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1. Hui P, Li N, Johnson C, De Wever I, Sciot R, Manfioletti G, Tallini G: HMGA proteins in malignant peripheral nerve sheath tumor and synovial sarcoma: preferential expression of HMGA2 in malignant peripheral nerve sheath tumor. Mod Pathol; 2005 Nov;18(11):1519-26
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] HMGA proteins in malignant peripheral nerve sheath tumor and synovial sarcoma: preferential expression of HMGA2 in malignant peripheral nerve sheath tumor.
  • Histological separation of synovial sarcomas from malignant peripheral nerve sheath tumors can be difficult and available immunohistochemical markers sometimes give rise to overlapping staining patterns.
  • To this end, we explored diagnostic applications of HMGA (HMGA1 and HMGA2) protein immunohistochemistry in comparable groups of synovial sarcoma and malignant peripheral nerve sheath tumors.
  • The histological diagnosis of these cases was confirmed by the presence or absence of synovial sarcoma specific SYT-SSX fusion transcript analyzed by real-time reverse transcription polymerase chain reaction.
  • In all, 13 malignant peripheral nerve sheath tumors and 15 synovial sarcomas were included in this study.
  • Immunohistochemically, most malignant peripheral nerve sheath tumors expressed both HMGA1 and HMGA2 protein (12/13 and 12/13 cases, respectively) with moderate to strong nuclear staining patterns.
  • However, significant immunoreactivity for HMGA2 was present in the glandular component of a biphasic tumor (1/1) and rarely detected in monophasic synovial sarcomas (1/14).
  • In summary, expression of HMGA2 is a feature of MPNST but not of synovial sarcoma and immunohistochemical staining of HMGA2 may be a useful marker to separate malignant peripheral nerve sheath tumor from synovial sarcoma.
  • [MeSH-major] HMGA Proteins / biosynthesis. Nerve Sheath Neoplasms / diagnosis. Sarcoma, Synovial / diagnosis
  • [MeSH-minor] Adult. Biomarkers, Tumor / analysis. Diagnosis, Differential. Female. Humans. Immunohistochemistry. Male. Oncogene Proteins, Fusion / genetics. Reverse Transcriptase Polymerase Chain Reaction

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  • [Copyright] Modern Pathology (2005) 18, 1519-1526. doi:10.1038/modpathol.3800464; published online 29 July 2005.
  • (PMID = 16056249.001).
  • [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 / HMGA Proteins; 0 / Oncogene Proteins, Fusion; 0 / SYT-SSX fusion protein
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2. 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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3. Bhola P, Banerjee S, Mukherjee J, Balasubramanium A, Arun V, Karim Z, Burrell K, Croul S, Gutmann DH, Guha A: Preclinical in vivo evaluation of rapamycin in human malignant peripheral nerve sheath explant xenograft. Int J Cancer; 2010 Jan 15;126(2):563-71
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  • [Title] Preclinical in vivo evaluation of rapamycin in human malignant peripheral nerve sheath explant xenograft.
  • Neurofibromatosis type 1 (NF1) patients are prone to the development of malignant tumors, the most common being Malignant Peripheral Nerve Sheath Tumor (MPNST).
  • NF1-MPNST patients have an overall poor survival due to systemic metastasis.
  • Recently, the NF1 gene product, neurofibromin, was shown to negatively regulate the phosphoinositide-3-kinase (PI3K)/Protein Kinase-B (Akt)/mammalian Target Of Rapamycin (mTOR) pathway, with loss of neurofibromin expression in established human MPNST cell lines associated with high levels of mTOR activity.
  • We developed and characterized a human NF1-MPNST explant grown subcutaneously in NOD-SCID mice, to evaluate the effect of the mTOR inhibitor rapamycin.
  • We demonstrate that rapamycin significantly inhibited human NF1-MPNST mTOR pathway activation and explant growth in vivo at doses as low as 1.0 mg/kg/day, without systemic toxicities.
  • While rapamycin was effective at reducing NF1-MPNST proliferation and angiogenesis, with decreased CyclinD1 and VEGF respectively, there was no increase in tumor apoptosis.
  • [MeSH-major] Neurofibromatosis 1 / drug therapy. Peripheral Nervous System Neoplasms / drug therapy. Sirolimus / pharmacology. Xenograft Model Antitumor Assays
  • [MeSH-minor] Animals. Antibiotics, Antineoplastic / pharmacology. Apoptosis / drug effects. Blotting, Western. Cyclin D1 / metabolism. Dose-Response Relationship, Drug. Drug Evaluation, Preclinical. Humans. Immunohistochemistry. Male. Mice. Mice, Inbred NOD. Mice, SCID. Protein Kinases / metabolism. Proto-Oncogene Proteins c-akt / metabolism. Ribosomal Protein S6 Kinases / metabolism. Signal Transduction / drug effects. TOR Serine-Threonine Kinases. Tumor Burden / drug effects. Vascular Endothelial Growth Factor A / metabolism. Young Adult

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  • (PMID = 19634141.001).
  • [ISSN] 1097-0215
  • [Journal-full-title] International journal of cancer
  • [ISO-abbreviation] Int. J. Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antibiotics, Antineoplastic; 0 / Vascular Endothelial Growth Factor A; 136601-57-5 / Cyclin D1; EC 2.7.- / Protein Kinases; EC 2.7.1.1 / MTOR protein, human; EC 2.7.1.1 / TOR Serine-Threonine Kinases; EC 2.7.1.1 / mTOR protein, mouse; EC 2.7.11.1 / Proto-Oncogene Proteins c-akt; EC 2.7.11.1 / Ribosomal Protein S6 Kinases; W36ZG6FT64 / Sirolimus
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4. Salla JT, Johann AC, Garcia BG, Aguiar MC, Mesquita RA: Retrospective analysis of oral peripheral nerve sheath tumors in Brazilians. Braz Oral Res; 2009 Jan-Mar;23(1):43-8
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  • [Title] Retrospective analysis of oral peripheral nerve sheath tumors in Brazilians.
  • Traumatic neuroma, neurofibroma, neurilemmoma, palisaded encapsulated neuroma and malignant peripheral nerve sheath tumor (MPNST) are peripheral nerve sheath tumors and present neural origin.
  • The goal of this study was to describe the epidemiological data of oral peripheral nerve sheath tumors in a sample of the Brazilian population.
  • Lesions diagnosed as peripheral nerve sheath tumors were submitted to morphologic and to immunohistochemical analyses.
  • Thirty-five oral peripheral nerve sheath tumors were found, representing 0.16% of all lesions archived in the Oral Pathology Service.
  • Neurilemmoma (4 cases) was more commonly observed in the buccal mucosa.
  • Malignant peripheral nerve sheath tumors (3 cases) occurred in the mandible, palate, and tongue.
  • The data confirmed that oral peripheral nerve sheath tumors are uncommon in the oral region, with some lesions presenting a predilection for a specific gender or site.
  • This study may be useful in clinical dentistry and oral pathology practice and may be used as baseline data regarding oral peripheral nerve sheath tumors in other populations.
  • [MeSH-major] Mouth Neoplasms / epidemiology. Nerve Sheath Neoplasms / epidemiology
  • [MeSH-minor] Adolescent. Adult. Aged. Aged, 80 and over. Biopsy. Brazil / epidemiology. Child. Female. Humans. Immunohistochemistry. Male. Middle Aged. Retrospective Studies. S100 Proteins / analysis. Young Adult

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  • (PMID = 19488471.001).
  • [ISSN] 1807-3107
  • [Journal-full-title] Brazilian oral research
  • [ISO-abbreviation] Braz Oral Res
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Brazil
  • [Chemical-registry-number] 0 / S100 Proteins
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5. Zheng L, Zhang JG, Yu GY, Gao Y: [Malignant peripheral nerve sheath tumors in oral maxillofacial region: clinical analysis of 11 cases]. Beijing Da Xue Xue Bao; 2010 Apr 18;42(2):216-20
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  • [Title] [Malignant peripheral nerve sheath tumors in oral maxillofacial region: clinical analysis of 11 cases].
  • OBJECTIVE: To investigate the clinicopathologic features, treatment and prognosis of the malignant peripheral nerve sheath tumors (MPNST) in oral and maxillofacial region.
  • CONCLUSION: MPNST is one of the most aggressive tumors with high risk of local recurrence and distant metastasis.
  • Complete removal of the tumor is the main modality of treatment and a most important prognostic factor of MPNST.
  • [MeSH-major] Mandibular Neoplasms / diagnosis. Maxillary Neoplasms / diagnosis. Mouth Neoplasms / diagnosis. Nerve Sheath Neoplasms / diagnosis
  • [MeSH-minor] Adult. Aged. Child. Female. Humans. Male. Middle Aged. Prognosis. Retrospective Studies. Young Adult

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  • (PMID = 20396368.001).
  • [ISSN] 1671-167X
  • [Journal-full-title] Beijing da xue xue bao. Yi xue ban = Journal of Peking University. Health sciences
  • [ISO-abbreviation] Beijing Da Xue Xue Bao
  • [Language] chi
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] China
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6. 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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7. Holtkamp N, Atallah I, Okuducu AF, Mucha J, Hartmann C, Mautner VF, Friedrich RE, Mawrin C, von Deimling A: MMP-13 and p53 in the progression of malignant peripheral nerve sheath tumors. Neoplasia; 2007 Aug;9(8):671-7
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  • [Title] MMP-13 and p53 in the progression of malignant peripheral nerve sheath tumors.
  • Malignant peripheral nerve sheath tumors (MPNST) are sarcomas with poor prognosis and limited treatment options.
  • Factors contributing to tumor progression are largely unknown.
  • We therefore examined MPNST from 22 neurofibromatosis type 1 (NF1) patients, 14 non-NF1 patients, and 14 neurofibroma patients for matrix metalloproteinase 13 (MMP-13) expression.
  • MMP-13 expression was detected in 58% of MPNST and was significantly associated with recurrent MPNST (P = .019).
  • p53 was observed in 78% of MPNST and was found to be strongly associated with MMP-13 expression (P = .005).
  • TP53 mutations were found in only 11% of MPNST and were associated with high tumor grades (P = .029).
  • No significant association between mutant TP53 and MMP-13 was observed, indicating that other factors drive MMP-13 expression in MPNST.
  • In summary, our data suggest that MMP-13 expression in nerve sheath tumors is coupled with malignant progression.
  • [MeSH-major] Biomarkers, Tumor / physiology. Genes, p53 / physiology. Matrix Metalloproteinase 13 / physiology. Nerve Sheath Neoplasms / metabolism. Nerve Sheath Neoplasms / pathology
  • [MeSH-minor] Adolescent. Adult. Aged. Disease Progression. Female. Follow-Up Studies. Gene Expression Regulation, Neoplastic / physiology. Humans. Male. Middle Aged. Mutation. Neurofibroma / genetics. Neurofibroma / metabolism. Neurofibroma / pathology

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  • (PMID = 17786186.001).
  • [ISSN] 1476-5586
  • [Journal-full-title] Neoplasia (New York, N.Y.)
  • [ISO-abbreviation] Neoplasia
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Canada
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; EC 3.4.24.- / Matrix Metalloproteinase 13
  • [Other-IDs] NLM/ PMC1950437
  • [Keywords] NOTNLM ; Malignant peripheral nerve sheath tumor / TP53 / malignant progression / matrix metalloproteinase 13 / neurofibromatosis type 1
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8. Ozturk E, Erdem I, Sonmez G, Haholu A, Sildiroglu HO, Mutlu H, Basekim CC, Kizilkaya E: Multicentric malignant peripheral nerve sheath tumor. Clin Imaging; 2007 Sep-Oct;31(5):363-6
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  • [Title] Multicentric malignant peripheral nerve sheath tumor.
  • We present a case of malignant peripheral nerve sheath tumor of multicentric origin, an extremely rare condition.
  • After surgical resection of the masses from the thoracic and inguinal regions, histological examination confirmed the preoperative diagnosis of malignant peripheral nerve sheath tumor.
  • [MeSH-major] Magnetic Resonance Imaging / methods. Neoplasms, Multiple Primary / diagnosis. Nerve Sheath Neoplasms / diagnosis. Tomography, X-Ray Computed / methods
  • [MeSH-minor] Adult. Humans. Male

