease.
  • A 1.5-cm papillary thyroid carcinoma was identified in thyroidectomy and an initial diagnosis of undifferentiated malignant neoplasm was rendered on the neck mass biopsy.
  • Subsequent surgical excision of the neck mass and immunohistochemical analysis revealed malignant peripheral nerve sheath tumor.
  • Rare malignancies including malignant peripheral nerve sheath tumor may be encountered along with the more common papillary thyroid carcinoma.
  • [MeSH-major] Carcinoma, Papillary / pathology. Neoplasms, Second Primary / pathology. Nerve Sheath Neoplasms / pathology. Peripheral Nervous System Neoplasms / pathology. Thyroid Neoplasms / pathology
  • [MeSH-minor] Adult. Epithelioid Cells / pathology. Female. Hodgkin Disease / radiotherapy. Humans. Neoplasms, Radiation-Induced / pathology. Neoplasms, Radiation-Induced / surgery. Thyroidectomy

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  • (PMID = 17972308.001).
  • [ISSN] 1043-3074
  • [Journal-full-title] Head & neck
  • [ISO-abbreviation] Head Neck
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / P30 CA016672
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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18. Ziadi A, Saliba I: Malignant peripheral nerve sheath tumor of intracranial nerve: a case series review. Auris Nasus Larynx; 2010 Oct;37(5):539-45
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  • [Title] Malignant peripheral nerve sheath tumor of intracranial nerve: a case series review.
  • OBJECTIVES: The incidence of malignant peripheral nerve sheath tumor (MPNST) is approximately 0.001%.
  • (1) to review all cases of intracranial MPNST described in the literature, (2) to highlight the suspicion of intracranial MPNST, (3) to identify the gross pathology, the histopathology, the immunohistochemistry, (4) to discuss the differential diagnosis, the treatment, the recurrence rate, the follow-up, the incidence of metastasis and the prognosis.
  • We used the following Keywords: "malignant peripheral nerve sheath tumor", "cranial nerve", "neurosarcoma", "malignant schwannoma", "neurofibroma", "malignant neurofibroma" and "nerve tumor".
  • We considered cases where MPNST involved an intracranial cranial nerve.
  • RESULTS: We identified 32 cases of cranial MPNST including our case.
  • Most cases are developed sporadically (50%), 31% arise from a malignant transformation of schwannoma and 19% from a neurofibroma.
  • The cranial nerve VIII is the most involved (15/32), followed by the Vth (10/32) and the VIIth (5/32).
  • MPNST will strongly express protein S-100 and collagen IV-laminin.
  • 13 cases were treated with radiotherapy for tumor recurrence and metastasis.
  • CONCLUSION: MPNST of cranial nerves are very rare.
  • In neurofibroma, even though MPNST is mainly associated to type 1, we should keep in mind its association to NF2.
  • Inaccessibility of cranial MPNST may explain the subtotal resection and thus the poor prognosis.
  • [MeSH-major] Cranial Nerve Neoplasms / diagnosis. Nerve Sheath Neoplasms / diagnosis
  • [MeSH-minor] Adolescent. Adult. Aged. Cell Transformation, Neoplastic / pathology. Child. Child, Preschool. Diagnosis, Differential. Disease-Free Survival. Female. Follow-Up Studies. Humans. Male. Middle Aged. Neoplasm Recurrence, Local / mortality. Neoplasm Recurrence, Local / pathology. Neurilemmoma / diagnosis. Neurilemmoma / pathology. Neurilemmoma / radiotherapy. Neurilemmoma / surgery. Neurofibroma / diagnosis. Neurofibroma / pathology. Neurofibroma / radiotherapy. Neurofibroma / surgery. Neurofibromatosis 1 / diagnosis. Neurofibromatosis 1 / pathology. Neurofibromatosis 1 / radiotherapy. Neurofibromatosis 1 / surgery. Neurofibromatosis 2 / diagnosis. Neurofibromatosis 2 / pathology. Neurofibromatosis 2 / radiotherapy. Neurofibromatosis 2 / surgery. Radiotherapy, Adjuvant. Spinal Neoplasms / mortality. Spinal Neoplasms / pathology. Spinal Neoplasms / secondary. Young Adult

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  • [Copyright] Copyright 2010 Elsevier Ireland Ltd. All rights reserved.
  • (PMID = 20399579.001).
  • [ISSN] 1879-1476
  • [Journal-full-title] Auris, nasus, larynx
  • [ISO-abbreviation] Auris Nasus Larynx
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] Netherlands
  • [Number-of-references] 35
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19. Aoki M, Nabeshima K, Nishio J, Ishiguro M, Fujita C, Koga K, Hamasaki M, Kaneko Y, Iwasaki H: Establishment of three malignant peripheral nerve sheath tumor cell lines, FU-SFT8611, 8710 and 9817: conventional and molecular cytogenetic characterization. Int J Oncol; 2006 Dec;29(6):1421-8
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  • [Title] Establishment of three malignant peripheral nerve sheath tumor cell lines, FU-SFT8611, 8710 and 9817: conventional and molecular cytogenetic characterization.
  • Malignant peripheral nerve sheath tumor (MPNST) is a rare malignant tumor, for which only a few cultured cell lines are available to date.
  • In the present study, we established three new MPNST cell lines, FU-SFT8611, FU-SFT8710 and FU-SFT9817, from a 40-year-old Japanese man without neurofibromatosis 1 (NF1), a 43-year-old Japanese woman with NF1, and a 61-year-old Japanese woman without NF1, respectively.
  • These newly established cell lines provide a valuable resource for biological and pathological investigations into new treatment regimes for MPNST.
  • [MeSH-major] Cell Line, Tumor. Nerve Sheath Neoplasms / genetics
  • [MeSH-minor] Adult. Animals. Cell Growth Processes / physiology. Cytogenetic Analysis / methods. Female. Humans. Immunohistochemistry. Karyotyping. Male. Mice. Mice, Inbred BALB C. Mice, Nude. Mice, SCID. Middle Aged. Neoplasm Transplantation. Neurofibromatosis 1 / genetics. Neurofibromatosis 1 / pathology. Nucleic Acid Hybridization. Transplantation, Heterologous

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  • (PMID = 17088980.001).
  • [ISSN] 1019-6439
  • [Journal-full-title] International journal of oncology
  • [ISO-abbreviation] Int. J. Oncol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Greece
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20. 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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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [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.
  • 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.
  • [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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21. Storlazzi CT, Brekke HR, Mandahl N, Brosjö O, Smeland S, Lothe RA, Mertens F: Identification of a novel amplicon at distal 17q containing the BIRC5/SURVIVIN gene in malignant peripheral nerve sheath tumours. J Pathol; 2006 Aug;209(4):492-500
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Identification of a novel amplicon at distal 17q containing the BIRC5/SURVIVIN gene in malignant peripheral nerve sheath tumours.
  • Previous studies have suggested that amplification of genes, notably the TOP2A gene, on chromosome arm 17q may be important for the development of malignant peripheral nerve sheath tumour (MPNST).
  • Increased copy numbers were seen for the ERBB2 and TOP2A genes in eight and nine cases, respectively, supporting a potential role for these two genes in MPNST tumourigenesis.
  • Among the genes mapping to this region, BIRC5, which encodes the baculoviral IAP repeat-containing protein 5/survivin protein, is a strong candidate target gene for amplification, as it has been previously shown to be overexpressed in neurofibromatosis type 1-associated MPNST.
  • Three other genes that co-amplified with BIRC5 represent other potential candidate genes: PTDSR involved in apoptosis; SEPT9 overexpressed in human malignant brain tumours; and SOCS3 involved in cell survival and differentiation of neurons.
  • [MeSH-major] Chromosomes, Human, Pair 17. Genes, Neoplasm. Microtubule-Associated Proteins / genetics. Neoplasm Proteins / genetics. Nerve Sheath Neoplasms / genetics. Sarcoma / genetics
  • [MeSH-minor] Adolescent. Adult. Aged. Child. Chromosome Banding. DNA Topoisomerases, Type II / genetics. Female. Follow-Up Studies. Gene Amplification. Gene Dosage. Genes, erbB-2. Humans. In Situ Hybridization, Fluorescence. Inhibitor of Apoptosis Proteins. Interphase. Male. Metaphase. Middle Aged. Neurofibromatosis 1 / genetics. Neurofibromatosis 1 / pathology

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  • [Copyright] Copyright 2006 Pathological Society of Great Britain and Ireland.
  • (PMID = 16721726.001).
  • [ISSN] 0022-3417
  • [Journal-full-title] The Journal of pathology
  • [ISO-abbreviation] J. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / BIRC5 protein, human; 0 / Inhibitor of Apoptosis Proteins; 0 / Microtubule-Associated Proteins; 0 / Neoplasm Proteins; EC 5.99.1.3 / DNA Topoisomerases, Type II
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22. 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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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [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.
  • 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.
  • [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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23. del Carmen Baena-Ocampo L, Reyes-Sánchez A, Alpízar-Aguirre A, Rosales-Olivares LM: Malignant peripheral nerve sheath tumors associated with neurofibromatosis type 1: report of two clinical cases. Cir Cir; 2009 Sep-Oct;77(5):391-5
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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 associated with neurofibromatosis type 1: report of two clinical cases.
  • BACKGROUND: Malignant peripheral nerve sheath tumor (MPNST) is a sarcoma with a high grade of malignancy originating in the nerve sheath components, fibroblasts, perineural cells, and Schwann cells.
  • CLINICAL CASES: We present two cases of NF-1-associated MPNST.
  • Histologically, it corresponded to a neurofibromatosis lesion in transition with malignant neoplasm.
  • Associated with the deformity, MRI showed a withering tumor in the posterior thoracic region (T1-T8), observing an infiltrating, cellular sarcomatous neoplasm with immunopositivity for S-100 protein and vimentin.
  • Due to the progressive growth of MPNST and the anatomic difficulty for its approach, there should be strict surveillance of patients with NF-1 for early detection of malignant transformation in these lesions.
  • [MeSH-major] Cervical Vertebrae. Nerve Sheath Neoplasms / genetics. Neurofibromatosis 1 / pathology. Spinal Neoplasms / genetics. Thoracic Vertebrae
  • [MeSH-minor] Adult. Biomarkers, Tumor / analysis. Fatal Outcome. Female. Humans. Kyphosis / etiology. Laminectomy. Magnetic Resonance Imaging. Male. Neoplasm Recurrence, Local / radiotherapy. Nerve Compression Syndromes / etiology. S100 Proteins / analysis. Scoliosis / etiology. Spinal Nerve Roots. Vimentin / analysis. Young Adult