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  • (PMID = 17825749.001).
  • [ISSN] 0899-7071
  • [Journal-full-title] Clinical imaging
  • [ISO-abbreviation] Clin Imaging
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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9. Thomas C, Somani N, Owen LG, Malone JC, Billings SD: Cutaneous malignant peripheral nerve sheath tumors. J Cutan Pathol; 2009 Aug;36(8):896-900
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  • [Title] Cutaneous malignant peripheral nerve sheath tumors.
  • Cutaneous malignant peripheral nerve sheath tumors (MPNSTs) are rare entities compared with their deep soft tissue counterparts.
  • The second case presented in an 88-year-old man with no stigmata of neurofibromatosis as a rapidly growing subcutaneous tumor of the right calf.
  • The diagnosis of MPNST was rendered in both cases.
  • [MeSH-minor] Adult. Aged, 80 and over. Female. Humans. Male. Mitosis

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  • (PMID = 19586501.001).
  • [ISSN] 1600-0560
  • [Journal-full-title] Journal of cutaneous pathology
  • [ISO-abbreviation] J. Cutan. Pathol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Denmark
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10. Fukushima S, Kageshita T, Wakasugi S, Matsushita S, Kaguchi A, Ishihara T, Ono T: Giant malignant peripheral nerve sheath tumor of the scalp. J Dermatol; 2006 Dec;33(12):865-8
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Giant malignant peripheral nerve sheath tumor of the scalp.
  • Herein, we describe a rare case of giant malignant peripheral nerve sheath tumor of the head in a 38-year-old Japanese man.
  • The tumor measured 210 mm at its largest diameter and was ulcerated, hemorrhagic, multilocular and non-mobile.
  • It should be noted that the patient stubbornly refused to see a doctor for a long time, resulting in the extreme growth of the tumor.
  • Dermatohistopathological findings of the biopsy indicated ancient schwannoma and total excision was therefore performed.
  • Post-mortem skin biopsy revealed features of malignant peripheral nerve sheath tumor.
  • We propose that the expressions of Ki67 and p16 should be checked for all lesions of peripheral nerve sheath tumor for distinguishing benign from malignant forms.
  • [MeSH-major] Neurilemmoma / diagnosis. Scalp / pathology. Skin Neoplasms / diagnosis
  • [MeSH-minor] Adult. Biomarkers, Tumor / analysis. Cyclin-Dependent Kinase Inhibitor p16 / analysis. Fatal Outcome. Hemorrhage / pathology. Humans. Ki-67 Antigen / analysis. Male. Skin Ulcer / pathology

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  • (PMID = 17169091.001).
  • [ISSN] 0385-2407
  • [Journal-full-title] The Journal of dermatology
  • [ISO-abbreviation] J. Dermatol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Japan
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Cyclin-Dependent Kinase Inhibitor p16; 0 / Ki-67 Antigen
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11. Rahman M, Cook DS, Ellis G, O'keefe PA: Malignant peripheral nerve sheath tumor of the heart. Asian Cardiovasc Thorac Ann; 2006 Oct;14(5):425-7
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Malignant peripheral nerve sheath tumor of the heart.
  • A 32-year-old man presented with dyspnea, chest pain, palpitations and ankle edema and was found to have a tumor involving the heart.
  • This was diagnosed as malignant peripheral nerve sheath tumor, a rare sarcoma of the heart.
  • [MeSH-major] Heart Neoplasms / diagnosis. Nerve Sheath Neoplasms / diagnosis
  • [MeSH-minor] Adult. Fatal Outcome. Humans. Male

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  • (PMID = 17005894.001).
  • [ISSN] 1816-5370
  • [Journal-full-title] Asian cardiovascular & thoracic annals
  • [ISO-abbreviation] Asian Cardiovasc Thorac Ann
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] England
  • [Number-of-references] 8
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12. Gheisari R, Roozbehi A: Malignant peripheral nerve sheath tumor of the infratemporal fossa. J Craniofac Surg; 2010 Mar;21(2):596-8
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Malignant peripheral nerve sheath tumor of the infratemporal fossa.
  • Malignant peripheral nerve sheath tumor is a rare pathologic lesion in a patient without neurofibromatosis diseases.
  • Clinical diagnosis of this tumor was made with delay owing to the lack of initial sign and symptoms and its location.
  • [MeSH-major] Facial Neoplasms / diagnosis. Nerve Sheath Neoplasms / diagnosis. Soft Tissue Neoplasms / diagnosis. Temporal Bone / pathology
  • [MeSH-minor] Adult. Diagnosis, Differential. Humans. Male. Paresthesia / diagnosis. S100 Proteins / analysis. Tomography, X-Ray Computed. Vimentin / analysis

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  • (PMID = 20489461.001).
  • [ISSN] 1536-3732
  • [Journal-full-title] The Journal of craniofacial surgery
  • [ISO-abbreviation] J Craniofac Surg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / S100 Proteins; 0 / Vimentin
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13. 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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14. Nicklas BJ, Smith AD, Wolff RD, Johnson FA: Malignant peripheral nerve sheath tumor arising in association with the sural nerve. J Foot Ankle Surg; 2006 Jan-Feb;45(1):38-41
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Malignant peripheral nerve sheath tumor arising in association with the sural nerve.
  • Malignant peripheral nerve sheath tumor is a rare sarcoma of peripheral nerves found most often in deep soft tissue.
  • This aggressive tumor is difficult to diagnose clinically and must be surgically excised for therapy.
  • The authors present a case of a 39-year-old African American woman with malignant peripheral nerve sheath tumor in association with the sural nerve.
  • The tumor was surgically removed and sent for pathologic studies.
  • [MeSH-major] Nerve Sheath Neoplasms / pathology. Soft Tissue Neoplasms / pathology. Sural Nerve / pathology
  • [MeSH-minor] Adult. Female. Humans. Magnetic Resonance Imaging

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  • (PMID = 16399558.001).
  • [ISSN] 1067-2516
  • [Journal-full-title] The Journal of foot and ankle surgery : official publication of the American College of Foot and Ankle Surgeons
  • [ISO-abbreviation] J Foot Ankle Surg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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15. Zhang J, Sun Y, Peng ZL: Malignant peripheral nerve sheath tumor of the vagina. Saudi Med J; 2009 May;30(5):705-7
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  • [Title] Malignant peripheral nerve sheath tumor of the vagina.
  • Malignant peripheral nerve sheath tumors (MPNSTs) usually develop in major nerve trunks, giving rise to tumors in the proximal portions of the upper and lower extremities and trunk.
  • We describe a case of MPNST of the vagina and discuss its diagnosis and treatment.
  • [MeSH-major] Peripheral Nerves / pathology. Vaginal Neoplasms / diagnosis
  • [MeSH-minor] Adult. Female. Humans. Treatment Outcome

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  • (PMID = 19417975.001).
  • [ISSN] 0379-5284
  • [Journal-full-title] Saudi medical journal
  • [ISO-abbreviation] Saudi Med J
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Saudi Arabia
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16. 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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17. Al Akloby O, Bukhari IA, El-Shawarby M, Al Mulhim F: Malignant peripheral nerve sheath tumor of the skin: case report. Am J Clin Dermatol; 2006;7(3):201-3
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Malignant peripheral nerve sheath tumor of the skin: case report.
  • Malignant peripheral nerve sheath tumors (MPNSTs) are rare tumors derived from Schwann cells or pluripotent cells of the neural crest.
  • In this report, we describe a 21-year-old Saudi woman who presented with an asymptomatic, solid, round, protruding tumor on the right upper back which was diagnosed as an MPNST with no stigmata of neurofibromatosis.
  • [MeSH-major] Nerve Sheath Neoplasms / diagnosis. Skin Neoplasms / diagnosis
  • [MeSH-minor] Adult. Back. Female. Humans. Magnetic Resonance Imaging

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  • (PMID = 16734508.001).
  • [ISSN] 1175-0561
  • [Journal-full-title] American journal of clinical dermatology
  • [ISO-abbreviation] Am J Clin Dermatol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] New Zealand
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18. Kumar P, Jaiswal S, Agrawal T, Verma A, Datta NR: Malignant peripheral nerve sheath tumor of the occipital region: case report. Neurosurgery; 2007 Dec;61(6):E1334-5; discussion E1335
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Malignant peripheral nerve sheath tumor of the occipital region: case report.
  • OBJECTIVE: A rare case of a malignant peripheral nerve sheath tumor of the occipital region is presented.
  • INTERVENTION: A small occipital craniectomy for total excision of the tumor was attempted.
  • However, as a result of intracranial extension to the transverse sinus, the tumor could not be completely excised.
  • CONCLUSION: An aggressive malignant peripheral nerve sheath tumor of an atypical site such as the scalp, in which complete surgery may not always be possible, could benefit from postoperative radiotherapy.
  • [MeSH-major] Brain Neoplasms. Nerve Sheath Neoplasms. Occipital Lobe / pathology
  • [MeSH-minor] Adult. Craniotomy / methods. Humans. Male. Tomography, X-Ray Computed / methods

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  • (PMID = 18162865.001).
  • [ISSN] 1524-4040
  • [Journal-full-title] Neurosurgery
  • [ISO-abbreviation] Neurosurgery
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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19. Gachiani J, Kim D, Nelson A, Kline D: Surgical management of malignant peripheral nerve sheath tumors. Neurosurg Focus; 2007;22(6):E13

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Surgical management of malignant peripheral nerve sheath tumors.
  • OBJECT: The aim of this study was to describe the presentation of patients harboring soft tissue sarcomas involving the nerves, most of which were malignant peripheral nerve sheath tumors (MPNSTs), and provide an algorithm for their treatment.
  • Tumor classifications are presented, together with patient numbers, locations of MPNSTs, surgical techniques, and adjunctive treatments.
  • CONCLUSIONS: Malignant peripheral nerve sheath tumors are rare.
  • [MeSH-major] Nerve Sheath Neoplasms / diagnosis. Nerve Sheath Neoplasms / surgery
  • [MeSH-minor] Adolescent. Adult. Aged. Aged, 80 and over. Antineoplastic Agents / therapeutic use. Diagnosis, Differential. Disease Management. Humans. Middle Aged. Retrospective Studies

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  • (PMID = 17613204.001).
  • [ISSN] 1092-0684
  • [Journal-full-title] Neurosurgical focus
  • [ISO-abbreviation] Neurosurg Focus
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents
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20. Nakagawa Y, Yoshida A, Numoto K, Kunisada T, Wai D, Ohata N, Takeda K, Kawai A, Ozaki T: Chromosomal imbalances in malignant peripheral nerve sheath tumor detected by metaphase and microarray comparative genomic hybridization. Oncol Rep; 2006 Feb;15(2):297-303
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  • [Title] Chromosomal imbalances in malignant peripheral nerve sheath tumor detected by metaphase and microarray comparative genomic hybridization.
  • Malignant peripheral nerve sheath tumors (MPNSTs) are highly malignant tumors affecting adolescents and adults.
  • There have been a few reports on chromosomal aberrations of MPNSTs; however, the tumor-specific alteration remains unknown.
  • [MeSH-major] Chromosome Aberrations. Nerve Sheath Neoplasms / genetics. Nucleic Acid Hybridization. Oligonucleotide Array Sequence Analysis. Soft Tissue Neoplasms / genetics
  • [MeSH-minor] Adult. Aged. Child. Female. Gene Dosage. Humans. Male. Metaphase. Middle Aged. Reproducibility of Results


21. 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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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Malignant peripheral nerve sheath tumor in 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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22. 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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23. Marçal NS, Teixeira E, Sotto-Mayor R, Manique A, Campos P, Cruz J, Mendes de Almeida M, Bugalho de Almeida A: [Malignant peripheral nerve sheath tumors: A case report]. Rev Port Pneumol; 2010 May-Jun;16(3):483-92
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  • [Title] [Malignant peripheral nerve sheath tumors: A case report].
  • [Transliterated title] Tumor maligno da bainha dos nervos periféricos do pulmão: A propósito de um caso clínico.
  • Malignant peripheral nerve sheath tumors comprehend a rare group of soft tissue sarcomas that tend to occur in patients with neurofibromatosis type 1 or several years after radiotherapy treatments.
  • The typical symptoms are due to nerve roots compression which can persist for several months or years before diagnosis.
  • Due to very few patients with this type of tumor its therapeutic approach is still a matter of permanent debate, being surgery the main treatment.
  • This tumor has a bad prognosis because of high local recurrence and metastasis.
  • [MeSH-major] Lung Neoplasms. Nerve Sheath Neoplasms
  • [MeSH-minor] Adult. Humans. Male

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  • (PMID = 20635063.001).
  • [ISSN] 2172-6825
  • [Journal-full-title] Revista portuguesa de pneumologia
  • [ISO-abbreviation] Rev Port Pneumol
  • [Language] por
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Portugal
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24. 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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25. Chen L, Mao Y, Chen H, Zhou LF: Diagnosis and management of intracranial malignant peripheral nerve sheath tumors. Neurosurgery; 2008 Apr;62(4):825-32; discussion 832

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Diagnosis and management of intracranial malignant peripheral nerve sheath tumors.
  • OBJECTIVE: Intracranial malignant peripheral nerve sheath tumors (MPNSTs) are rare and generally carry a poor prognosis.
  • The general strategy was to perform complete resection whenever possible and to provide adjuvant radiotherapy for residual tumor.
  • Total tumor resection was achieved in five patients.
  • There was one case of near total (>90%) and two cases of partial (<90%) tumor removal; the postoperative survival rate was 4, 4, and 2 months, respectively.
  • [MeSH-major] Cranial Nerve Neoplasms / diagnosis. Cranial Nerve Neoplasms / surgery. Nerve Sheath Neoplasms / diagnosis. Nerve Sheath Neoplasms / surgery
  • [MeSH-minor] Adolescent. Adult. Child. Child, Preschool. Female. Humans. Male. Middle Aged. Treatment Outcome

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  • (PMID = 18496188.001).
  • [ISSN] 1524-4040
  • [Journal-full-title] Neurosurgery
  • [ISO-abbreviation] Neurosurgery
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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26. 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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  • [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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27. Verdijk RM, den Bakker MA, Dubbink HJ, Hop WC, Dinjens WN, Kros JM: TP53 mutation analysis of malignant peripheral nerve sheath tumors. J Neuropathol Exp Neurol; 2010 Jan;69(1):16-26
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  • [Title] TP53 mutation analysis of malignant peripheral nerve sheath tumors.
  • Mutations in TP53 underlie the development of malignant peripheral nerve sheath tumors (MPNSTs) in animal models, but there is controversy regarding the extent of TP53 mutations in human MPNSTs.
  • A minority of patients (n = 26, 30%) had neurofibromatosis type 1 (NF1); in these patients, the diagnosis of MPNST was made at younger ages (33 [SD, 3.6] years vs 49 [SD, 2.9] years in NF1 vs non-NF1; p = 0.003).
  • These results indicate that TP53 mutations are relatively rare in human MPNST and that they are not positively correlated with the presence of NF1.
  • [MeSH-major] Genetic Predisposition to Disease / genetics. Mutation / genetics. Nerve Sheath Neoplasms / genetics. Peripheral Nervous System Neoplasms / genetics. Tumor Suppressor Protein p53 / genetics
  • [MeSH-minor] Adolescent. Adult. Aged. Aged, 80 and over. Chi-Square Distribution. Child. DNA Mutational Analysis. Female. Humans. Male. Middle Aged. Neurofibromatosis 1 / genetics. Retrospective Studies. Statistics, Nonparametric. Ubiquitin-Protein Ligases / metabolism. Young Adult

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  • (PMID = 20010306.001).
  • [ISSN] 1554-6578
  • [Journal-full-title] Journal of neuropathology and experimental neurology
  • [ISO-abbreviation] J. Neuropathol. Exp. Neurol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Tumor Suppressor Protein p53; EC 6.3.2.19 / MIB1 ligase, human; EC 6.3.2.19 / Ubiquitin-Protein Ligases
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28. Katenkamp K, Katenkamp D: [Low-malignant peripheral nerve sheath tumors of nasal and sinonasal mucous membranes]. Pathologe; 2005 Mar;26(2):90-5

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Low-malignant peripheral nerve sheath tumors of nasal and sinonasal mucous membranes].
  • Sinonasal malignant peripheral nerve sheath tumors (MPNST) are infrequent neoplasms.
  • 16 cases of low-malignant MPNST in this localization were retrieved from the files of soft tissue tumors established in Jena.
  • The importance of an only partial immunostaining by S100 protein antibodies for diagnosis and differential diagnostic discrimination to benign peripheral nerve sheath tumors (schwannomas and neurofibromas) is explained.
  • [MeSH-major] Nerve Sheath Neoplasms / pathology. Paranasal Sinus Neoplasms / pathology. S100 Proteins / analysis. Soft Tissue Neoplasms / pathology
  • [MeSH-minor] Adult. Aged. Biomarkers, Tumor / analysis. Diagnosis, Differential. Female. Humans. Male. Middle Aged

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  • (PMID = 15657686.001).
  • [ISSN] 0172-8113
  • [Journal-full-title] Der Pathologe
  • [ISO-abbreviation] Pathologe
  • [Language] ger
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / S100 Proteins
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29. Wasa J, Nishida Y, Tsukushi S, Shido Y, Sugiura H, Nakashima H, Ishiguro N: MRI features in the differentiation of malignant peripheral nerve sheath tumors and neurofibromas. AJR Am J Roentgenol; 2010 Jun;194(6):1568-74
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] MRI features in the differentiation of malignant peripheral nerve sheath tumors and neurofibromas.
  • OBJECTIVE: The objective of this study was to identify the MRI criteria that best differentiate malignant peripheral nerve sheath tumors from benign neurofibromas.
  • MATERIALS AND METHODS: We retrospectively analyzed MR images obtained for 41 histologically diagnosed cases of malignant peripheral nerve sheath tumor and 20 cases of neurofibroma that had been treated at four tertiary institutions.
  • Twenty of the patients with malignant peripheral nerve sheath tumors and 14 patients with neurofibromas developed the disease in association with neurofibromatosis 1.
  • The MR images were evaluated with regard to tumor size, signal intensity, heterogeneity of T1- and T2-weighted MR images, enhancement pattern, definition of margins, presence of perilesional edemalike zone, and presence of intratumoral cystic lesions.
  • RESULTS: Significant differences between malignant peripheral nerve sheath tumors and neurofibromas were noted for the largest dimension of the mass, peripheral enhancement pattern, perilesional edemalike zone, and intratumoral cystic lesion.
  • In cases associated with neurofibromatosis 1, heterogenicity on T1-weighted images was also significant in differentiating neurofibroma from malignant peripheral nerve sheath tumor.
  • The presence of two or more of the four features suggestive of malignancy indicated malignant peripheral nerve sheath tumor with a sensitivity of 61% and a specificity of 90%.
  • CONCLUSION: The MR features described in this study are useful for distinguishing malignant peripheral nerve sheath tumors from neurofibromas.
  • If a tumor has two or more of the four statistically significant features, it can be considered to be highly suspicious of malignancy and should be subjected to a biopsy for early diagnosis.
  • [MeSH-major] Magnetic Resonance Imaging / methods. Nerve Sheath Neoplasms / diagnosis. Neurofibromatosis 1 / pathology
  • [MeSH-minor] Adolescent. Adult. Aged. Aged, 80 and over. Chi-Square Distribution. Contrast Media. Diagnosis, Differential. Female. Humans. Male. Middle Aged. Neurofibroma / diagnosis. Neurofibroma / pathology. Retrospective Studies. Statistics, Nonparametric

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  • (PMID = 20489098.001).
  • [ISSN] 1546-3141
  • [Journal-full-title] AJR. American journal of roentgenology
  • [ISO-abbreviation] AJR Am J Roentgenol
  • [Language] eng
  • [Publication-type] Journal Article; Multicenter Study; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Contrast Media
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30. Matsuda E, Okabe K, Matsuoka T, Hirazawa K, Azuma T, Murakami T, Sugi K: [Lung metastasis of malignant peripheral nerve sheath tumor: report of a case]. Kyobu Geka; 2007 Sep;60(10):950-3
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Lung metastasis of malignant peripheral nerve sheath tumor: report of a case].
  • He had underwent extended radical tumorectomy for malignant peripheral nerve sheath tumor (MPNST) in left lower limb 33 months before.
  • Chest X-ray and computed tomography (CT) scan revealed solitary tumor on right S10.
  • Tumor was resected under thoracoscopic surgery.
  • Histological diagnosis was metastasis of MPNST.
  • MPNST with lung metastasis showing very poor prognosis.
  • Careful follow up is important for MPNST.
  • [MeSH-major] Lung Neoplasms / secondary. Nerve Sheath Neoplasms / secondary. Peripheral Nervous System Neoplasms / pathology
  • [MeSH-minor] Adult. Humans. Male. Neurofibromatosis 1 / complications. Prognosis. Survivors. Thoracoscopy. Tomography, X-Ray Computed

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  • (PMID = 17877020.001).
  • [ISSN] 0021-5252
  • [Journal-full-title] Kyobu geka. The Japanese journal of thoracic surgery
  • [ISO-abbreviation] Kyobu Geka
  • [Language] jpn
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Japan
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31. 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


32. 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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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Malignant peripheral nerve sheath tumor of 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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33. Houreih MA, Eyden B, Deolekar M, Banerjee S: A case of fibroblastic low-grade malignant peripheral nerve sheath tumor--a true neurofibrosarcoma. Ultrastruct Pathol; 2007 Sep-Oct;31(5):347-56
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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 fibroblastic low-grade malignant peripheral nerve sheath tumor--a true neurofibrosarcoma.
  • The authors report a case of low-grade retroperitoneal malignant peripheral nerve sheath tumor (MPNST) showing Schwannian and fibroblastic differentiation in individual tumor cells.
  • The tumor was detected in a 29-year-old male and posed diagnostic difficulty because of the unusual morphologic and immunophenotypic features.
  • Morphologic examination of the H&E sections revealed a rather circumscribed, highly vascular, moderately cellular spindle cell tumor.
  • There were satellite nodules outside the main tumor mass and low mitotic activity but no necrosis.
  • The tumor cells stained strongly and diffusely for both S-100 protein and CD34.
  • Although the capacity of MPNST to exhibit divergent differentiation is well known, fibroblastic differentiation is generally poorly and inconsistently documented.
  • The present case represents an unambiguous demonstration of the co-expression within individual tumor cells of Schwannian and fibroblastic differentiation in a low-grade MPNST.
  • [MeSH-major] Fibroblasts / pathology. Nerve Sheath Neoplasms / pathology. Neurofibrosarcoma / pathology
  • [MeSH-minor] Adult. Antigens, CD34 / analysis. Biomarkers, Tumor / analysis. Cell Nucleus / ultrastructure. Cytoplasm / ultrastructure. Humans. Male. Microscopy, Electron, Transmission. S100 Proteins / analysis. Schwann Cells / ultrastructure