24. 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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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [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.
  • [MeSH-major] Melanoma / pathology. Nerve Sheath Neoplasms / pathology. Uterine Cervical Neoplasms / pathology
  • [MeSH-minor] Adult. Diagnosis, Differential. Female. Histocytochemistry. Humans


25. Aguirre-Quezada DE, Martínez-Anda JJ, Aguilar-Ayala EL, Chávez-Macías L, Olvera-Rabiela JE: [Intracranial and intramedullary peripheral nerve sheath tumours. Case reports from 20 autopsies]. Rev Neurol; 2006 Aug 16-31;43(4):197-200
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  • [Title] [Intracranial and intramedullary peripheral nerve sheath tumours. Case reports from 20 autopsies].
  • INTRODUCTION: Tumors arising from the sheath of peripheral nerves, both intracranial and intraspinal, are uncommon and are sometimes of difficult clinical diagnosis, especially when they occur in unusual sites.
  • Histological malignancy of this neoplasm is rare.
  • MATERIALS AND METHODS: The clinical and pathological findings of 20 autopsy cases of intracranial and intraspinal peripheral nerve tumors are analyzed.
  • RESULTS: 19 were schwannomas, 13 of the 8th cranial nerve (two associated with neurofibromatosis type 2), two originated in the trigeminal and one in the 12th nerves.
  • Three were intraspinal, one of this underwent malignant changes and was part of neurofibromatosis type 1 (NF-1), another was an intraspinal lumbar mass with schwannomatosis and the third was a case of multiple intraspinal neurofibromas as a part of NF-1.
  • The importance of early detection on intracranial and intraspinal peripheral tumors is paramount, since the large size of these histologically benign neoplasms makes them biologically malignant.
  • [MeSH-major] Brain Neoplasms / pathology. Cranial Nerve Neoplasms / pathology. Nerve Sheath Neoplasms / pathology. Spinal Cord Neoplasms / pathology
  • [MeSH-minor] Adolescent. Adult. Aged. Autopsy. Diagnosis, Differential. Female. Humans. Male. Middle Aged. Neurilemmoma / pathology. Neurofibromatosis 1 / pathology. Neurofibromatosis 2 / pathology. Retrospective Studies

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  • (PMID = 16883507.001).
  • [ISSN] 0210-0010
  • [Journal-full-title] Revista de neurologia
  • [ISO-abbreviation] Rev Neurol
  • [Language] spa
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Spain
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26. 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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  • [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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27. Kobayashi C, Oda Y, Takahira T, Izumi T, Kawaguchi K, Yamamoto H, Tamiya S, Yamada T, Oda S, Tanaka K, Matsuda S, Iwamoto Y, Tsuneyoshi M: Chromosomal aberrations and microsatellite instability of malignant peripheral nerve sheath tumors: a study of 10 tumors from nine patients. Cancer Genet Cytogenet; 2006 Mar;165(2):98-105
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Chromosomal aberrations and microsatellite instability of malignant peripheral nerve sheath tumors: a study of 10 tumors from nine patients.
  • Malignant peripheral nerve sheath tumor (MPNST) is an uncommon soft tissue neoplasm with a poor prognosis, occurring sporadically or associated with neurofibromatosis type 1 (NF1); however, the histogenesis of MPNST remains unclear, especially in sporadic tumors.
  • To elucidate the chromosomal aberration as a consequence of CIN and MSI status of MPNST, we karyotyped 10 MPNSTs from nine patients, and examined the MSI of seven microsatellite markers using high-resolution fluorescence microsatellite analysis; 2 out of 10 cases (20%) had normal karyotypes, and 8 out of 10 cases (80%) revealed structural and numerical chromosomal aberrations.
  • These findings suggest that chromosomal aberration as a consequence of CIN has a greater role in the pathogenesis of MPNST than does that due to MSI.
  • [MeSH-major] Chromosome Aberrations. Microsatellite Repeats / genetics. Nerve Sheath Neoplasms / genetics
  • [MeSH-minor] Adult. Aged. Child, Preschool. Female. Humans. Immunohistochemistry. Infant. Karyotyping. Male. Middle Aged

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  • (PMID = 16527603.001).
  • [ISSN] 0165-4608
  • [Journal-full-title] Cancer genetics and cytogenetics
  • [ISO-abbreviation] Cancer Genet. Cytogenet.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
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28. Anghileri M, Miceli R, Fiore M, Mariani L, Ferrari A, Mussi C, Lozza L, Collini P, Olmi P, Casali PG, Pilotti S, Gronchi A: Malignant peripheral nerve sheath tumors: prognostic factors and survival in a series of patients treated at a single institution. Cancer; 2006 Sep 1;107(5):1065-74

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Malignant peripheral nerve sheath tumors: prognostic factors and survival in a series of patients treated at a single institution.
  • BACKGROUND: The authors explored the prognostic factors and clinical outcomes of patients who had malignant peripheral nerve sheath tumors (MPNST) with and without neurofibromatosis type 1 (NF-1).
  • METHODS: Two hundred five patients with localized MPNST who underwent surgery at the Istituto Nazionale per lo Studio e la Cura dei Tumori (Milan, Italy) over 25 years were reviewed.
  • Presentation with either primary or recurrent disease, tumor size, and tumor site (trunk vs. extremity) were the strongest independent predictors of survival.
  • The results confirmed that patients with MPNST share similar prognostic factors with patients who have other soft tissue sarcomas and have some of the worst clinical outcomes.
  • [MeSH-major] Nerve Sheath Neoplasms / mortality. Neurofibromatosis 1 / complications
  • [MeSH-minor] Adolescent. Adult. Combined Modality Therapy. Disease-Free Survival. Female. Humans. Male. Neoplasm Metastasis. Neoplasm Recurrence, Local. Prognosis. Survival Rate

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  • (PMID = 16881077.001).
  • [ISSN] 0008-543X
  • [Journal-full-title] Cancer
  • [ISO-abbreviation] Cancer
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article
  • [Publication-country] United States
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29. Nepka C, Karadana M, Karasavvidou F, Barbanis S, Kalodimos G, Koukoulis G: Fine needle aspiration cytology of a primary malignant peripheral nerve sheath tumor arising in the parotid gland: a case report. Acta Cytol; 2009 Jul-Aug;53(4):423-6
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  • [Title] Fine needle aspiration cytology of a primary malignant peripheral nerve sheath tumor arising in the parotid gland: a case report.
  • BACKGROUND: Malignant peripheral nerve sheath tumor (MPNST) is an uncommon mesenchymal neoplasm showing nerve sheath differentiation, usually arising in large nerves of the trunk and extremities.
  • We describe fine needle aspiration (FNA) findings in a case of MPNST in the parotid gland.
  • Smears were cellular, with clusters of tightly packed spindle or oval cells arranged in a storiform or whorled pattern, showing clearly malignant features.
  • The background contained abundant necrotic material with dispersed malignant nuclei.
  • Cytologic diagnosis was positive for malignant cells consistent with a spindle cell sarcoma, with morphologic features compatible to neural differentiation, confirmed by histologic examination.
  • CONCLUSION: This case illustrates that attention to moiphologic criteria suggestive of nerve sheath phenotype supported by immunocytochemical data is extremely helpful and reliable in the diagnostic approach to MPNSTs, even in rare locations.
  • [MeSH-major] Biopsy, Fine-Needle. Nerve Sheath Neoplasms / pathology. Parotid Neoplasms / pathology
  • [MeSH-minor] Adult. Diagnosis, Differential. Humans. Male

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  • [CommentIn] Acta Cytol. 2010 Sep-Oct;54(5 Suppl):1073-4 [21053609.001]
  • (PMID = 19697728.001).
  • [ISSN] 0001-5547
  • [Journal-full-title] Acta cytologica
  • [ISO-abbreviation] Acta Cytol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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30. Niwa T, Aida N, Fujita K, Kitagawa N, Sato Y, Tanaka Y, Inoue T: Diffusion-weighted imaging of retroperitoneal malignant peripheral nerve sheath tumor in a patient with neurofibromatosis type 1. Magn Reson Med Sci; 2008;7(1):49-53
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  • [Title] Diffusion-weighted imaging of retroperitoneal malignant peripheral nerve sheath tumor in a patient with neurofibromatosis type 1.
  • We present the diffusion-weighted imaging (DWI) findings for a malignant peripheral nerve sheath tumor arising in a retroperitoneal plexiform neurofibroma in a patient with neurofibromatosis type 1.
  • Signal intensity of the malignant area was high on DWI and low on the apparent diffusion coefficient map and differed from findings for the benign area.
  • DWI enabled clear differentiation between malignant and benign areas of the tumor.
  • [MeSH-major] Diffusion Magnetic Resonance Imaging / methods. Nerve Sheath Neoplasms / diagnosis. Neurofibromatosis 1 / complications. Retroperitoneal Neoplasms / diagnosis
  • [MeSH-minor] Adult. Diagnosis, Differential. Female. Humans. Neoplasm Recurrence, Local. Reoperation


31. Murovic JA, Charles Cho S, Park J: Surgical strategies for managing foraminal nerve sheath tumors: the emerging role of CyberKnife ablation. Eur Spine J; 2010 Feb;19(2):242-56
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Surgical strategies for managing foraminal nerve sheath tumors: the emerging role of CyberKnife ablation.
  • Sixteen Stanford University Medical Center (SUMC) patients with foraminal nerve sheath tumors had charts reviewed.
  • Parameters were evaluated for 16 foraminal nerve sheath tumors undergoing surgery, some with CyberKnife.
  • The malignant peripheral nerve sheath tumor (MPNST) had prior field-radiation and adds another case.
  • The MPNST had a hemi-laminotomy then laminectomy/total facetectomy/fusion, followed by CyberKnife.
  • Of 11 single-operation-peripheral nerve sheath tumors, the asymptomatic case remained stable, nine (92%) improved and one (9%) worsened.
  • The MPNST had presentation improvement after the first operation, worsened and after the second surgery and CyberKnife, the patient expired from tumor spread.
  • In conclusion, surgery is beneficial for pain relief and function preservation in foraminal nerve sheath tumors.
  • [MeSH-major] Laminectomy / methods. Nerve Sheath Neoplasms / surgery. Radiosurgery / methods. Spinal Neoplasms / surgery. Spinal Nerve Roots / surgery. Spine / surgery
  • [MeSH-minor] Adult. Aged. Aged, 80 and over. Dura Mater / pathology. Dura Mater / radiography. Dura Mater / surgery. Female. Humans. Male. Middle Aged. Neoplasm Recurrence, Local. Postoperative Complications. Radiotherapy / methods. Retrospective Studies. Spinal Canal / pathology. Spinal Canal / surgery. Survival Rate. Thoracotomy / methods. Treatment Outcome. Young Adult