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  • (PMID = 17963184.001).
  • [ISSN] 1521-0758
  • [Journal-full-title] Ultrastructural pathology
  • [ISO-abbreviation] Ultrastruct Pathol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antigens, CD34; 0 / Biomarkers, Tumor; 0 / S100 Proteins
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34. Allison KH, Patel RM, Goldblum JR, Rubin BP: Superficial malignant peripheral nerve sheath tumor: a rare and challenging diagnosis. Am J Clin Pathol; 2005 Nov;124(5):685-92
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Superficial malignant peripheral nerve sheath tumor: a rare and challenging diagnosis.
  • We reviewed the clinicopathologic features of 5 cases of malignant peripheral nerve sheath tumor (MPNST) manifesting in superficial locations associated with cutaneous neurofibromas (4 cases) or superficial peripheral nerve (1 case).
  • However, identification of a benign precursor or origin from a nerve may be the most definitive way to properly classify these rare lesions.
  • [MeSH-major] Nerve Sheath Neoplasms / diagnosis
  • [MeSH-minor] Adolescent. Adult. Aged. Diagnosis, Differential. Female. Humans. Immunohistochemistry. Male. S100 Proteins / analysis

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  • (PMID = 16203275.001).
  • [ISSN] 0002-9173
  • [Journal-full-title] American journal of clinical pathology
  • [ISO-abbreviation] Am. J. Clin. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / S100 Proteins
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35. 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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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [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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36. Hara M, Matsuzaki Y, Shimizu T, Tomita M, Ayabe T, Enomoto Y, Wada S, Tanaka H, Kataoka H, Nabeshima K, Onitsuka T: Malignant peripheral nerve sheath tumor with Horner's syndrome: a case report. Ann Thorac Cardiovasc Surg; 2008 Aug;14(4):246-8
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Malignant peripheral nerve sheath tumor with Horner's syndrome: a case report.
  • We report on a 42-year-old woman with malignant peripheral nerve sheath tumor (MPNST) arising from the cervical sympathetic nerve.
  • The mass was spindle shaped and connected to the sympathetic trunk on the cranial and caudal sides, and it compressed the left carotid sheath on the median side.
  • [MeSH-major] Horner Syndrome / complications. Peripheral Nervous System Neoplasms / pathology. Sympathetic Nervous System / pathology
  • [MeSH-minor] Adult. Female. Humans. Magnetic Resonance Imaging. Radiotherapy, Adjuvant. Sternum / surgery. Tomography, X-Ray Computed. Treatment Outcome

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  • (PMID = 18818575.001).
  • [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; Review
  • [Publication-country] Japan
  • [Number-of-references] 8
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37. 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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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [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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38. Sanchez-Mejia RO, Pham DN, Prados M, Tihan T, Cha S, El-Sayed I, McDermott MW: Management of a sporadic malignant subfrontal peripheral nerve sheath tumor. J Neurooncol; 2006 Jan;76(2):165-9
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Management of a sporadic malignant subfrontal peripheral nerve sheath tumor.
  • Malignant subfrontal (olfactory) peripheral nerve sheath tumors (MPNSTs) are exceedingly rare.
  • In this paper, we describe a patient with a subfrontal MPNST with unusual histological characteristics and present a review of the literature.
  • The patient underwent both a bifrontal transbasal craniotomy and a transnasal approach for an attempt at total resection of both the intradural and extradural components of the MPNST.
  • The specific cell and nerve of origin for these tumors remains unknown.
  • Our case shows that these rare lesions can present as a malignant variant and thus require aggressive surgical and postoperative management to provide long-term tumor control.
  • [MeSH-major] Brain Neoplasms / pathology. Nerve Sheath Neoplasms / pathology
  • [MeSH-minor] Adult. Craniotomy. Female. Headache / etiology. Humans. Magnetic Resonance Imaging. Neurosurgical Procedures. Olfaction Disorders / etiology. Tomography, X-Ray Computed

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  • (PMID = 16132491.001).
  • [ISSN] 0167-594X
  • [Journal-full-title] Journal of neuro-oncology
  • [ISO-abbreviation] J. Neurooncol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Netherlands
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39. 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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40. Rawal A, Yin Q, Roebuck M, Sinopidis C, Kalogrianitis S, Helliwell TR, Frostick S: Atypical and malignant peripheral nerve-sheath tumors of the brachial plexus: report of three cases and review of the literature. Microsurgery; 2006;26(2):80-6

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

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  • (PMID = 16538633.001).
  • [ISSN] 0738-1085
  • [Journal-full-title] Microsurgery
  • [ISO-abbreviation] Microsurgery
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 24
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41. Hagel C, Zils U, Peiper M, Kluwe L, Gotthard S, Friedrich RE, Zurakowski D, von Deimling A, Mautner VF: Histopathology and clinical outcome of NF1-associated vs. sporadic malignant peripheral nerve sheath tumors. J Neurooncol; 2007 Apr;82(2):187-92
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Histopathology and clinical outcome of NF1-associated vs. sporadic malignant peripheral nerve sheath tumors.
  • The differences in the clinical course and histopathology of sporadic and neurofibromatosis type 1 (NF1)-associated malignant peripheral nerve sheath tumors (MPNST) were investigated retrospectively.
  • In patients with the original histopathological data available (22 NF1 patients, 14 sporadic cases), NF1-associated MPNST showed a significantly higher cellularity compared to sporadic tumors (p<0.001) whereas sporadic MPNST featured a significantly higher pleomorphism (p<0.01).
  • Most importantly, while histopathological variables correlated with French Fédération Nationale des Centres de Lutte Contre le Cancer grading in sporadic MPNST, this was not the case for NF1-associated tumors.
  • The differences between NF1-associated and sporadic MPNST in regard to the clinical course and histopathology may reflect some fundamental differences in biology and pathomechanism of the two tumor groups.
  • [MeSH-major] Nerve Sheath Neoplasms / pathology. Neurofibromatosis 1 / pathology. Peripheral Nervous System Neoplasms / pathology
  • [MeSH-minor] Adolescent. Adult. Aged. Child. Female. Humans. Incidence. Male. Middle Aged. Survival Rate

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  • (PMID = 17111191.001).
  • [ISSN] 0167-594X
  • [Journal-full-title] Journal of neuro-oncology
  • [ISO-abbreviation] J. Neurooncol.
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Netherlands
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42. Park SK, Yi HJ, Paik SS, Kim YJ, Ko Y, Oh SJ: Metastasizing malignant peripheral nerve sheath tumor initially presenting as intracerebral hemorrhage. Case report and review of the literature. Surg Neurol; 2007 Jul;68(1):79-84; discussion 84
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  • [Title] Metastasizing malignant peripheral nerve sheath tumor initially presenting as intracerebral hemorrhage. Case report and review of the literature.
  • BACKGROUND: Malignant peripheral nerve sheath tumors, infrequent sarcomas arising within a peripheral nerve, mostly metastasize to the lung at terminal stage of disease.
  • Surgical removal and adjuvant therapy was performed for pathologically proven MPNST.
  • Concurrent painful chest masses were also confirmed as MPNST through surgical resection.
  • The MPNST actually can exhibit an apoplectic manifestation even without pulmonary involvement in a young adult, albeit this is quite rare.
  • Thus, high index of suspicion should be paid to minute complaints regarding MPNST in peripheral locations so as not to overlook an advanced or metastasized disease.
  • [MeSH-major] Brain Neoplasms / diagnosis. Brain Neoplasms / secondary. Cerebral Hemorrhage / etiology. Nerve Sheath Neoplasms / complications. Nerve Sheath Neoplasms / secondary. Peripheral Nervous System Neoplasms / pathology
  • [MeSH-minor] Adult. Craniotomy. Fatal Outcome. Humans. Lung Neoplasms / radiography. Lung Neoplasms / secondary. Magnetic Resonance Imaging. Male. Radiography, Thoracic. Tomography, X-Ray Computed

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  • (PMID = 17586234.001).
  • [ISSN] 0090-3019
  • [Journal-full-title] Surgical neurology
  • [ISO-abbreviation] Surg Neurol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 17
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43. Baek WS, Pytel P, Undevia SD, Rubeiz H: Spinal cord metastasis of a non-neurofibromatosis type-1 malignant peripheral nerve sheath tumor: an unusual manifestation of a rare tumor. J Neurooncol; 2005 Sep;74(2):183-5
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  • [Title] Spinal cord metastasis of a non-neurofibromatosis type-1 malignant peripheral nerve sheath tumor: an unusual manifestation of a rare tumor.
  • Malignant peripheral nerve sheath tumors are rare spindle-cell sarcomas derived from Schwann cells or pluripotent cells of the neural crest.
  • They arise from the spinal roots, peripheral nerves, brachial and lumbosacral plexi, cranial nerves and terminal nerve fibers within soft tissue, intestine, lung and bone.
  • Spinal cord metastasis from malignant nerve sheath tumors associated with neurofibromatosis type 1 is very rare.
  • We describe a rare case of near-total spinal cord metastasis in a patient with malignant nerve sheath tumor in the absence of neurofibromatosis, and highlight the microscopic findings and natural history of this disease process.
  • [MeSH-major] Lung Neoplasms / secondary. Nerve Sheath Neoplasms / pathology. Neurofibroma / pathology. Spinal Cord Neoplasms / secondary
  • [MeSH-minor] Adult. Benzenesulfonates / therapeutic use. Chemotherapy, Adjuvant. Fatal Outcome. Female. Humans. Magnetic Resonance Imaging. Niacinamide / analogs & derivatives. Phenylurea Compounds. Pyridines / therapeutic use


44. Kóbori L, Nagy P, Máthé Z, Hartmann E, Doros A, Paku S, Dezso K, Sápi Z: Malignant peripheral nerve sheath tumor of the liver: a case report. Pathol Oncol Res; 2008 Sep;14(3):329-32
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  • [Title] Malignant peripheral nerve sheath tumor of the liver: a case report.
  • A large, rapidly growing malignant peripheral nerve sheath tumor (MPNST) of the liver in a young female patient, not associated with von Recklinghausen's disease, is presented.
  • As far as we know, this is the first reported, unambiguously proven "de novo" MPNST in the liver.
  • [MeSH-major] Liver Neoplasms / diagnosis. Nerve Sheath Neoplasms / diagnosis
  • [MeSH-minor] Diagnosis, Differential. Female. Humans. Young Adult

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  • (PMID = 18758998.001).
  • [ISSN] 1219-4956
  • [Journal-full-title] Pathology oncology research : POR
  • [ISO-abbreviation] Pathol. Oncol. Res.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] Netherlands
  • [Number-of-references] 14
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45. 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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46. Benz MR, Czernin J, Dry SM, Tap WD, Allen-Auerbach MS, Elashoff D, Phelps ME, Weber WA, Eilber FC: Quantitative F18-fluorodeoxyglucose positron emission tomography accurately characterizes peripheral nerve sheath tumors as malignant or benign. Cancer; 2010 Jan 15;116(2):451-8
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  • [Title] Quantitative F18-fluorodeoxyglucose positron emission tomography accurately characterizes peripheral nerve sheath tumors as malignant or benign.
  • BACKGROUND: Correct pretreatment classification is critical for optimizing diagnosis and treatment of patients with peripheral nerve sheath tumors (PNSTs).
  • The aim of this study was to evaluate whether F18-fluorodeoxyglucose positron emission tomography (FDG PET) can differentiate malignant (MPNST) from benign PNSTs.
  • METHODS: Thirty-four adult patients presenting with PNST who underwent a presurgical FDG PET/computed tomography (CT) scan between February 2005 and November 2008 were included in the study.
  • Tumors were characterized histologically, by FDG maximum standardized uptake value (SUV(max) [g/mL]), and by CT size (tumor maximal diameter [cm]).
  • The accuracy of FDG PET for differentiating MPNSTs from benign PNSTs (neurofibroma and schwannoma) was evaluated by receiver operating characteristic (ROC) curve analysis.
  • SUV(max) was significantly higher in MPNST compared with benign PNST (12.0 +/- 7.1 vs 3.4 +/- 1.8; P < .001).
  • By ROC curve analysis, SUV(max) reliably differentiated between benign and malignant PNSTs (area under the ROC curve of 0.97).
  • Given the difficulties in clinically evaluating PNST and in distinguishing benign PNST from MPNST, FDG PET imaging should be used for diagnostic intervention planning and for optimizing treatment strategies.

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  • (PMID = 19924789.001).
  • [ISSN] 0008-543X
  • [Journal-full-title] Cancer
  • [ISO-abbreviation] Cancer
  • [Language] ENG
  • [Grant] United States / NCI NIH HHS / CA / P50 CA086306; United States / NCI NIH HHS / CA / 5 P50 CA086306
  • [Publication-type] Clinical Trial; Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, Non-P.H.S.; Research Support, U.S. Gov't, P.H.S.
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Radiopharmaceuticals; 0Z5B2CJX4D / Fluorodeoxyglucose F18
  • [Other-IDs] NLM/ NIHMS324300; NLM/ PMC3188986
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47. Thanapaisal C, Koonmee S, Siritunyaporn S: Malignant peripheral nerve sheath tumor of breast in patient without Von Recklinghausen's neurofibromatosis: a case report. J Med Assoc Thai; 2006 Mar;89(3):377-9
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  • [Title] Malignant peripheral nerve sheath tumor of breast in patient without Von Recklinghausen's neurofibromatosis: a case report.
  • Malignant peripheral nerve sheath tumor (MPNST) of the breast without Von Recklinghausen's neurofibromatosis (VRN) is extremely rare.
  • The histologic diagnosis was made with immunohistochemical study in which the tumor showed positivity of vimentin, S-100 protein, neuron-specific protein(NSE), neurofilament protein(NF) and glial fibrillary acidic protein(GFAP).
  • [MeSH-major] Breast Neoplasms / diagnosis. Nerve Sheath Neoplasms / diagnosis
  • [MeSH-minor] Adult. Biopsy. Female. Glial Fibrillary Acidic Protein / analysis. Humans. Neurofibroma / diagnosis. Prognosis. Rare Diseases. S100 Proteins / analysis. Vimentin / analysis

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  • (PMID = 16696423.001).
  • [ISSN] 0125-2208
  • [Journal-full-title] Journal of the Medical Association of Thailand = Chotmaihet thangphaet
  • [ISO-abbreviation] J Med Assoc Thai
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Thailand
  • [Chemical-registry-number] 0 / Glial Fibrillary Acidic Protein; 0 / S100 Proteins; 0 / Vimentin
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48. 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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49. Shimizu K, Okita R, Uchida Y, Hihara J: Long survival after resection for lung metastasis of malignant peripheral nerve sheath tumor in neurofibromatosis 1. Ann Thorac Cardiovasc Surg; 2008 Oct;14(5):322-4
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  • [Title] Long survival after resection for lung metastasis of malignant peripheral nerve sheath tumor in neurofibromatosis 1.
  • A 31-year-old man with neurofibromatosis 1 (NF1) was admitted for the treatment of solitary lung tumor.
  • Nine months earlier he had undergone a large resection for malignant peripheral nerve sheath tumors (MPNSTs) in his back.
  • Surgical resection of the right lower lobe was performed, and the tumor was pathologically diagnosed as a metastasis of MPNST.
  • The survival of patients with pulmonary metastasis of MPNST is extremely poor, especially of those with NF1, but this patient has survived 5 years without recurrence.
  • [MeSH-major] Lung Neoplasms / surgery. Nerve Sheath Neoplasms / surgery. Neurofibromatosis 1 / surgery. Pneumonectomy
  • [MeSH-minor] Adult. Humans. Male. Time Factors. Tomography, X-Ray Computed. Treatment Outcome

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  • (PMID = 18989250.001).
  • [ISSN] 2186-1005
  • [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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50. Wimmer K: [Neurofibromatosis: the most frequent hereditary tumor predisposition syndrome]. Wien Med Wochenschr; 2005 Jun;155(11-12):273-80
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  • [Title] [Neurofibromatosis: the most frequent hereditary tumor predisposition syndrome].
  • [Transliterated title] Neurofibromatose: die häufigste Tumor-disponierende genetische Erkrankung.
  • However, the disorder should not be underestimated as a "mere cosmetic problem", since NF1 patients are at increased risk to also develop malignant tumours, such as malignant peripheral nerve sheath tumours (MPNST), juvenile myelomonocytic leukaemia (JMML), optic glioma and pheochomocytoma.
  • [MeSH-minor] Adolescent. Adult. Child. Chromosome Aberrations. DNA Mutational Analysis. Disease Susceptibility. Genetic Counseling. Genetic Predisposition to Disease / genetics. Genetic Testing. Genotype. Heterozygote Detection. Humans. Neurofibromin 1 / genetics. Neurofibromin 2 / genetics. Phenotype. Risk

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  • (PMID = 16035388.001).
  • [ISSN] 0043-5341
  • [Journal-full-title] Wiener medizinische Wochenschrift (1946)
  • [ISO-abbreviation] Wien Med Wochenschr
  • [Language] ger
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Austria
  • [Chemical-registry-number] 0 / Neurofibromin 1; 0 / Neurofibromin 2
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51. Pusceddu S, Bajetta E, Buzzoni R, Carcangiu ML, Platania M, Del Vecchio M, Ditto A: Primary uterine cervix melanoma resembling malignant peripheral nerve sheath tumor: a case report. Int J Gynecol Pathol; 2008 Oct;27(4):596-600
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  • [Title] Primary uterine cervix melanoma resembling malignant peripheral nerve sheath tumor: a case report.
  • A rare variant of malignant melanoma (MM) of the uterine cervix that mimics a malignant peripheral nerve sheath tumor (MPNST) is described.
  • Neoadjuvant chemotherapy and total abdominal hysterectomy and bilateral salpingo-ovariectomy plus pelvic lymphadenectomy were performed, and the diagnosis was MPNST, FIGO IIB.
  • A pathological review was obtained in our institution by a gynecological pathologist, who defined the primary neoplasm in the cervix as an MM, with a pattern of growth histologically simulating an MPNST, metastatic to the vagina.
  • To our knowledge, this is the first report in literature of MM of the uterine cervix resembling MPNST.
  • Despite its rarity, this variant of MM should be considered when a diagnosis of cervix MPNST is made.
  • [MeSH-major] Melanoma / pathology. Nerve Sheath Neoplasms / pathology. Uterine Cervical Neoplasms / pathology
  • [MeSH-minor] Adult. Diagnosis, Differential. Female. Histocytochemistry. Humans


52. Shimizu J, Arano Y, Murata T, Ishikawa N, Yachi T, Nomura T, Minato H: A case of intrathoracic giant malignant peripheral nerve sheath tumor in neurofibromatosis type I (von Recklinghausen's disease). Ann Thorac Cardiovasc Surg; 2008 Feb;14(1):42-7
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] A case of intrathoracic giant malignant peripheral nerve sheath tumor in neurofibromatosis type I (von Recklinghausen's disease).
  • She underwent surgery to alleviate respiratory and circulatory disorders caused by compression of the right lung and inferior vena cava due to the giant tumor.
  • Intraoperatively, the tumor was found to have originated from the 5th intercostal nerve.
  • The resected tumor was 20x17x15 cm in size and 2,300 g in weight.
  • It was histologically diagnosed as a malignant peripheral nerve sheath tumor.
  • Seven months after surgery, however, a recurrent tumor was found in the right thoracic cavity.
  • She died of rapid growth of recurrent tumor 3 months thereafter.
  • This tumor often complicates neurofibromatosis I and has a high frequency of local recurrence and distant metastasis, resulting in poor prognosis.
  • Neither an optimal extent of resection needed for complete resection of this tumor nor an optimal regimen of chemotherapy, radiotherapy, or other therapy for the tumor has yet been established.
  • [MeSH-major] Nerve Sheath Neoplasms / surgery. Neurofibromatosis 1 / complications. Thoracic Neoplasms / surgery
  • [MeSH-minor] Adult. Diagnosis, Differential. Fatal Outcome. Humans. Magnetic Resonance Imaging. Neoplasm Recurrence, Local. Tomography, X-Ray Computed

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  • (PMID = 18292741.001).
  • [ISSN] 1341-1098
  • [Journal-full-title] Annals of thoracic and cardiovascular surgery : official journal of the Association of Thoracic and Cardiovascular Surgeons of Asia
  • [ISO-abbreviation] Ann Thorac Cardiovasc Surg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Japan
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53. Miura T, Yamada Y, Muramaki M, Tanaka K, Hara I, Fujisawa M: [A case of retroperitoneal malignant peripheral nerve sheath tumor in a patient with neurofibromatosis]. Hinyokika Kiyo; 2006 Mar;52(3):207-9
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  • [Title] [A case of retroperitoneal malignant peripheral nerve sheath tumor in a patient with neurofibromatosis].
  • We report a case of retroperitoneal malignant peripheral nerve sheath tumor (MPNST) in a patient with neurofibromatosis 1.
  • We performed complete resection of the tumor, confirming the margin status by frozen section examination intraoperatively.
  • The histopathological examination revealed MPNST.
  • [MeSH-major] Nerve Sheath Neoplasms / surgery. Neurofibromatosis 1 / complications. Retroperitoneal Neoplasms / surgery
  • [MeSH-minor] Adult. Female. Humans. Magnetic Resonance Imaging. Prognosis. Tomography, X-Ray Computed

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  • (PMID = 16617875.001).
  • [ISSN] 0018-1994
  • [Journal-full-title] Hinyokika kiyo. Acta urologica Japonica
  • [ISO-abbreviation] Hinyokika Kiyo
  • [Language] jpn
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Japan
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54. Kim JG, Sung WJ, Kim DH, Kim YH, Sohn SK, Lee KB: Malignant peripheral nerve sheath tumor in neurofibromatosis type I: unusual presentation of intraabdominal or intrathoracic mass. Korean J Intern Med; 2005 Mar;20(1):100-4
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  • [Title] Malignant peripheral nerve sheath tumor in neurofibromatosis type I: unusual presentation of intraabdominal or intrathoracic mass.
  • A malignant peripheral nerve sheath tumor (MPNST) is an extremely rare soft tissue tumor in the general population.
  • On the other hand, there is a higher incidence of MPNST in patients with neurofibromatosis type I (von Recklinghausen's disease).
  • This paper reports two patients, a 31 year-old woman with multiple neurofibromatosis presenting as an intraabdominal malignant peripheral nerve sheath tumor, and a 33 year-old woman with an intrathoracic malignant peripheral nerve sheath tumor.
  • [MeSH-major] Abdominal Neoplasms / diagnosis. Nerve Sheath Neoplasms / diagnosis. Neurofibromatosis 1 / complications. Thoracic Neoplasms / diagnosis
  • [MeSH-minor] Adult. Female. Humans

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  • (PMID = 15906964.001).
  • [ISSN] 1226-3303
  • [Journal-full-title] The Korean journal of internal medicine
  • [ISO-abbreviation] Korean J. Intern. Med.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Korea (South)
  • [Other-IDs] NLM/ PMC3891405
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55. Levi AD, Ross AL, Cuartas E, Qadir R, Temple HT: The surgical management of symptomatic peripheral nerve sheath tumors. Neurosurgery; 2010 Apr;66(4):833-40

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] The surgical management of symptomatic peripheral nerve sheath tumors.
  • OBJECTIVE: To determine the clinical presentation and morbidity of the surgical management of peripheral nerve sheath tumors (PNSTs).
  • RESULTS: There were a total of 140 cases, including 87 schwannomas, 34 neurofibromas, and 19 malignant peripheral nerve sheath tumors (MPNSTs).
  • Tumor size was the best predictor of adverse outcome, as all MPNST mortalities occurred in patients with a tumor size of more than 7 cm.
  • [MeSH-major] Nerve Sheath Neoplasms / surgery. Neurilemmoma / surgery. Neurofibroma / surgery. Neurosurgery / methods
  • [MeSH-minor] Adolescent. Adult. Aged. Aged, 80 and over. Female. Humans. Magnetic Resonance Imaging. Male. Middle Aged. Retrospective Studies. Treatment Outcome. Young Adult