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  • (PMID = 19798517.001).
  • [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] Journal Article
  • [Publication-country] Germany
  • [Other-IDs] NLM/ PMC2899818
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32. Kobayashi C, Oda Y, Takahira T, Izumi T, Kawaguchi K, Yamamoto H, Tamiya S, Yamada T, Iwamoto Y, Tsuneyoshi M: Aberrant expression of CHFR in malignant peripheral nerve sheath tumors. Mod Pathol; 2006 Apr;19(4):524-32
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Aberrant expression of CHFR in malignant peripheral nerve sheath tumors.
  • In MPNST, complex karyotypes showing numerical and structural aberrations have been described.
  • We examined the expression of CHFR in 96 cases of MPNST by immunohistochemical and molecular methods.
  • We found reduced (score, < or = 3) expression of CHFR in 63 out of 96 (66%) cases of MPNST, and such alteration was significantly correlated with a high mitotic count, a high Ki-67-labeling index, and a poor prognosis.
  • In addition, MPNST with normal karyotype showed a strong (score, =5) expression of CHFR.
  • Our results support the assertion that CHFR functions as an inhibitor of tumor proliferation.
  • [MeSH-major] Cell Cycle Proteins / genetics. Neoplasm Proteins / genetics. Nerve Sheath Neoplasms / pathology. Soft Tissue Neoplasms / pathology
  • [MeSH-minor] Adolescent. Adult. Aged. Aged, 80 and over. Cell Line, Tumor. Child. Child, Preschool. Female. Gene Expression Regulation, Neoplastic. Humans. Immunohistochemistry. Infant. Ki-67 Antigen / analysis. Male. Middle Aged. Multivariate Analysis. Prognosis. RNA, Messenger / genetics. RNA, Messenger / metabolism. Reverse Transcriptase Polymerase Chain Reaction. Survival Analysis

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  • (PMID = 16554732.001).
  • [ISSN] 0893-3952
  • [Journal-full-title] Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc
  • [ISO-abbreviation] Mod. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / CHFR protein, human; 0 / Cell Cycle Proteins; 0 / Ki-67 Antigen; 0 / Neoplasm Proteins; 0 / RNA, Messenger
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33. Strom T, Kleinschmidt-Demasters BK, Donson A, Foreman NK, Lillehei KO: Rare nerve lesions of non-nerve sheath origin: a 17-year retrospective series. Arch Pathol Lab Med; 2009 Sep;133(9):1391-402
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Rare nerve lesions of non-nerve sheath origin: a 17-year retrospective series.
  • CONTEXT: Peripheral nerve masses are frequently encountered in surgical pathology practice.
  • However, once a peripheral nerve mass is determined not to be a nerve sheath neoplasm, differential diagnostic considerations drop off sharply.
  • OBJECTIVE: To review our experience with surgically resected nerve masses.
  • After elimination of common lesions (mostly nerve sheath tumors), 37 cases (8%) remained, almost all of which were of non-nerve sheath origin: for example, hemangioma, metastatic neuroendocrine pancreatic carcinoma, meningiomas invading nerve fascicles, and primary extrarenal rhabdoid tumor and Ewing sarcoma of nerve.
  • The gene expression pattern of an undifferentiated sarcoma, presenting as ropelike nerve enlargement, clustered with malignant peripheral nerve sheath neoplasms but not other sarcomas or neuroepithelial tumors.
  • CONCLUSIONS: Diverse benign and malignant conditions can affect peripheral nerve.
  • [MeSH-major] Hemangioma / pathology. Meningioma / pathology. Pancreatic Neoplasms / pathology. Peripheral Nervous System Neoplasms / pathology. Rhabdoid Tumor / pathology. Sarcoma, Ewing / pathology
  • [MeSH-minor] Adolescent. Adult. Aged. Diagnosis, Differential. Female. Gene Rearrangement, B-Lymphocyte, Heavy Chain / genetics. Humans. Immunoglobulin Heavy Chains / genetics. In Situ Hybridization, Fluorescence. Male. Middle Aged. Oligonucleotide Array Sequence Analysis. Retrospective Studies. Young Adult

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  • (PMID = 19722745.001).
  • [ISSN] 1543-2165
  • [Journal-full-title] Archives of pathology & laboratory medicine
  • [ISO-abbreviation] Arch. Pathol. Lab. Med.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Immunoglobulin Heavy Chains
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34. Albayrak BS, Gorgulu A, Kose T: A case of intra-dural malignant peripheral nerve sheath tumor in thoracic spine associated with neurofibromatosis type 1. J Neurooncol; 2006 Jun;78(2):187-90
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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 intra-dural malignant peripheral nerve sheath tumor in thoracic spine associated with neurofibromatosis type 1.
  • Histopathological diagnosis was malignant peripheral nerve sheath tumor (MPNST), a rare sarcoma with a dismal prognosis.
  • Tumor recurred in its previous site with an adjacent apical mass in the left lung 7 weeks following initial surgery and repeat surgery was performed with complete removal of intra-dural tumor.
  • We report the first patient with intra-dural MPNST localized proximal to conus medullaris; in upper thoracic spine.
  • It must always be considered the possibility of a rare but a devastating tumor, MPNST beside schwannomas and neurofibromas in patients with NF1 when an intra-spinal mass is diagnosed.
  • [MeSH-major] Nerve Sheath Neoplasms / pathology. Neurofibromatosis 1 / pathology. Spinal Cord Neoplasms / pathology
  • [MeSH-minor] Adult. Dura Mater / pathology. Humans. Male. Neoplasm Recurrence, Local / surgery. Thoracic Vertebrae. Treatment Outcome


35. Ferner RE, Golding JF, Smith M, Calonje E, Jan W, Sanjayanathan V, O'Doherty M: [18F]2-fluoro-2-deoxy-D-glucose positron emission tomography (FDG PET) as a diagnostic tool for neurofibromatosis 1 (NF1) associated malignant peripheral nerve sheath tumours (MPNSTs): a long-term clinical study. Ann Oncol; 2008 Feb;19(2):390-4
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [18F]2-fluoro-2-deoxy-D-glucose positron emission tomography (FDG PET) as a diagnostic tool for neurofibromatosis 1 (NF1) associated malignant peripheral nerve sheath tumours (MPNSTs): a long-term clinical study.
  • BACKGROUND: Malignant peripheral nerve sheath tumours (MPNSTs) are difficult to detect in neurofibromatosis 1 (NF1) individuals.
  • The purpose was to evaluate [(18)F]2-fluoro-2-deoxy-D-glucose positron emission tomography (FDG PET) and PET computed tomography (CT) as a diagnostic tool for MPNST in NF1 patients with symptomatic plexiform neurofibromas and to verify the diagnosis by pathology and clinical follow-up.
  • Qualitative FDG PET and PET CT associated with semi-quantitative maximum standard uptake value (SUVmax) assessed possible malignant change.
  • RESULTS: In all, 116 lesions were detected in 105 patients aged 5-71 years, including 80 plexiform neurofibromas, five atypical neurofibromas, 29 MPNST and two other cancers.
  • Biopsy confirmed the findings in 59 tumours and no MPNST was diagnosed on clinical follow-up of 23 lesions diagnosed as benign on FDG PET and PET CT.
  • CONCLUSION: FDG PET and PET CT is a sensitive and specific diagnostic tool for NF1-associated MPNST.


36. James G, Crocker M, King A, Bodi I, Ibrahim A, Chitnavis BP: Malignant triton tumors of the spine. J Neurosurg Spine; 2008 Jun;8(6):567-73
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Malignant triton tumors of the spine.
  • Malignant triton tumors (MTTs) are malignant peripheral nerve sheath tumors with rhabdomyosarcomatous differentiation.
  • Malignant triton tumors affecting the spine are rare but present special challenges to the neurosurgeon.
  • Nine patients presented with symptoms related to the spinal cord, cauda equina, or nerve root compression.
  • Seven patients had intradural extension of tumor.
  • Malignant triton tumors are rare but should be included in the differential diagnosis of spinal tumors, particularly in patients who have undergone previous radiotherapy or who have neurofibromatosis.
  • [MeSH-major] Neurilemmoma / diagnosis. Spinal Neoplasms / diagnosis
  • [MeSH-minor] Adolescent. Adult. Desmin / analysis. Diagnosis, Differential. Female. Humans. Ki-67 Antigen / analysis. Lumbar Vertebrae / pathology. Male. Neoplasm Recurrence, Local / diagnosis. S100 Proteins / analysis. Sacrum / pathology. Spinal Canal / pathology. Spinal Cord Compression / diagnosis. Thoracic Vertebrae / pathology. Vimentin / analysis

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  • (PMID = 18518679.001).
  • [ISSN] 1547-5654
  • [Journal-full-title] Journal of neurosurgery. Spine
  • [ISO-abbreviation] J Neurosurg Spine
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Desmin; 0 / Ki-67 Antigen; 0 / S100 Proteins; 0 / Vimentin
  • [Number-of-references] 13
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37. Zisis C, Fragoulis S, Rontogianni D, Stratakos G, Bellenis I: Malignant triton tumour of the anterior mediastinum as incidental finding. Monaldi Arch Chest Dis; 2006 Dec;65(4):222-4

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Malignant triton tumour of the anterior mediastinum as incidental finding.
  • A rare case of malignant peripheral nerve sheath tumour with rhabdomyoblastic differentiation (malignant triton tumour) of the anterior mediastinum in a 30-year-old male is reported.

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  • (PMID = 17393668.001).
  • [ISSN] 1122-0643
  • [Journal-full-title] Monaldi archives for chest disease = Archivio Monaldi per le malattie del torace
  • [ISO-abbreviation] Monaldi Arch Chest Dis
  • [Language] ENG
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Italy
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38. Ballas K, Kontoulis TM, Papavasiliou A, Pissas D, Pavlidis T, Katsiki E, Venizelos I, Sakadamis A: A rare case of malignant triton tumor with pluridirectional differentiation. South Med J; 2009 Apr;102(4):435-7
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] A rare case of malignant triton tumor with pluridirectional differentiation.
  • Based on immunohistochemistry, a malignant triton tumor, an uncommon subtype of peripheral nerve sheath tumor with rhabdomyosarcomatous elements, was diagnosed.
  • The important feature of this neoplasm was that it showed pluridirectional differentiation to osteosarcoma and chondrosarcoma.
  • This pathologic finding is rare and seen in only a few cases of all malignant triton tumors.
  • [MeSH-major] Nerve Sheath Neoplasms / pathology. Rhabdomyosarcoma / pathology. Soft Tissue Neoplasms / pathology
  • [MeSH-minor] Adult. Biomarkers, Tumor / analysis. Chondrosarcoma / pathology. Diagnosis, Differential. Humans. Immunohistochemistry. Male. Osteosarcoma / pathology

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  • (PMID = 19279515.001).
  • [ISSN] 1541-8243
  • [Journal-full-title] Southern medical journal
  • [ISO-abbreviation] South. Med. J.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor
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39. 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.
  • [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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40. Sengöz A, Taşdemiroğlu E, Togay H: Is clear cell sarcoma a malignant form of psammomatous melanotic schwannoma? Case report. Neurosurg Focus; 2006;21(6):E11
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Is clear cell sarcoma a malignant form of psammomatous melanotic schwannoma? Case report.
  • The authors present a case of clear cell sarcoma (CCS) in which the tumor originated in the S-1 nerve root and had been previously diagnosed as psammomatous melanotic schwannoma (PMS).
  • This is the third case of a spinal nerve root origin for CCS reported in the English-language literature.
  • The similar histogenesis of CCS and malignant melanoma supports the hypothesis that biological agents or immunotherapy are potentially important areas of investigation.
  • The border of the resection was extended 1 cm distal to the tumor margin.
  • The new histopathological diagnosis was CCS (malignant melanoma of soft tissue).
  • A CCS originating from peripheral nerves is quite rare.
  • [MeSH-major] Neurilemmoma / classification. Peripheral Nervous System Neoplasms / classification. Sarcoma, Clear Cell / classification. Spinal Nerve Roots / pathology
  • [MeSH-minor] Adolescent. Adult. Antigens, Neoplasm. Biomarkers, Tumor / analysis. Breast Neoplasms. Diagnosis, Differential. Diagnostic Errors. Female. Fibroadenoma. Humans. Keratins / analysis. Male. Melanins / analysis. Melanoma-Specific Antigens. Neoplasm Invasiveness. Neoplasm Proteins / analysis. Neoplasm Recurrence, Local. Neoplasms, Multiple Primary. Neoplastic Syndromes, Hereditary / diagnosis. Neoplastic Syndromes, Hereditary / genetics. Nerve Sheath Neoplasms / pathology. Pigmentation Disorders / diagnosis. Pigmentation Disorders / genetics. Prognosis. S100 Proteins / analysis. Sacrococcygeal Region. Syndrome. Vimentin / analysis

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  • (PMID = 17341045.001).
  • [ISSN] 1092-0684
  • [Journal-full-title] Neurosurgical focus
  • [ISO-abbreviation] Neurosurg Focus
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antigens, Neoplasm; 0 / Biomarkers, Tumor; 0 / Melanins; 0 / Melanoma-Specific Antigens; 0 / Neoplasm Proteins; 0 / S100 Proteins; 0 / Vimentin; 68238-35-7 / Keratins
  • [Number-of-references] 17
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41. Subhashraj K, Balanand S, Pajaniammalle S: Ancient schwannoma arising from mental nerve. A case report and review. Med Oral Patol Oral Cir Bucal; 2009 Jan;14(1):E12-4
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Ancient schwannoma arising from mental nerve. A case report and review.
  • Schwannoma is an intraoral rare, benign neoplasm derived from the nerve sheath of peripheral nerves.
  • "Ancient schwannoma" shows histopathological features, such as degenerative changes and atypical nuclei, and may easily be confused with malignant neoplasms.
  • Ancient schwannoma of the head and neck region is relatively uncommon and very few cases had been reported in the oral cavity.
  • We present a case of ancient schwannoma arising from the mental nerve in a 19 year old male which was of eight months duration.
  • Ultrasonography showed that the tumor was closely associated with the mental nerve on the left side, suggestive of a peripheral neural sheath tumor.
  • Complete excision of the lesion was done under local anesthesia, preserving the mental nerve.
  • The histological picture was strongly suggestive of ancient schwannoma (Antoni A type).
  • [MeSH-major] Chin / innervation. Neurilemmoma / pathology. Peripheral Nervous System Neoplasms / pathology
  • [MeSH-minor] Humans. Male. Young Adult

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  • (PMID = 19114949.001).
  • [ISSN] 1698-6946
  • [Journal-full-title] Medicina oral, patología oral y cirugía bucal
  • [ISO-abbreviation] Med Oral Patol Oral Cir Bucal
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] Spain
  • [Number-of-references] 11
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42. 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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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [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.
  • Immunohistochemistry confirmed the neurogenic differentiation with varying S-100 expression and the rhabdomyoblastic differentiation with desmin and myoglobin positivity in all cases.
  • Malignant triton tumor is an uncommon tumor associated with an aggressive behavior.
  • [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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43. 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

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [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.
  • [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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44. Bahrami A, Folpe AL: Adult-type fibrosarcoma: A reevaluation of 163 putative cases diagnosed at a single institution over a 48-year period. Am J Surg Pathol; 2010 Oct;34(10):1504-13
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  • [Title] Adult-type fibrosarcoma: A reevaluation of 163 putative cases diagnosed at a single institution over a 48-year period.
  • Adult-type fibrosarcoma (FS) was once considered the most common adult sarcoma, but is now considered a diagnosis of exclusion.
  • One hundred ninety-five cases diagnosed as adult FS in somatic soft tissue were retrieved from our institutional archives for the period 1960 to 2008.
  • Non-FS (137 cases) were reclassified as: undifferentiated pleomorphic sarcoma (32 cases), SS (21 cases), solitary fibrous tumor (14 cases), myxofibrosarcoma (11 cases), malignant peripheral nerve sheath tumor (8 cases), FS dermatofibrosarcoma protuberans, and desmoplastic melanoma (4 cases each), low-grade fibromyxoid sarcoma, sarcomatoid carcinoma, desmoid-type fibromatosis, rhabdomyosarcoma, myofibroblastic sarcoma, spindle-cell liposarcoma (3 cases each), sclerosing epithelioid FS, fibroma-like epithelioid sarcoma, leiomyosarcoma, cellular fibrous histiocytoma (2 cases each), and others (17 cases).
  • Exclusive of undifferentiated pleomorphic sarcoma, the distinction of which from FS is subjective, 64% of putative FS were reclassified, most commonly as monophasic SS and solitary fibrous tumor.
  • We conclude that true FS is exceedingly rare, accounting for <1% of approximately 10,000 adult soft tissue sarcomas seen at our institution during this time period, and should be diagnosed with great caution.
  • [MeSH-minor] Adolescent. Adult. Age Factors. Aged. Aged, 80 and over. Biomarkers, Tumor / metabolism. Child. Child, Preschool. DNA, Neoplasm / analysis. Female. Gene Rearrangement. Humans. In Situ Hybridization, Fluorescence. Male. Middle Aged. Minnesota / epidemiology. Proto-Oncogene Proteins / genetics. Proto-Oncogene Proteins / metabolism. Repressor Proteins / genetics. Repressor Proteins / metabolism. Young Adult

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  • (PMID = 20829680.001).
  • [ISSN] 1532-0979
  • [Journal-full-title] The American journal of surgical pathology
  • [ISO-abbreviation] Am. J. Surg. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / DNA, Neoplasm; 0 / Proto-Oncogene Proteins; 0 / Repressor Proteins; 0 / SS18 protein, human
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45. Jokinen CH, Argenyi ZB: Atypical neurofibroma of the skin and subcutaneous tissue: clinicopathologic analysis of 11 cases. J Cutan Pathol; 2010 Jan;37(1):35-42
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  • BACKGROUND: Neurofibroma (NF) is a relatively common cutaneous tumor, which typically presents little diagnostic difficulty.
  • Occasionally, however, pleomorphic cells may be present in NF raising consideration of other neoplasms like malignant peripheral nerve sheath tumor (MPNST).
  • Some tumors were hypercellular, but marked density characteristic of MPNST was not observed.
  • Of six patients with available follow-up, no tumor recurred and none developed malignancy (range 6-63, mean 33 months).
  • CONCLUSIONS: Superficial atypical NF, while morphologically unusual, has no apparent association with neurofibromatosis-1 or short-term risk of recurrence or malignant transformation.
  • Awareness of this variant is important in order to avoid misdiagnosis of a more aggressive neoplasm.
  • [MeSH-minor] Adult. Aged. Biomarkers, Tumor / metabolism. Child. Diagnosis, Differential. Disease-Free Survival. Female. Humans. Male. Middle Aged. Neoplasm Recurrence, Local. Nerve Sheath Neoplasms / pathology. S100 Proteins / metabolism. Young Adult

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  • [Copyright] Copyright © 2009 John Wiley & Sons A/S.
  • (PMID = 19469864.001).
  • [ISSN] 1600-0560
  • [Journal-full-title] Journal of cutaneous pathology
  • [ISO-abbreviation] J. Cutan. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Denmark
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / S100 Proteins
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46. Nelson DB, Greer L, Wendel G: Neurofibromatosis and pregnancy: a report of maternal cardiopulmonary compromise. Obstet Gynecol; 2010 Aug;116 Suppl 2:507-9
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  • BACKGROUND: Neurofibromatosis is a rare pregnancy complication that can present with rapidly enlarging masses that have malignant potential.
  • A malignant mediastinal mass was diagnosed and resected, with additional treatment options declined.
  • CONCLUSION: Neurofibromatosis type 1 with malignant peripheral nerve sheath tumors complicating pregnancy requires an experienced, multidisciplinary team of care offering an aggressive evaluation to rule out malignant transformation or recurrence when there is any change in clinical status of a patient, as this may signal a potentially fatal change in the lesion.
  • [MeSH-major] Airway Obstruction / etiology. Mediastinal Neoplasms / complications. Neoplasm Recurrence, Local / complications. Nerve Sheath Neoplasms / complications. Neurofibromatosis 1 / complications. Pregnancy Complications, Neoplastic
  • [MeSH-minor] Fatal Outcome. Female. Humans. Pregnancy. Pregnancy Outcome. Young Adult

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  • (PMID = 20664435.001).
  • [ISSN] 1873-233X
  • [Journal-full-title] Obstetrics and gynecology
  • [ISO-abbreviation] Obstet Gynecol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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47. Gladdy RA, Qin LX, Moraco N, Edgar MA, Antonescu CR, Alektiar KM, Brennan MF, Singer S: Do radiation-associated soft tissue sarcomas have the same prognosis as sporadic soft tissue sarcomas? J Clin Oncol; 2010 Apr 20;28(12):2064-9
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  • A matched-cohort analysis was performed for radiation-associated and sporadic malignant fibrous histiocytoma (MFH).
  • DSS was significantly worse in primary RAS malignant peripheral nerve sheath tumors (MPNSTs) compared with unmatched sporadic MPNSTs (P = .001).
  • CONCLUSION Histologic type, margin status, and tumor size are the most important independent predictors of DSS in patients with RASs.
  • DSS in patients with primary RAS is significantly worse compared with sporadic STS independent of sarcoma histologic type.