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  • (PMID = 20190660.001).
  • [ISSN] 1524-4040
  • [Journal-full-title] Neurosurgery
  • [ISO-abbreviation] Neurosurgery
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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56. Chamoun RB, Whitehead WE, Dauser RC, Luerssen TG, Okcu MF, Adesina AM, Jea A: Primary disseminated intradural malignant peripheral nerve sheath tumor of the spine in a child: case report and review of the literature. Pediatr Neurosurg; 2009;45(3):230-6
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Primary disseminated intradural malignant peripheral nerve sheath tumor of the spine in a child: case report and review of the literature.
  • The authors report a rare case of primary disseminated intradural malignant peripheral nerve sheath tumor (MPNST) of the spine in a 5-year-old child without neurofibromatosis type I (NF-I).
  • MRI revealed a large intradural extramedullary tumor at C4-5 with dissemination to the thoracic spine, cauda equina and leptomeninges.
  • Following a 2-level cervical laminectomy, the tumor was biopsied and debulked.
  • Based on pathological and immunohistological findings, the tumor was diagnosed as an MPNST.
  • The reported clinical outcomes for adult and pediatric patients with intradural MPNST are very poor.
  • We report the first pediatric case--without or with NF-I--of disseminated intradural MPNST primarily localized proximal to the conus medullaris.
  • [MeSH-major] Magnetic Resonance Imaging. Nerve Sheath Neoplasms / pathology. Spinal Cord Neoplasms / pathology

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  • [Copyright] Copyright 2009 S. Karger AG, Basel.
  • (PMID = 19521138.001).
  • [ISSN] 1423-0305
  • [Journal-full-title] Pediatric neurosurgery
  • [ISO-abbreviation] Pediatr Neurosurg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] Switzerland
  • [Number-of-references] 38
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57. Moon SJ, Lee JK, Seo BR, Kim JH, Kim SH, Lee KH, Lee MC: An intraosseous malignant peripheral nerve sheath tumor of the cervical spine: a case report and review of the literature. Spine (Phila Pa 1976); 2008 Sep 1;33(19):E712-6
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  • [Title] An intraosseous malignant peripheral nerve sheath tumor of the cervical spine: a case report and review of the literature.
  • OBJECTIVES: To report a rare case of intraosseous malignant peripheral nerve sheath tumors (MPNST), and review the pertinent medical literature.
  • SUMMARY OF BACKGROUND DATA: The spinal MPNST that develops from spinal nerve roots and secondary bony erosion is well-known entity.
  • Complete excision of the tumor and posterior stabilization were performed through a posterior approach.
  • The tumor was noted to originate from the posterior element of C7.
  • RESULTS: The histopathology was diagnostic for a MPNST.
  • CONCLUSION: We report an intraosseous MPNST of the cervical spine.
  • MPNST should be added to the differential diagnosis of primary bone tumors causing spinal cord compression.
  • [MeSH-major] Cervical Vertebrae / pathology. Nerve Sheath Neoplasms / pathology. Spinal Neoplasms / pathology
  • [MeSH-minor] Adult. Antigens, CD / metabolism. Antigens, CD34 / metabolism. Biomarkers, Tumor / metabolism. Cell Adhesion Molecules / metabolism. Chemotherapy, Adjuvant. Combined Modality Therapy. Disease-Free Survival. Humans. Ki-67 Antigen / metabolism. Magnetic Resonance Imaging. Male. S100 Proteins / metabolism. Spinal Cord Compression / etiology. Spinal Cord Compression / pathology. Vimentin / metabolism

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  • (PMID = 18758353.001).
  • [ISSN] 1528-1159
  • [Journal-full-title] Spine
  • [ISO-abbreviation] Spine
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antigens, CD; 0 / Antigens, CD34; 0 / Biomarkers, Tumor; 0 / CD99 protein, human; 0 / Cell Adhesion Molecules; 0 / Ki-67 Antigen; 0 / S100 Proteins; 0 / Vimentin
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58. Shimada S, Tsuzuki T, Kuroda M, Nagasaka T, Hara K, Takahashi E, Hayakawa S, Ono K, Maeda N, Mori N, Illei PB: Nestin expression as a new marker in malignant peripheral nerve sheath tumors. Pathol Int; 2007 Feb;57(2):60-7

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Nestin expression as a new marker in malignant peripheral nerve sheath tumors.
  • Malignant peripheral nerve sheath tumor (MPNST) can be difficult to diagnose because it lacks specific immunohistochemical markers.
  • S-100, which is a useful marker of MPNST, has limited diagnostic utility.
  • The diagnostic utility of immunostains for nestin and three other neural markers (S-100, CD56 and protein gene product 9.5 (PGP 9.5)) were evaluated in 35 cases of MPNST and in other spindle cell tumors.
  • All MPNST cases were strongly positive for nestin and had cytoplasmic staining.
  • Nestin was negative in 10/10 leiomyomas, and weak nestin expression was seen in 10/10 schwannomas, 3/10 neurofibromas, 2/8 synovial sarcomas, 2/10 liposarcomas, 4/7 carcinosarcomas and 3/7 malignant fibrous histiocytomas.
  • Nestin is more sensitive for MPNST than other neural markers and immunostains for nestin in combination with other markers could be useful in the diagnosis of MPNST.
  • [MeSH-major] Biomarkers, Tumor / metabolism. Intermediate Filament Proteins / metabolism. Nerve Sheath Neoplasms / metabolism. Nerve Tissue Proteins / metabolism
  • [MeSH-minor] Adolescent. Adult. Aged. Cauda Equina / metabolism. Cauda Equina / pathology. Cell Transformation, Neoplastic / genetics. Cell Transformation, Neoplastic / metabolism. Cell Transformation, Neoplastic / pathology. Child. Female. Gene Expression Regulation, Neoplastic. Humans. Leiomyosarcoma / metabolism. Leiomyosarcoma / pathology. Male. Melanoma / metabolism. Melanoma / pathology. Middle Aged. Nestin. Neurilemmoma / metabolism. Neurilemmoma / pathology. Rhabdomyosarcoma / metabolism. Rhabdomyosarcoma / pathology. Sarcoma / metabolism. Sarcoma / pathology. Schwann Cells / metabolism. Schwann Cells / pathology

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  • (PMID = 17300669.001).
  • [ISSN] 1320-5463
  • [Journal-full-title] Pathology international
  • [ISO-abbreviation] Pathol. Int.
  • [Language] eng
  • [Publication-type] Evaluation Studies; Journal Article
  • [Publication-country] Australia
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Intermediate Filament Proteins; 0 / NES protein, human; 0 / Nerve Tissue Proteins; 0 / Nestin
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59. Spurlock G, Knight SJ, Thomas N, Kiehl TR, Guha A, Upadhyaya M: Molecular evolution of a neurofibroma to malignant peripheral nerve sheath tumor (MPNST) in an NF1 patient: correlation between histopathological, clinical and molecular findings. J Cancer Res Clin Oncol; 2010 Dec;136(12):1869-80
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Molecular evolution of a neurofibroma to malignant peripheral nerve sheath tumor (MPNST) in an NF1 patient: correlation between histopathological, clinical and molecular findings.
  • OBJECTIVE: Neurofibromatosis type 1 (NF1) patients have a 13% risk of developing a malignant peripheral nerve sheath tumor (MPNST).
  • Many MPNSTs are histopathologically complex, with regions exhibiting features of the original benign plexiform neurofibroma (PNF), of an atypical PNF, or of MPNST showing varying degrees of de-differentiation.
  • This study analyzed the genetic alterations associated with this pathological heterogeneity in order to identify the genetic processes involved in transformation from a benign to an aggressive malignant tumor.
  • METHODS: A histological and molecular analysis of a single MPNST tumor that was subdivided into three histopathologically distinct regions, a benign PNF (region 1), an atypical PNF (region 2), and a high-grade MPNST (region 3), was carried out.
  • Tumor DNA from each region was analyzed in conjunction with the patient's lymphocyte DNA.
  • The NF1-associated LOH analysis found that LOH increased in the three tumor areas, with 9, 42, and 97% LOH evident in regions 1, 2, and 3, respectively.
  • Additional genetic changes, including losses of TP53, RB1, CDKN2A, and of several oncogenes and cell-cycle genes, were found only in the malignant MPNST (region 3).
  • DISCUSSION: This is the first study that correlates the histological and molecular changes associated with MPNST development, confirming the significant cellular and genetic heterogeneity that poses both diagnostic and therapeutic challenges.
  • [MeSH-major] Nerve Sheath Neoplasms / genetics. Neurofibroma / genetics. Neurofibromatosis 1 / genetics. Neurofibromin 1 / genetics
  • [MeSH-minor] Adult. Base Sequence. Comparative Genomic Hybridization. DNA Mutational Analysis. Disease Progression. Germ-Line Mutation. Humans. Loss of Heterozygosity. Male. Molecular Sequence Data


60. Furniss D, Swan MC, Morritt DG, Lim J, Khanna T, Way BL, Athanasou NA, Giele H, Critchley P: A 10-year review of benign and malignant peripheral nerve sheath tumors in a single center: clinical and radiographic features can help to differentiate benign from malignant lesions. Plast Reconstr Surg; 2008 Feb;121(2):529-33
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] A 10-year review of benign and malignant peripheral nerve sheath tumors in a single center: clinical and radiographic features can help to differentiate benign from malignant lesions.
  • BACKGROUND: Malignant peripheral nerve sheath tumors are rare, and their aggressive nature mandates treatment in specialist centers.
  • In contrast, benign peripheral nerve sheath tumors are common and are treated by a variety of specialist surgeons, including plastic surgeons.
  • The authors aimed to detect features in the clinical presentation of peripheral nerve sheath tumors that point toward a diagnosis of malignant peripheral nerve sheath tumor and therefore prompt referral to a specialist center.
  • METHODS: All histologically diagnosed primary peripheral nerve sheath tumors from January of 1995 to December of 2004 were identified from histopathology records.
  • RESULTS: During the study period, 32 cases of malignant peripheral nerve sheath tumor in 30 patients were treated.
  • Factors in the clinical evaluation that significantly predicted the presence of malignant peripheral nerve sheath tumor included site, large size, depth in relation to the deep fascia, short duration of symptoms, and pain.
  • Interestingly, schwannomata were harder to distinguish from malignant peripheral nerve sheath tumors both clinically and radiologically.
  • CONCLUSIONS: The authors have reviewed their institutional experience of peripheral nerve sheath tumors over a 10-year period.
  • [MeSH-major] Magnetic Resonance Imaging / methods. Neurilemmoma / diagnosis. Neurofibroma / diagnosis. Peripheral Nervous System Neoplasms / diagnosis. Tomography, X-Ray Computed / methods
  • [MeSH-minor] Adolescent. Adult. Aged. Aged, 80 and over. Child. Child, Preschool. Diagnosis, Differential. Female. Follow-Up Studies. Humans. Male. Middle Aged. Retrospective Studies. Sensitivity and Specificity. Time Factors