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  • [Cites] Ann Surg Oncol. 2007 Jun;14(6):1953-67 [17356953.001]
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  • (PMID = 20308666.001).
  • [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
  • [Grant] United States / NCI NIH HHS / CA / P01 CA047179; United States / NCI NIH HHS / CA / P50 CA140146
  • [Publication-type] Comparative Study; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Other-IDs] NLM/ PMC3651600
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48. Shah H, Pervez S: Immunophenotypic characterization of high grade pleomorphic sarcomas: a demographic and immunohistochemical study in a major referral center of Pakistan. J Pak Med Assoc; 2005 Mar;55(3):101-4
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  • RESULTS: Of the 134 cases which were characterized, 38.1% were pleomorphic leiomyosarcoma, followed by pleomorphic rhabdomyosarcoma 14.9%, Malignant Peripheral Nerve Sheath Tumour 9%, pleomorphic liposarcoma 3.7% and pleomorphic storiform Malignant Fibrous Histiocytoma 0.7%.
  • Mean/ median age for Leiomyosarcoma was 50/50, for Rhabdomyosarcomas 33/22, for MPNST 42/41, for Liposarcoma 52/50 and for Malignant Fibrous Histiocytoma 46/46 respectively.
  • CONCLUSION: It was concluded that the most common pleomorphic sarcoma occurring in our adult population was Leiomyosarcoma, and that immunohistochemical stains are essential in most cases for further characterization of pleomorphic high grade sarcoma.
  • [MeSH-major] Sarcoma / pathology. Soft Tissue Neoplasms / pathology
  • [MeSH-minor] Adult. Age Distribution. Aged. Antigens, Neoplasm / immunology. Biomarkers, Tumor / analysis. Cross-Sectional Studies. Humans. Immunoenzyme Techniques. Immunohistochemistry. Leiomyosarcoma / pathology. Liposarcoma / genetics. Liposarcoma / pathology. Middle Aged. Nerve Sheath Neoplasms / genetics. Nerve Sheath Neoplasms / pathology. Pakistan. Phenotype. Rhabdomyosarcoma / genetics. Rhabdomyosarcoma / pathology. Surveys and Questionnaires

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  • (PMID = 15852744.001).
  • [ISSN] 0030-9982
  • [Journal-full-title] JPMA. The Journal of the Pakistan Medical Association
  • [ISO-abbreviation] J Pak Med Assoc
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Pakistan
  • [Chemical-registry-number] 0 / Antigens, Neoplasm; 0 / Biomarkers, Tumor
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49. Siqueira MG, Martins RS, Teixeira MJ: Management of brachial plexus region tumours and tumour-like conditions: relevant diagnostic and surgical features in a consecutive series of eighteen patients. Acta Neurochir (Wien); 2009 Sep;151(9):1089-98
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  • FINDINGS: The tumours comprised a heterogeneous group of lesions, including schwannomas, neurofibromas, malignant peripheral nerve sheath tumour (MPNST), sarcomas, metastases, desmoids and an aneurysmal bone cyst.
  • Eleven tumours were benign and 7 were malignant.
  • Some of the malignant tumours could be controlled by surgery plus adjuvant therapy, but this category is still associated with high morbidity and mortality rates.
  • [MeSH-major] Brachial Plexus / pathology. Brachial Plexus / surgery. Brachial Plexus Neuropathies / diagnosis. Brachial Plexus Neuropathies / surgery. Peripheral Nervous System Neoplasms / diagnosis. Peripheral Nervous System Neoplasms / surgery
  • [MeSH-minor] Adolescent. Adult. Bone Cysts, Aneurysmal / diagnosis. Bone Cysts, Aneurysmal / pathology. Bone Cysts, Aneurysmal / surgery. Child. Female. Fibromatosis, Aggressive / diagnosis. Fibromatosis, Aggressive / physiopathology. Fibromatosis, Aggressive / surgery. Humans. Magnetic Resonance Imaging. Male. Middle Aged. Neoplasm Metastasis / diagnosis. Neoplasm Metastasis / physiopathology. Neoplasm Recurrence, Local / epidemiology. Nerve Sheath Neoplasms / diagnosis. Nerve Sheath Neoplasms / physiopathology. Nerve Sheath Neoplasms / surgery. Neurilemmoma / diagnosis. Neurilemmoma / physiopathology. Neurilemmoma / surgery. Neurofibroma / diagnosis. Neurofibroma / physiopathology. Neurofibroma / surgery. Neurosurgical Procedures. Pain / etiology. Paresthesia / etiology. Postoperative Complications / epidemiology. Retrospective Studies. Sarcoma / diagnosis. Sarcoma / physiopathology. Sarcoma / surgery. Tomography, X-Ray Computed. Treatment Outcome. Young Adult

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  • (PMID = 19448970.001).
  • [ISSN] 0942-0940
  • [Journal-full-title] Acta neurochirurgica
  • [ISO-abbreviation] Acta Neurochir (Wien)
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Austria
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50. Lehnhardt M, Daigeler A, Homann HH, Hauser J, Langer S, Steinsträsser L, Soimaru C, Puls A, Steinau HU: [Importance of specialized centers in diagnosis and treatment of extremity-soft tissue sarcomas. Review of 603 cases]. Chirurg; 2009 Apr;80(4):341-7
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  • The records of 603 patients with soft tissue tumors of the extremities were reviewed concerning mismatches in primary and definite diagnoses relating to entity, evaluation of primary or recurrent tumor specimens, and the diagnosing pathology institution.
  • Liposarcoma and malignant fibrous histiocytoma were the most often diagnosed subgroups at 24% and 22.6%, respectively.
  • In the eight most frequent sarcoma types, malignant peripheral nerve sheath tumors and leiomyosarcoma had the highest rates of false primary diagnosis, 78.4% and 74.2% of cases, respectively.
  • [MeSH-major] Cancer Care Facilities. Extremities / surgery. Hospitals, Special. Hospitals, University. Sarcoma / diagnosis. Sarcoma / surgery. Soft Tissue Neoplasms / diagnosis. Soft Tissue Neoplasms / surgery
  • [MeSH-minor] Adolescent. Adult. Aged. Aged, 80 and over. Chemotherapy, Adjuvant. Child. Combined Modality Therapy. Diagnostic Errors. Female. Germany. Histiocytoma, Benign Fibrous / diagnosis. Histiocytoma, Benign Fibrous / pathology. Histiocytoma, Benign Fibrous / surgery. Humans. Leiomyosarcoma / diagnosis. Leiomyosarcoma / pathology. Leiomyosarcoma / surgery. Liposarcoma / diagnosis. Liposarcoma / pathology. Liposarcoma / surgery. Male. Middle Aged. Neoplasm Staging. Nerve Sheath Neoplasms / diagnosis. Nerve Sheath Neoplasms / pathology. Nerve Sheath Neoplasms / surgery. Radiotherapy, Adjuvant. Referral and Consultation. Retrospective Studies. Young Adult

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  • (PMID = 18523742.001).
  • [ISSN] 1433-0385
  • [Journal-full-title] Der Chirurg; Zeitschrift fur alle Gebiete der operativen Medizen
  • [ISO-abbreviation] Chirurg
  • [Language] ger
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Germany
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51. Prayson RA, Yoder BJ, Barnett GH: Epidermal growth factor receptor is not amplified in schwannomas. Ann Diagn Pathol; 2007 Oct;11(5):326-9
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  • Both neurofibromas and schwannomas are capable of progression to malignant peripheral nerve sheath tumors (MPNSTs).
  • The mitogenic signaling for schwannomas is unlikely to be related to overexpression or amplification of EGFR; however, acquiring this signaling pathway might contribute to the progression of a subset of benign peripheral nerve sheath tumors to MPNST.
  • [MeSH-major] Nerve Sheath Neoplasms / metabolism. Neurilemmoma / metabolism. Receptor, Epidermal Growth Factor / metabolism
  • [MeSH-minor] Adult. Aged. DNA, Neoplasm / genetics. Disease Progression. Female. Gene Amplification. Gene Expression Regulation, Neoplastic. Humans. Immunohistochemistry. In Situ Hybridization, Fluorescence. Male. Middle Aged

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  • (PMID = 17870017.001).
  • [ISSN] 1092-9134
  • [Journal-full-title] Annals of diagnostic pathology
  • [ISO-abbreviation] Ann Diagn Pathol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / DNA, Neoplasm; EC 2.7.10.1 / Receptor, Epidermal Growth Factor
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52. Matsumine A, Shintani K, Kusuzaki K, Matsubara T, Satonaka H, Wakabayashi T, Iino T, Uchida A: Expression of decorin, a small leucine-rich proteoglycan, as a prognostic factor in soft tissue tumors. J Surg Oncol; 2007 Oct 1;96(5):411-8

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  • RESULTS: Lower levels of decorin were expressed in liposarcoma and malignant peripheral nerve sheath tumor than in lipoma (<0.01) and neurofibroma (P < 0.05), respectively.
  • An immunohistochemical analysis for spindle-cell sarcomas demonstrated decorin protein to be produced by myofibroblastic cells in the peripheral stromal extracellular spaces.
  • CONCLUSIONS: A reduced decorin expression was found to be a useful biomarker of aggressiveness in soft tissue tumor.
  • [MeSH-major] Extracellular Matrix Proteins / metabolism. Neoplasms, Connective and Soft Tissue / metabolism. Nerve Sheath Neoplasms / metabolism. Proteoglycans / metabolism. Soft Tissue Neoplasms / metabolism
  • [MeSH-minor] Adolescent. Adult. Aged. Aged, 80 and over. Biomarkers, Tumor. Child. Child, Preschool. DNA, Neoplasm / metabolism. Decorin. Female. Humans. Immunohistochemistry. Male. Middle Aged. Polymerase Chain Reaction. Prognosis. Proportional Hazards Models. RNA, Neoplasm / metabolism. Survival Analysis

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  • (PMID = 17579351.001).
  • [ISSN] 0022-4790
  • [Journal-full-title] Journal of surgical oncology
  • [ISO-abbreviation] J Surg Oncol
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / DCN protein, human; 0 / DNA, Neoplasm; 0 / Decorin; 0 / Extracellular Matrix Proteins; 0 / Proteoglycans; 0 / RNA, Neoplasm
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53. Chirife AM, Bello L, Celeste F, Giménez L, Gorostidy S: [Primary sarcomas of the breast]. Medicina (B Aires); 2006;66(2):135-8
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  • Primary sarcomas of the breast are extremely rare with less than 1% of all malignant tumours of the breast reported in literature.
  • At our Institution 1315 malignant tumours of the breast were diagnosed between 1999-2004; nine of them corresponded to primary sarcomas: angiosarcoma (3), leiomyosarcoma (1), low-grade fibromyxoid sarcoma (1), dematofibrosarcoma protuberans (1), liposarcoma (1), osteosarcoma (1), malignant peripheral nerve sheath tumour (1).
  • Most of the tumours (67%) showed p53 (mayor or equal to 20% of nuclear staining) over-expression but this did not show a direct relationship with the evolution of each neoplasm.
  • [MeSH-major] Breast Neoplasms. Sarcoma
  • [MeSH-minor] Adult. Argentina / epidemiology. Biomarkers, Tumor / genetics. Biomarkers, Tumor / metabolism. Female. Humans. Incidence. Middle Aged. Phenotype. Prevalence. Prognosis. Retrospective Studies. Tumor Suppressor Protein p53 / genetics. Tumor Suppressor Protein p53 / metabolism

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  • (PMID = 16715762.001).
  • [ISSN] 0025-7680
  • [Journal-full-title] Medicina
  • [ISO-abbreviation] Medicina (B Aires)
  • [Language] spa
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Argentina
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Tumor Suppressor Protein p53
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54. Puhaindran ME, Athanasian EA: Double ray amputation for tumors of the hand. Clin Orthop Relat Res; 2010 Nov;468(11):2976-9
MedlinePlus Health Information. consumer health - Soft Tissue Sarcoma.

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  • BACKGROUND: Partial hand amputations for malignant tumors allow tumor resection with negative resection margins, which is associated with lower local recurrence rates and improved overall survival while preserving native tissue, which improves functional outcome.
  • All five patients had high-grade soft tissue sarcomas of the hand, two synovial sarcomas, two malignant peripheral nerve sheath tumors, and one undifferentiated sarcoma.
  • No patients developed local tumor recurrence.
  • Functional assessment showed a mean Musculoskeletal Tumor Society score of 24 (range, 19-28) and mean grip strength 24% of the contralateral side (range, 17%-35%).
  • CONCLUSIONS: Although double ray amputation results in worse functional outcome than single ray, good key, tip, and tripod pinch can be preserved when the deep motor branch of the ulnar nerve is preserved, and this hand can still assist in bimanual hand activities.
  • [MeSH-major] Amputation. Hand / surgery. Sarcoma / surgery. Soft Tissue Neoplasms / surgery
  • [MeSH-minor] Adolescent. Adult. Child. Disease-Free Survival. Hand Strength. Humans. Middle Aged. Neoplasm Recurrence, Local. New York City. Recovery of Function. Retrospective Studies. Time Factors. Treatment Outcome. Ulnar Nerve / physiopathology. Young Adult

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  • (PMID = 20490732.001).
  • [ISSN] 1528-1132
  • [Journal-full-title] Clinical orthopaedics and related research
  • [ISO-abbreviation] Clin. Orthop. Relat. Res.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Other-IDs] NLM/ PMC2947675
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55. Perrin GQ, Fishbein L, Thomson SA, Thomas SL, Stephens K, Garbern JY, DeVries GH, Yachnis AT, Wallace MR, Muir D: Plexiform-like neurofibromas develop in the mouse by intraneural xenograft of an NF1 tumor-derived Schwann cell line. J Neurosci Res; 2007 May 1;85(6):1347-57
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  • [Title] Plexiform-like neurofibromas develop in the mouse by intraneural xenograft of an NF1 tumor-derived Schwann cell line.
  • Plexiform neurofibromas are peripheral nerve sheath tumors that arise frequently in neurofibromatosis type 1 (NF1) and have a risk of malignant progression.
  • Past efforts to establish xenograft models for neurofibroma involved the implantation of tumor fragments or heterogeneous primary cultures, which rarely achieved significant tumor growth.
  • We report a practical and reproducible animal model of plexiform-like neurofibroma by xenograft of an immortal human NF1 tumor-derived Schwann cell line into the peripheral nerve of scid mice.
  • Localized intraneural injection of the cell line sNF94.3 produced consistent and slow growing tumors that infiltrated and disrupted the host nerve.
  • [MeSH-major] Cell Line, Tumor. Lung Neoplasms / pathology. Neurofibromatosis 1 / pathology. Schwann Cells / cytology
  • [MeSH-minor] Adult. Animals. Blotting, Western. Female. Humans. Mice. Mice, SCID. Neoplasm Transplantation / methods. Nerve Tissue Proteins / metabolism. Neurofibromin 1 / genetics. Transplantation, Heterologous / methods. ras Proteins / metabolism

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  • [Copyright] (c) 2007 Wiley-Liss, Inc.
  • (PMID = 17335073.001).
  • [ISSN] 0360-4012
  • [Journal-full-title] Journal of neuroscience research
  • [ISO-abbreviation] J. Neurosci. Res.
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / T32-CA09126-27
  • [Publication-type] 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 / Nerve Tissue Proteins; 0 / Neurofibromin 1; EC 3.6.5.2 / ras Proteins
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56. Langman G, Rathinam S, Papadaki L: Primary localised pleural neurofibroma: expanding the spectrum of spindle cell tumours of the pleura. J Clin Pathol; 2010 Feb;63(2):116-8
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  • The second patient was known to have neurofibromatosis type I, and the pleural lesion was found incidentally during excision of a metastatic malignant peripheral nerve sheath tumour of the lung.
  • There was variable immunoreactivity for S100 and CD34, while ultrastructure examination in the two cases showed a mixture of nerve sheath cell types.
  • The presence of a bland spindle cell pleural neoplasm immunoreactive for CD34 may potentially be mistaken for a solitary fibrous tumour.
  • [MeSH-minor] Adult. Aged. Diagnosis, Differential. Female. Humans. Neurofibromatosis 1 / diagnosis. Neurofibromatosis 1 / radiography. Solitary Fibrous Tumor, Pleural / diagnosis. Tomography, X-Ray Computed

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  • (PMID = 20154031.001).
  • [ISSN] 1472-4146
  • [Journal-full-title] Journal of clinical pathology
  • [ISO-abbreviation] J. Clin. Pathol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
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57. Yoshida K, Kawase T, Tomita T, Ogawa K, Kawana H, Yago K, Asanami S: Surgical strategy for tumors located in or extending from the intracranial space to the infratemporal fossa-Advantages of the transcranial approach (zygomatic infratemporal fossa approach) and the indications for a combined transcranial and transcervical approach-. Neurol Med Chir (Tokyo); 2009 Dec;49(12):580-6
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  • A case of mandibular nerve schwannoma, which extended 1 cm below the external orifice of the foramen ovale, was completely removed via the epidural subtemporal approach without zygomatic osteotomy with partial removal of the middle cranial base.
  • The inferior margin of infratemporal tumor could be accessed via the transcranial route with zygomatic or orbitozygomatic osteotomy without complications including facial nerve injury in nine cases, and the lowest level of the infratemporal tumors was approximately 4.5 cm below the outer surface of the middle cranial base.
  • In five of these 9 cases (2 schwannomas, 1 myxoma, 1 chondrosarcoma, and 1 malignant peripheral nerve sheath tumor), the tumors were localized in the infratemporal fossa, and in the other 4 cases (2 meningiomas, 1 glioblastoma, and 1 ameloblastoma), the tumors extended to both the intracranial space and the infratemporal fossa.
  • In two cases (recurrent jugular schwannoma and mandibular osteosarcoma), a combined transcranial and transcervical approach (mandibular swing approach) was essential, because the resection line of the lower margin was too far from the middle cranial base.
  • [MeSH-minor] Adult. Aged. Cranial Nerve Neoplasms / pathology. Cranial Nerve Neoplasms / radiography. Cranial Nerve Neoplasms / surgery. Cranial Nerves / anatomy & histology. Cranial Nerves / pathology. Cranial Nerves / surgery. Disease Progression. Female. Humans. Magnetic Resonance Imaging. Male. Meningioma / pathology. Meningioma / radiography. Meningioma / surgery. Middle Aged. Neck / anatomy & histology. Neck / surgery. Neoplasm Invasiveness / pathology. Neoplasm Invasiveness / physiopathology. Orbit / anatomy & histology. Orbit / surgery. Osteotomy / methods. Postoperative Complications / prevention & control. Retrospective Studies. Sarcoma / pathology. Sarcoma / radiography. Sarcoma / surgery. Skull Base Neoplasms / pathology. Skull Base Neoplasms / radiography. Skull Base Neoplasms / surgery. Tomography, X-Ray Computed. Treatment Outcome. Young Adult. Zygoma / anatomy & histology. Zygoma / surgery

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  • (PMID = 20035132.001).
  • [ISSN] 1349-8029
  • [Journal-full-title] Neurologia medico-chirurgica
  • [ISO-abbreviation] Neurol. Med. Chir. (Tokyo)
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Japan
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58. Puhaindran ME, Steensma MR, Athanasian EA: Partial hand preservation for large soft tissue sarcomas of the hand. J Hand Surg Am; 2010 Feb;35(2):291-5
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  • In patients with large soft tissue sarcomas of the hand, partial hand preservation is extremely challenging for surgeons attempting a complete resection of the tumor with negative resection margins.
  • We identified 8 patients who had tumors at least 5 cm in maximum dimension and had tumor resection with partial hand preservation.
  • Two patients had myxofibrosarcoma, 2 patients had synovial sarcoma, 2 patients had malignant fibrous histiocytoma, 1 patient had a malignant peripheral nerve sheath tumor, and 1 patient had a liposarcoma.
  • Hand function was evaluated using Musculoskeletal Tumor Society criteria.
  • RESULTS: Of the 8 patients, 1 died of distant metastatic disease, 1 developed local tumor recurrence and is alive with locally recurrent disease, and the other 6 patients are completely disease-free.
  • The mean Musculoskeletal Tumor Society score was 26 (range, 19-29), with the 2 patients who had received double-ray amputations having the lower scores (19 and 24).
  • [MeSH-major] Hand / surgery. Limb Salvage / methods. Neoplasm Recurrence, Local / pathology. Sarcoma / diagnosis. Sarcoma / surgery. Soft Tissue Neoplasms / surgery
  • [MeSH-minor] Adolescent. Adult. Aged. Aged, 80 and over. Amputation / methods. Arm. Artificial Limbs. Child. Cohort Studies. Female. Follow-Up Studies. Humans. Male. Middle Aged. Neoplasm Staging. Prosthesis Fitting. Retrospective Studies. Risk Assessment. Survival Analysis. Treatment Outcome. Young Adult