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  • (PMID = 18300972.001).
  • [ISSN] 1529-4242
  • [Journal-full-title] Plastic and reconstructive surgery
  • [ISO-abbreviation] Plast. Reconstr. Surg.
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article; Multicenter Study
  • [Publication-country] United States
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61. Okada K, Hasegawa T, Tajino T, Hotta T, Yanagisawa M, Osanai T, Nishida J, Seki K, Itoi E: Clinical relevance of pathological grades of malignant peripheral nerve sheath tumor: a multi-institution TMTS study of 56 cases in Northern Japan. Ann Surg Oncol; 2007 Feb;14(2):597-604
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  • [Title] Clinical relevance of pathological grades of malignant peripheral nerve sheath tumor: a multi-institution TMTS study of 56 cases in Northern Japan.
  • BACKGROUND: Malignant peripheral nerve sheath tumor (MPNST) is a relatively rare soft tissue tumor, and its clinical relevance of pathological grades remains obscure.
  • METHODS: Fifty-six cases of MPNST identified from the files of seven oncology centers of the Tohoku Musculoskeletal Tumor Society (TMTS) and National Cancer Center were analyzed for histologic grades, demographics, treatments, and prognostic factors.
  • Twenty-one (39.6%) of 53 patients who underwent tumor excision developed local recurrences.
  • Multivariate analysis revealed a large tumor and metastasis at presentation to be independent prognostic factors.
  • CONCLUSIONS: The current study involving 56 patients with MPNST showed the aggressive clinical behavior of the tumor.
  • In the treatment for a large and high-grade MPNST, an alternative strategy should be further considered.
  • [MeSH-major] Nerve Sheath Neoplasms / pathology. Soft Tissue Neoplasms / pathology
  • [MeSH-minor] Adolescent. Adult. Aged. Aged, 80 and over. Demography. Female. Humans. Japan. Male. Middle Aged. Prognosis. Societies, Medical. Survival Analysis

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  • (PMID = 17103076.001).
  • [ISSN] 1068-9265
  • [Journal-full-title] Annals of surgical oncology
  • [ISO-abbreviation] Ann. Surg. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article; Multicenter Study
  • [Publication-country] United States
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62. Sabah M, Cummins R, Leader M, Kay E: Loss of p16 (INK4A) expression is associated with allelic imbalance/loss of heterozygosity of chromosome 9p21 in microdissected malignant peripheral nerve sheath tumors. Appl Immunohistochem Mol Morphol; 2006 Mar;14(1):97-102
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  • [Title] Loss of p16 (INK4A) expression is associated with allelic imbalance/loss of heterozygosity of chromosome 9p21 in microdissected malignant peripheral nerve sheath tumors.
  • The p16 is a tumor suppressor gene on the short arm of chromosome 9p21.
  • This study was designed to assess the frequency of genetic loss of 9p21 and to determine the role of p16 the pathogenesis of sporadic and neurofibromatosis 1 (NF1)-associated malignant peripheral nerve sheath tumors (MPNSTs).

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  • (PMID = 16540739.001).
  • [ISSN] 1541-2016
  • [Journal-full-title] Applied immunohistochemistry & molecular morphology : AIMM
  • [ISO-abbreviation] Appl. Immunohistochem. Mol. Morphol.
  • [Language] ENG
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Cyclin-Dependent Kinase Inhibitor p16
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63. 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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64. Karabatsou K, Kiehl TR, Wilson DM, Hendler A, Guha A: Potential role of 18fluorodeoxyglucose-positron emission tomography/computed tomography in differentiating benign neurofibroma from malignant peripheral nerve sheath tumor associated with neurofibromatosis 1. Neurosurgery; 2009 Oct;65(4 Suppl):A160-70
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  • [Title] Potential role of 18fluorodeoxyglucose-positron emission tomography/computed tomography in differentiating benign neurofibroma from malignant peripheral nerve sheath tumor associated with neurofibromatosis 1.
  • OBJECTIVE: Benign plexiform neurofibromas (PNfib), especially those occurring in patients with neurofibromatosis type 1, are at a significant risk of progressing to a malignant peripheral nerve sheath tumor (MPNST).
  • Early diagnosis, followed by radical surgery and adjuvant radiation to maintain local tumor control, is of critical importance to prevent metastasis and subsequent mortality from MPNSTs.
  • However, early diagnosis is hampered by the sensitivity of current imaging modalities such as computed tomography (CT) or magnetic resonance imaging to reliably detect this malignant transformation, which can occur heterogeneously in a PNfib to a MPNST.
  • METHODS: In this prospective study, 9 neurofibromatosis type 1-associated PNfibs suspected to have undergone transformation to an MPNST were preoperatively evaluated by 18FDG-PET/CT and magnetic resonance imaging.
  • A detailed histological evaluation correlated the average and regional standard uptake value (SUV) from the 18FDG-PET/CT to grade of malignancy of the suspected MPNST.
  • Stratification of the maximal SUV to low (<4.0), intermediate (4.0-7.0), or high (>7.0) correlated to the proliferative index (Ki-67) and grade of MPNST.
  • A maximal SUV of more than 7.0 was closely correlated to a focus of malignant transformation.
  • [MeSH-major] Nerve Sheath Neoplasms / radionuclide imaging. Neurofibroma / radionuclide imaging. Neurofibromatosis 1 / radionuclide imaging. Peripheral Nerves / radionuclide imaging. Peripheral Nervous System Neoplasms / radionuclide imaging. Positron-Emission Tomography / methods
  • [MeSH-minor] Adult. Diagnosis, Differential. Female. Fluorodeoxyglucose F18. Humans. Male. Middle Aged. Predictive Value of Tests. Prospective Studies. Tomography, X-Ray Computed. Young Adult


65. Tawbi H, Thomas D, Lucas DR, Biermann JS, Schuetze SM, Hart AL, Chugh R, Baker LH: Epidermal growth factor receptor expression and mutational analysis in synovial sarcomas and malignant peripheral nerve sheath tumors. Oncologist; 2008 Apr;13(4):459-66
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  • [Title] Epidermal growth factor receptor expression and mutational analysis in synovial sarcomas and malignant peripheral nerve sheath tumors.
  • BACKGROUND: Synovial sarcomas (SnSrcs) and malignant peripheral nerve sheath tumors (MPNSTs) are rare mesenchymal tumors of adolescence and young adulthood.
  • METHODS: Tissue microarrays containing 48 cases of SnSrc and 32 cases of MPNST were stained for EGFR, EGFRvIII, and activated EGFR (pY1068-EGFR).
  • Tumor DNA was extracted from fresh and formalin-fixed, paraffin-embedded tissue blocks and sequenced for exons 17-21 of EGFR and exon 2 of K-ras and b-raf.
  • No K-ras or b-raf mutations were observed in either tumor type.
  • CONCLUSIONS: Expression of EGFR in SnSrcs and MPNSTs with an intact EGFR/mitogen-activated protein kinase pathway has been hypothesized to contribute to the malignant potential of these tumors.
  • [MeSH-major] Mutation. Nerve Sheath Neoplasms / diagnosis. Protein Kinase Inhibitors / therapeutic use. Receptor, Epidermal Growth Factor / genetics. Sarcoma, Synovial / diagnosis
  • [MeSH-minor] Adolescent. Adult. Aged. Child. DNA Mutational Analysis. Female. Gene Expression Regulation, Neoplastic. Genes, ras / genetics. Humans. Immunohistochemistry. Male. Middle Aged. Proto-Oncogene Proteins B-raf / genetics. Translocation, Genetic


66. 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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67. 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


68. Brekke HR, Kolberg M, Skotheim RI, Hall KS, Bjerkehagen B, Risberg B, Domanski HA, Mandahl N, Liestøl K, Smeland S, Danielsen HE, Mertens F, Lothe RA: Identification of p53 as a strong predictor of survival for patients with malignant peripheral nerve sheath tumors. Neuro Oncol; 2009 Oct;11(5):514-28
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  • [Title] Identification of p53 as a strong predictor of survival for patients with malignant peripheral nerve sheath tumors.
  • The purpose of this study was to identify new prognostic biomarkers with clinical impact in malignant peripheral nerve sheath tumor (MPNST), a highly aggressive malignancy for which no consensus therapy exists besides surgery.
  • We have used tissue microarrays (TMAs) to assess in situ expression of 14 cell-cycle-regulating proteins in 64 well-characterized MPNST patients: 36 sporadic and 28 with neurofibromatosis type 1 (NF1).
  • We developed a new software application for evaluation and logistics of the TMA images and performed a literature survey of cell cycle proteins in MPNST.
  • For the total series of MPNSTs, p53 was shown to be an independent predictor of survival, and patients without remission, with tumor size larger than 8 cm, and with positive p53 expression had a 60 times greater risk of dying within the first 5 years compared with the remaining patients (p = 0.000002).
  • This is the most comprehensive study of in situ protein expression in MPNST so far, and expressed p53 was found to be a strong surrogate marker for outcome.
  • Patients in complete remission with a primary p53-positive MPNST diagnosis may be considered in a high-risk subgroup and candidates for adjuvant treatment.
  • [MeSH-major] Biomarkers, Tumor / analysis. Nerve Sheath Neoplasms / mortality. Nerve Sheath Neoplasms / pathology. Tumor Suppressor Protein p53 / biosynthesis
  • [MeSH-minor] Adult. Blotting, Western. Comparative Genomic Hybridization. Disease-Free Survival. Female. Gene Expression. Humans. Image Processing, Computer-Assisted / methods. Immunohistochemistry. Kaplan-Meier Estimate. Male. Middle Aged. Software. Tissue Array Analysis

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  • (PMID = 19182148.001).
  • [ISSN] 1522-8517
  • [Journal-full-title] Neuro-oncology
  • [ISO-abbreviation] Neuro-oncology
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Tumor Suppressor Protein p53
  • [Other-IDs] NLM/ PMC2765341
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69. Martinez Devesa P, Mitchell TE, Scott I, Moffat DA: Malignant peripheral nerve sheath tumors of the head and neck: two cases and a review of the literature. Ear Nose Throat J; 2006 Jun;85(6):392-6

  • [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: two cases and a review of the literature.
  • Malignant peripheral nerve sheath tumors are uncommon lesions that occasionally affect the head and neck.
  • One tumor involved the parotid gland and resulted in erosion of the temporal bone, and the other affected the lower lip.
  • A rapid diagnosis has significant implications for management because of the tumor's potential for aggressive behavior and its high rate of recurrence.
  • [MeSH-major] Lip Neoplasms / diagnosis. Nerve Sheath Neoplasms / diagnosis. Parotid Neoplasms / diagnosis
  • [MeSH-minor] Adult. Angiography. Hearing Loss, Sensorineural / etiology. Humans. Immunohistochemistry. Magnetic Resonance Imaging. Male. Middle Aged. Parotid Gland / surgery. Tomography, X-Ray Computed

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  • (PMID = 16866118.001).
  • [ISSN] 0145-5613
  • [Journal-full-title] Ear, nose, & throat journal
  • [ISO-abbreviation] Ear Nose Throat J
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 23
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70. 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
Genetic Alliance. consumer health - Malignant peripheral nerve sheath tumor.