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  • [Copyright] Copyright 2010 American Society for Surgery of the Hand. Published by Elsevier Inc. All rights reserved.
  • (PMID = 20141899.001).
  • [ISSN] 1531-6564
  • [Journal-full-title] The Journal of hand surgery
  • [ISO-abbreviation] J Hand Surg Am
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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59. De Corti F, Dall'Igna P, Bisogno G, Casara D, Rossi CR, Foletto M, Alaggio R, Carli M, Cecchetto G: Sentinel node biopsy in pediatric soft tissue sarcomas of extremities. Pediatr Blood Cancer; 2009 Jan;52(1):51-4
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  • BACKGROUND: Sentinel Node Biopsy is an established staging technique in many adult malignancies.
  • The diagnosis was rhabdomyosarcoma in 5 and other soft tissue sarcomas in 12: Ewing/PNET sarcoma 6, epithelioid sarcoma 1, malignant peripheral-nerve-sheath tumor 1, undifferentiated sarcoma 1, myxoid liposarcoma 2, adult-type fibrosarcoma 1.
  • Nodes were positive for metastasis in two patients with alveolar rhabdomyosarcoma and undifferentiated sarcoma.
  • [MeSH-minor] Adolescent. Child. Child, Preschool. Female. Humans. Male. Neoplasm Metastasis / pathology. Sentinel Lymph Node Biopsy. Soft Tissue Neoplasms / pathology. Young Adult

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  • (PMID = 18819127.001).
  • [ISSN] 1545-5017
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Publication-type] Evaluation Studies; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
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60. Assaad MW, Pantanowitz L, Otis CN: Diagnostic accuracy of image-guided percutaneous fine needle aspiration biopsy of the mediastinum. Diagn Cytopathol; 2007 Nov;35(11):705-9
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Primary neoplasms (N = 38) included 24 lymphomas (6 Hodgkin and 18 non-Hodgkin), 7 thymomas, 1 thymic carcinoma, and 6 peripheral nerve sheath tumors.
  • There were 4 non-neoplastic lesions (1 granulomatous process; 2 bronchogenic and 1 pericardial cyst), 1 case of undifferentiated malignant large cell neoplasm, 13 cases negative for malignancy, and 29 (18%) that were indeterminate, due largely to insufficient cellularity.
  • There were 6 discordant cases, including 5 FNAB that were of adequate cellularity but interpreted as negative for malignant cells (on subsequent histology 2 turned out to be Hodgkin lymphoma, 2 carcinomas, and 1 diffuse large cell lymphoma), and 1 diagnosed as thymoma that on histologic evaluation was a thymic large cell lymphoma.
  • [MeSH-minor] Adolescent. Adult. Aged. Aged, 80 and over. Child. Child, Preschool. Female. Humans. Infant. Male. Middle Aged. Reproducibility of Results. Retrospective Studies

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  • [Copyright] (c) 2007 Wiley-Liss, Inc.
  • (PMID = 17924408.001).
  • [ISSN] 8755-1039
  • [Journal-full-title] Diagnostic cytopathology
  • [ISO-abbreviation] Diagn. Cytopathol.
  • [Language] eng
  • [Publication-type] Evaluation Studies; Journal Article
  • [Publication-country] United States
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61. Charest M, Hickeson M, Lisbona R, Novales-Diaz JA, Derbekyan V, Turcotte RE: FDG PET/CT imaging in primary osseous and soft tissue sarcomas: a retrospective review of 212 cases. Eur J Nucl Med Mol Imaging; 2009 Dec;36(12):1944-51
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  • METHODS: We retrospectively evaluated 212 consecutive patients with known soft tissue or osseous sarcoma who had undergone a FDG PET/CT study for the initial staging or assessment of recurrence of disease.
  • The sensitivities of the most common sarcoma histologies were 100% for leiomyosarcomas, 94.7% for osteosarcomas, 100% for Ewing's sarcomas, 88.9% for liposarcomas, 80.0% for synovial sarcomas, 100% for gastrointestinal stromal tumors, 87.5% for malignant peripheral nerve sheath tumors, 100% for fibroblastic and myoblastic sarcomas, and 100% for malignant fibrohistiocytic tumors.
  • [MeSH-major] Bone Neoplasms / diagnostic imaging. Fluorodeoxyglucose F18. Positron-Emission Tomography. Sarcoma / diagnostic imaging. Tomography, X-Ray Computed
  • [MeSH-minor] Adolescent. Adult. Aged. Aged, 80 and over. Biological Transport. Child. Child, Preschool. Humans. Middle Aged. Neoplasm Staging. Retrospective Studies. Young Adult

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  • [CommentIn] Eur J Nucl Med Mol Imaging. 2009 Dec;36(12):1940-3 [19633842.001]
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  • (PMID = 19593561.001).
  • [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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62. Thorson JA, Weigelin HC, Ruiz RE, Howard JK, Lucas DR: Identification of SYT-SSX transcripts from synovial sarcomas using RT-multiplex PCR and capillary electrophoresis. Mod Pathol; 2006 May;19(5):641-7
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Synovial sarcomas are highly malignant tumors of soft tissue which are characterized by the t(X;18) resulting in SYT-SSX fusion transcript production.
  • In a study of 32 formalin-fixed soft tissue tumor specimens, the assay detected chimeric transcripts from 17/22 (77%) synovial sarcomas.
  • Chimeric transcripts were not detected in 9/9 malignant peripheral nerve sheath tumors or 1/1 epithelioid sarcoma.
  • [MeSH-major] Biomarkers, Tumor / genetics. Electrophoresis, Capillary / methods. Oncogene Proteins, Fusion / genetics. Reverse Transcriptase Polymerase Chain Reaction / methods. Sarcoma, Synovial / pathology
  • [MeSH-minor] Adolescent. Adult. Aged. Base Sequence. Child. Female. Genotype. Humans. Male. Middle Aged. Molecular Sequence Data. RNA, Neoplasm / genetics. RNA, Neoplasm / metabolism. Reproducibility of Results. Sequence Analysis, DNA. Transcription, Genetic / genetics

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  • (PMID = 16547469.001).
  • [ISSN] 0893-3952
  • [Journal-full-title] Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc
  • [ISO-abbreviation] Mod. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Oncogene Proteins, Fusion; 0 / RNA, Neoplasm; 0 / SYT-SSX fusion protein
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63. Serizawa I, Kagei K, Kamada T, Imai R, Sugahara S, Okada T, Tsuji H, Ito H, Tsujii H: Carbon ion radiotherapy for unresectable retroperitoneal sarcomas. Int J Radiat Oncol Biol Phys; 2009 Nov 15;75(4):1105-10
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  • PURPOSE: To evaluate the applicability of carbon ion radiotherapy (CIRT) for unresectable retroperitoneal sarcomas with regard to normal tissue morbidity and local tumor control.
  • METHODS AND MATERIALS: From May 1997 to February 2006, 24 patients (17 male and 7 female) with unresectable retroperitoneal sarcoma received CIRT.
  • Histologic diagnoses were as follows: malignant fibrous histiocytoma in 6, liposarcoma in 3, malignant peripheral nerve sheath tumor in 3, Ewing/primitive neuroectodermal tumor (PNET) in 2, and miscellaneous in 10 patients.
  • [MeSH-major] Carbon Radioisotopes / therapeutic use. Neoplasm Recurrence, Local / radiotherapy. Retroperitoneal Neoplasms / radiotherapy. Sarcoma / radiotherapy. Soft Tissue Neoplasms / radiotherapy
  • [MeSH-minor] Adolescent. Adult. Aged. Carbon. Female. Follow-Up Studies. Humans. Magnetic Resonance Spectroscopy. Male. Middle Aged. Radiodermatitis / pathology. Radiotherapy Dosage. Relative Biological Effectiveness. Survival Rate. Tomography, X-Ray Computed. Tumor Burden. Young Adult

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  • (PMID = 19467578.001).
  • [ISSN] 1879-355X
  • [Journal-full-title] International journal of radiation oncology, biology, physics
  • [ISO-abbreviation] Int. J. Radiat. Oncol. Biol. Phys.
  • [Language] eng
  • [Publication-type] Evaluation Studies; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Carbon Radioisotopes; 7440-44-0 / Carbon
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64. Malagón HD, Valdez AM, Moran CA, Suster S: Germ cell tumors with sarcomatous components: a clinicopathologic and immunohistochemical study of 46 cases. Am J Surg Pathol; 2007 Sep;31(9):1356-62
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  • The germ cell component consisted of pure mature or immature teratoma (23 cases), teratoma mixed with other seminomatous or nonseminomatous components (17), pure seminoma (2), intratubular germ cell neoplasia (1), and yolk sac tumor (1).
  • The SC included embryonal rhabdomyosarcoma (29), angiosarcoma (6), leiomyosarcoma (4), undifferentiated sarcoma (3), myxoid liposarcoma (1), malignant peripheral nerve sheath tumor (1), malignant "triton" tumor (1), and epithelioid hemangioendothelioma (1).
  • Thirty-two of 40 patients either died of tumor (25/40; 62.5%) or were alive with advanced, progressive disease (7/40; 17.5%), and only 8/40 (20%) were alive and free of disease between 5 to 40 months (mean=18 mo).
  • [MeSH-major] Immunohistochemistry. Mediastinal Neoplasms / diagnosis. Neoplasms, Germ Cell and Embryonal / diagnosis. Ovarian Neoplasms / diagnosis. Retroperitoneal Neoplasms / diagnosis. Sarcoma / diagnosis. Testicular Neoplasms / diagnosis
  • [MeSH-minor] Adolescent. Adult. Aged. Antineoplastic Combined Chemotherapy Protocols. Disease-Free Survival. Female. Follow-Up Studies. Humans. Male. Middle Aged. Neoplasm Metastasis. Neoplasm Staging. Orchiectomy. Ovariectomy. Time Factors. Treatment Outcome

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  • (PMID = 17721191.001).
  • [ISSN] 0147-5185
  • [Journal-full-title] The American journal of surgical pathology
  • [ISO-abbreviation] Am. J. Surg. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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65. Amary MF, Berisha F, Bernardi Fdel C, Herbert A, James M, Reis-Filho JS, Fisher C, Nicholson AG, Tirabosco R, Diss TC, Flanagan AM: Detection of SS18-SSX fusion transcripts in formalin-fixed paraffin-embedded neoplasms: analysis of conventional RT-PCR, qRT-PCR and dual color FISH as diagnostic tools for synovial sarcoma. Mod Pathol; 2007 Apr;20(4):482-96
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  • [Title] Detection of SS18-SSX fusion transcripts in formalin-fixed paraffin-embedded neoplasms: analysis of conventional RT-PCR, qRT-PCR and dual color FISH as diagnostic tools for synovial sarcoma.
  • Synovial Sarcoma consistently harbors t(X;18) resulting in SS18-SSX1, SS18-SSX2 and rarely SS18-SSX4 fusion transcripts.
  • Of 328 cases included in our study, synovial sarcoma was either the primary diagnosis or was very high in the differential diagnosis in 134 cases: of these, amplifiable cDNA was obtained from 131.
  • SS18-SSX1 was present in 56 monophasic and 18 biphasic synovial sarcoma: SS18-SSX2 was detected in 41 monophasic and 11 biphasic synovial sarcoma.
  • Following clinical input, a diagnosis of mesothelioma was favored in one case, a sarcoma, not otherwise specified in another and a solitary fibrous tumor in the third case.
  • The possibility of a malignant peripheral nerve sheath tumor could not be excluded in the other two cases.
  • We concluded that the employment of a combination of molecular approaches is a powerful aid to diagnosing synovial sarcoma giving at least 96% sensitivity and 100% specificity but results must be interpreted in the light of other modalities such as clinical findings and immunohistochemical data.
  • [MeSH-major] Antigens, Neoplasm / metabolism. Neoplasm Proteins / metabolism. Proto-Oncogene Proteins / metabolism. Repressor Proteins / metabolism. Sarcoma, Synovial / metabolism. Soft Tissue Neoplasms / metabolism
  • [MeSH-minor] Adolescent. Adult. Aged. Aged, 80 and over. Biomarkers, Tumor / metabolism. Child. Child, Preschool. Female. Formaldehyde. Humans. Immunohistochemistry. Male. Middle Aged. Oncogene Proteins, Fusion / genetics. Oncogene Proteins, Fusion / metabolism. Paraffin Embedding. RNA, Messenger / metabolism. Recombinant Fusion Proteins / genetics. Reverse Transcriptase Polymerase Chain Reaction. Tissue Array Analysis. Tissue Fixation

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  • (PMID = 17334349.001).
  • [ISSN] 0893-3952
  • [Journal-full-title] Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc
  • [ISO-abbreviation] Mod. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antigens, Neoplasm; 0 / Biomarkers, Tumor; 0 / Neoplasm Proteins; 0 / Oncogene Proteins, Fusion; 0 / Proto-Oncogene Proteins; 0 / RNA, Messenger; 0 / Recombinant Fusion Proteins; 0 / Repressor Proteins; 0 / SS18 protein, human; 0 / SYT-SSX fusion protein; 164289-47-8 / synovial sarcoma X breakpoint proteins; 1HG84L3525 / Formaldehyde
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66. Maki RG, D'Adamo DR, Keohan ML, Saulle M, Schuetze SM, Undevia SD, Livingston MB, Cooney MM, Hensley ML, Mita MM, Takimoto CH, Kraft AS, Elias AD, Brockstein B, Blachère NE, Edgar MA, Schwartz LH, Qin LX, Antonescu CR, Schwartz GK: Phase II study of sorafenib in patients with metastatic or recurrent sarcomas. J Clin Oncol; 2009 Jul 01;27(19):3133-40
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  • PURPOSE Since activity of sorafenib was observed in sarcoma patients in a phase I study, we performed a multicenter phase II study of daily oral sorafenib in patients with recurrent or metastatic sarcoma.
  • PATIENTS AND METHODS We employed a multiarm study design, each representing a sarcoma subtype with its own Simon optimal two-stage design.
  • In each arm, 12 patients who received 0 to 1 prior lines of therapy were treated (0 to 3 for angiosarcoma and malignant peripheral-nerve sheath tumor).
  • If at least one Response Evaluation Criteria in Solid Tumors (RECIST) was observed, 25 further patients with that sarcoma subtype were accrued.
  • Further evaluation of sorafenib in these and possibly other sarcoma subtypes appears warranted, presumably in combination with cytotoxic or kinase-specific agents.
  • [MeSH-major] Antineoplastic Agents / therapeutic use. Benzenesulfonates / therapeutic use. Neoplasm Recurrence, Local / drug therapy. Pyridines / therapeutic use. Sarcoma / drug therapy
  • [MeSH-minor] Adolescent. Adult. Aged. Aged, 80 and over. Disease-Free Survival. Female. Humans. Kaplan-Meier Estimate. Male. Middle Aged. Niacinamide / analogs & derivatives. Phenylurea Compounds. Young Adult

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  • (PMID = 19451436.001).
  • [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
  • [Grant] United States / NCI NIH HHS / CA / N01CM62202; United States / NCI NIH HHS / CA / P01 CA047179
  • [Publication-type] Clinical Trial, Phase II; Journal Article; Multicenter Study; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Benzenesulfonates; 0 / Phenylurea Compounds; 0 / Pyridines; 25X51I8RD4 / Niacinamide; 9ZOQ3TZI87 / sorafenib
  • [Other-IDs] NLM/ PMC2716936
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67. 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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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • 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.
  • 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-major] Heart Neoplasms / pathology. Sarcoma / pathology
  • [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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  • (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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68. 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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  • 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.
  • 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
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69. 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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  • 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.
  • Seven patients received >or=3 cycles, and two had prolonged stable disease (Wilms' tumor, 38 cycles; synovial sarcoma, 8 cycles).

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  • (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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70. Dawamneh MF, Amra NK, Amr SS: Low grade fibromyxoid sarcoma: report of a case with fine needle aspiration cytology and histologic correlation. Acta Cytol; 2006 Mar-Apr;50(2):208-12

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Low grade fibromyxoid sarcoma: report of a case with fine needle aspiration cytology and histologic correlation.
  • BACKGROUND: Low grade fibromyxoid sarcoma has been fully described histologically; however, the fine needle aspiration (FNA) cytologic findings are scantily defined, and the distinction from other benign and malignant soft tissue tumors can be difficult.
  • The excised tumor was well circumscribed, focally infiltrating the surrounding muscles.
  • The tumor cells were spindly, with fusiform, uniform nuclei.
  • The tumor cells were positive for vimentin, alpha-1-antitrypsin and lysozyme and negative for S-100, actin, desmin and CD34.
  • CONCLUSION: Although low grade fibromyxoid sarcoma is a rare neoplasm, it should be recognized and distinguished from other soft tissue tumors because of its low malignant potential.
  • The definitive FNA cytologic diagnosis can be challenging but is possible if the tumor is adequately sampled, with multiple passes from different areas.
  • All spindle cell tumors with myxoid changes, such as myxoid liposarcoma, myxofibrosarcoma, cellular myxoma, myxoid leiomyosarcoma and peripheral nerve sheath tumors, should be considered in the differential diagnosis.
  • [MeSH-minor] Adult. Biopsy, Fine-Needle / methods. Diagnosis, Differential. Female. Follow-Up Studies. Humans. Sensitivity and Specificity. Treatment Outcome

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  • (PMID = 16610692.001).
  • [ISSN] 0001-5547
  • [Journal-full-title] Acta cytologica
  • [ISO-abbreviation] Acta Cytol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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71. Kang GH, Kim KM, Noh JH, Sohn TS, Kim S, Park CK, Lee CS, Kang DY: WT-1 expression in gastrointestinal stromal tumours. Pathology; 2010 Jan;42(1):54-7
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  • Leiomyosarcomas, schwannomas, malignant peripheral nerve sheath tumours (MPNSTs) and melanomas showed cytoplasmic staining, whereas leiomyomas and mesenteric fibromatoses were totally negative for WT-1.
  • [MeSH-major] Biomarkers, Tumor / metabolism. Gastrointestinal Stromal Tumors / metabolism. Soft Tissue Neoplasms / metabolism. WT1 Proteins / metabolism
  • [MeSH-minor] Adult. Aged. Aged, 80 and over. Cytoplasm / metabolism. Cytoplasm / pathology. Female. Humans. Male. Middle Aged. Neoplasm Recurrence, Local. Prognosis. Receptor, Platelet-Derived Growth Factor alpha / metabolism

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  • (PMID = 20025481.001).
  • [ISSN] 1465-3931
  • [Journal-full-title] Pathology
  • [ISO-abbreviation] Pathology
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / WT1 Proteins; EC 2.7.10.1 / Receptor, Platelet-Derived Growth Factor alpha
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72. Kim HJ, Kim CH, Kim H, Yoon JH: Malignant peripheral nerve sheath tumour of the paranasal sinus. J Otolaryngol; 2007 Jun;36(3):E9-11

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Malignant peripheral nerve sheath tumour of the paranasal sinus.
  • [MeSH-major] Ethmoid Sinus. Nerve Sheath Neoplasms / surgery. Paranasal Sinus Neoplasms / surgery
  • [MeSH-minor] Adult. Humans. Immunohistochemistry. Male. Maxillary Sinus Neoplasms / pathology. Maxillary Sinus Neoplasms / surgery. Neoplasm Invasiveness. Orbit / pathology. Tomography, X-Ray Computed

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  • (PMID = 17711765.001).
  • [ISSN] 0707-7270
  • [Journal-full-title] The Journal of otolaryngology
  • [ISO-abbreviation] J Otolaryngol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Canada
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73. Chao BH, Stogner-Underwood KA, Kiev J, Smith TJ: Intrathoracic malignant peripheral nerve sheath tumor in neurofibromatosis 1. J Clin Oncol; 2008 May 1;26(13):2216-8
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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 in neurofibromatosis 1.
  • [MeSH-major] Neoplasms, Multiple Primary. Neurofibromatosis 1 / pathology. Peripheral Nervous System Neoplasms / pathology. Thoracic Neoplasms / pathology
  • [MeSH-minor] Adult. Bronchoscopy. Fatal Outcome. Humans. Lung Neoplasms / pathology. Male. Mediastinal Neoplasms / pathology. Neoplasm Invasiveness. Palliative Care. Radiography, Thoracic. Thoracotomy. Tomography, X-Ray Computed. Treatment Outcome

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  • (PMID = 18445849.001).
  • [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] Case Reports; Journal Article
  • [Publication-country] United States
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