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [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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71. Kretschmer T, Antoniadis G, Heinen C, Börm W, Scheller C, Richter HP, Koenig RW: Nerve sheath tumor surgery: case-guided discussion of ambiguous findings, appropriateness of removal, repeated surgery, and nerve repairs. Neurosurg Focus; 2007;22(6):E19
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  • [Title] Nerve sheath tumor surgery: case-guided discussion of ambiguous findings, appropriateness of removal, repeated surgery, and nerve repairs.
  • In this article the authors attempt to raise awareness of the pitfalls and controversial issues in nerve tumor surgery.
  • In a case-guided format, examples of ambiguous findings, inappropriate tumor removal, repeated surgery, and nerve repairs are provided.
  • The authors also discuss the need to establish a correct diagnosis preoperatively and to avoid the erroneous identification of malignant peripheral nerve sheath tumors (MPNSTs).
  • They emphasize that not all of the principles of soft tissue sarcoma treatment protocols are applicable to MPNST.
  • A situation of repeated surgery for supposedly malignant tumor is described, and an outline of the indications for, and an approach to, repair after lesion removal is given.
  • [MeSH-major] Nerve Sheath Neoplasms / diagnosis. Nerve Sheath Neoplasms / surgery. Neurosurgical Procedures / methods. Peripheral Nerve Injuries. Peripheral Nerves / surgery. Reconstructive Surgical Procedures / methods
  • [MeSH-minor] Adolescent. Adult. Aged. Female. Humans. Male. Middle Aged. Reoperation / adverse effects. Reoperation / methods

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  • (PMID = 17613210.001).
  • [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
  • [Number-of-references] 48
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72. 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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73. 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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  • [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


74. Scheithauer BW, Erdogan S, Rodriguez FJ, Burger PC, Woodruff JM, Kros JM, Gokden M, Spinner RJ: Malignant peripheral nerve sheath tumors of cranial nerves and intracranial contents: a clinicopathologic study of 17 cases. Am J Surg Pathol; 2009 Mar;33(3):325-38
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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 cranial nerves and intracranial contents: a clinicopathologic study of 17 cases.
  • Malignant peripheral nerve sheath tumors (MPNSTs) arising from cranial nerves or their branches are very uncommon.
  • In addition, 1 tumor involved the optic chiasm (n=1).
  • Only 1 tumor arose in brain parenchyma of (frontal lobe).
  • One patient with a vestibular tumor and presumed NF2 had previously undergone resection of a contralateral vestibular cellular schwannoma.
  • One posterior fossa tumor was a malignant melanotic schwannoma.
  • Four patients had postirradiation malignant peripheral nerve sheath tumors, 2 having been treated for optic chiasm glioma, both being NF1 affected.
  • Identifiable precursor lesions included schwannoma (n=4), plexiform neurofibroma (n=2), and solitary intraneural neurofibroma (n=2).
  • Malignant cranial nerve sheath tumors are rare and are associated with the same poor prognosis as those of spinal nerves at other sites.
  • [MeSH-major] Brain Neoplasms / pathology. Cranial Nerve Neoplasms / pathology. Nerve Sheath Neoplasms / pathology
  • [MeSH-minor] Adult. Aged. Child, Preschool. Female. Humans. Immunohistochemistry. Male. Middle Aged

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  • (PMID = 19065105.001).
  • [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
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75. Agesen TH, Flørenes VA, Molenaar WM, Lind GE, Berner JM, Plaat BE, Komdeur R, Myklebost O, van den Berg E, Lothe RA: Expression patterns of cell cycle components in sporadic and neurofibromatosis type 1-related malignant peripheral nerve sheath tumors. J Neuropathol Exp Neurol; 2005 Jan;64(1):74-81
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  • [Title] Expression patterns of cell cycle components in sporadic and neurofibromatosis type 1-related malignant peripheral nerve sheath tumors.
  • The molecular biology underlying the development of highly malignant peripheral nerve sheath tumors (MPNSTs) remains mostly unknown.
  • All tumor samples expressed normal sized RB1, cyclin D3, CDK2, CDK4, p21(CIP1), and p27(KlP1) proteins, and only a single tumor showed an aberrant protein band for one of these proteins, p21(CIP1).
  • Cyclin D1 was absent in four tumors; all except one tumor showed expression of TP53 protein, and three of nine MPNSTs had expression of normal-sized MDM2.
  • The data imply that the complete absence of p16(INK4A) is sufficient for activation of the cell cycle in most MPNSTs; thus, it is not necessary for tumor proliferation to further stimulate the cycle through alteration of other central components.
  • [MeSH-major] Cell Cycle. Cell Cycle Proteins / biosynthesis. Gene Expression Regulation, Neoplastic. Nerve Sheath Neoplasms / genetics. Nerve Sheath Neoplasms / metabolism. Neurofibromatosis 1 / genetics. Neurofibromatosis 1 / metabolism
  • [MeSH-minor] Adult. Aged. Blotting, Western. Cyclin-Dependent Kinase Inhibitor p16 / biosynthesis. Cyclin-Dependent Kinase Inhibitor p16 / genetics. Female. Humans. Male. Middle Aged. Retinoblastoma Protein / biosynthesis. Retinoblastoma Protein / genetics. Tumor Suppressor Protein p53 / biosynthesis. Tumor Suppressor Protein p53 / genetics


76. 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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  • [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


77. Widemann BC: Current status of sporadic and neurofibromatosis type 1-associated malignant peripheral nerve sheath tumors. Curr Oncol Rep; 2009 Jul;11(4):322-8
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  • [Title] Current status of sporadic and neurofibromatosis type 1-associated malignant peripheral nerve sheath tumors.
  • Malignant peripheral nerve sheath tumors (MPNSTs) are highly aggressive soft tissue sarcomas that rarely occur in the general population but have a lifetime incidence of 8% to 13% in those with neurofibromatosis type 1 (NF1).
  • The response rate of MPNSTs to standard chemotherapeutic agents used to treat pediatric and adult soft tissue sarcomas is unknown and is currently undergoing evaluation in a multi-institutional clinical trial.
  • This knowledge, coupled with the availability of preclinical MPNST models, likely will accelerate the development of effective treatments for this malignancy.
  • [MeSH-major] Nerve Sheath Neoplasms / genetics. Nerve Sheath Neoplasms / therapy. Neurofibromatosis 1 / complications
  • [MeSH-minor] Clinical Trials as Topic. DNA-Binding Proteins / genetics. Genetic Predisposition to Disease. Genome-Wide Association Study. Nuclear Proteins / genetics. Receptor, Epidermal Growth Factor / genetics. Tumor Suppressor Protein p53 / genetics. Tumor Suppressor Proteins / genetics. Vascular Endothelial Growth Factor A / genetics

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  • (PMID = 19508838.001).
  • [ISSN] 1534-6269
  • [Journal-full-title] Current oncology reports
  • [ISO-abbreviation] Curr Oncol Rep
  • [Language] eng
  • [Grant] United States / Intramural NIH HHS / /
  • [Publication-type] Journal Article; Research Support, N.I.H., Intramural; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / DNA-Binding Proteins; 0 / Nuclear Proteins; 0 / TP53 protein, human; 0 / Tumor Suppressor Protein p53; 0 / Tumor Suppressor Proteins; 0 / Vascular Endothelial Growth Factor A; 0 / tumor suppressor protein p73; EC 2.7.10.1 / Receptor, Epidermal Growth Factor
  • [Number-of-references] 80
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78. 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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  • [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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79. 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

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  • [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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80. Kinebuchi Y, Noguchi W, Igawa Y, Nishizawa O: Recurrent retroperitoneal malignant nerve sheath tumor associated with neurofibromatosis type 1 responding to carboplatin and etoposide combined chemotherapy. Int J Clin Oncol; 2005 Oct;10(5):353-6
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  • [Title] Recurrent retroperitoneal malignant nerve sheath tumor associated with neurofibromatosis type 1 responding to carboplatin and etoposide combined chemotherapy.
  • The tumor was resected, and the pathological diagnosis was malignant peripheral nerve sheath tumor (MPNST).
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Nerve Sheath Neoplasms / drug therapy. Neurofibromatosis 1 / complications. Peripheral Nervous System Neoplasms / drug therapy. Retroperitoneal Neoplasms / drug therapy
  • [MeSH-minor] Adult. Carboplatin / administration & dosage. Etoposide / administration & dosage. Humans. Lung Neoplasms / secondary. Male

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  • (PMID = 16247664.001).
  • [ISSN] 1341-9625
  • [Journal-full-title] International journal of clinical oncology
  • [ISO-abbreviation] Int. J. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Japan
  • [Chemical-registry-number] 6PLQ3CP4P3 / Etoposide; BG3F62OND5 / Carboplatin
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81. Mantripragada KK, Díaz de Ståhl T, Patridge C, Menzel U, Andersson R, Chuzhanova N, Kluwe L, Guha A, Mautner V, Dumanski JP, Upadhyaya M: Genome-wide high-resolution analysis of DNA copy number alterations in NF1-associated malignant peripheral nerve sheath tumors using 32K BAC array. Genes Chromosomes Cancer; 2009 Oct;48(10):897-907
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  • [Title] Genome-wide high-resolution analysis of DNA copy number alterations in NF1-associated malignant peripheral nerve sheath tumors using 32K BAC array.
  • Neurofibromatosis Type I (NF1) is an autosomal dominant disorder characterized by the development of both benign and malignant tumors.
  • The lifetime risk for developing a malignant peripheral nerve sheath tumor (MPNST) in NF1 patients is approximately 10% with poor survival rates.
  • To date, the molecular basis of MPNST development remains unclear.
  • Here, we report the first genome-wide and high-resolution analysis of DNA copy number alterations in MPNST using the 32K bacterial artificial chromosome microarray on a series of 24 MPNSTs and three neurofibroma samples.
  • The profiles of malignant samples, however, revealed specific loss of chromosomal regions including 1p35-33, 1p21, 9p21.3, 10q25, 11q22-23, 17q11, and 20p12.2 as well as gain of 1q25, 3p26, 3q13, 5p12, 5q11.2-q14, 5q21-23, 5q31-33, 6p23-p21, 6p12, 6q15, 6q23-q24, 7p22, 7p14-p13, 7q21, 7q36, 8q22-q24, 14q22, and 17q21-q25.
  • Copy number gains were more frequent than deletions in the MPNST samples (62% vs. 38%).
  • [MeSH-major] Comparative Genomic Hybridization / methods. Genes, Neurofibromatosis 1. Nerve Sheath Neoplasms / genetics. Neurofibromatosis 1 / genetics. Oligonucleotide Array Sequence Analysis / methods. Skin Neoplasms / genetics
  • [MeSH-minor] Adult. Chromosome Aberrations. Chromosomes, Artificial, Bacterial. Female. Gene Dosage. Genome, Human. Humans. Male. Middle Aged

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  • [Copyright] (c) 2009 Wiley-Liss, Inc.
  • (PMID = 19603524.001).
  • [ISSN] 1098-2264
  • [Journal-full-title] Genes, chromosomes & cancer
  • [ISO-abbreviation] Genes Chromosomes Cancer
  • [Language] eng
  • [Grant] United Kingdom / Cancer Research UK / /
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
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82. Rekhi B, Ingle A, Kumar R, DeSouza MA, Dikshit R, Jambhekar NA: Malignant peripheral nerve sheath tumors: clinicopathological profile of 63 cases diagnosed at a tertiary cancer referral center in Mumbai, India. Indian J Pathol Microbiol; 2010 Oct-Dec;53(4):611-8
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  • [Title] Malignant peripheral nerve sheath tumors: clinicopathological profile of 63 cases diagnosed at a tertiary cancer referral center in Mumbai, India.
  • BACKGROUND: A malignant peripheral nerve sheath tumor (MPNST) is a rare sarcoma, characterized by an aggressive course and forms a diagnostic challenge, in view of its varied histomorphology.
  • Average tumor (T) size was 9.9 cm, with 72.9% cases having T size > 5 cm.
  • CONCLUSIONS: A MPNST has multifaceted histomorphology.
  • [MeSH-major] Nerve Sheath Neoplasms / pathology
  • [MeSH-minor] Adolescent. Adult. Age Distribution. Aged. Child. Female. Histocytochemistry. Humans. India. Male. Microscopy. Middle Aged. Recurrence. Severity of Illness Index. Survival Analysis. Young Adult

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  • (PMID = 21045379.001).
  • [ISSN] 0974-5130
  • [Journal-full-title] Indian journal of pathology & microbiology
  • [ISO-abbreviation] Indian J Pathol Microbiol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] India
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83. Matsumine A, Kusuzaki K, Nakamura T, Nakazora S, Niimi R, Matsubara T, Uchida K, Murata T, Kudawara I, Ueda T, Naka N, Araki N, Maeda M, Uchida A: Differentiation between neurofibromas and malignant peripheral nerve sheath tumors in neurofibromatosis 1 evaluated by MRI. J Cancer Res Clin Oncol; 2009 Jul;135(7):891-900
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  • [Title] Differentiation between neurofibromas and malignant peripheral nerve sheath tumors in neurofibromatosis 1 evaluated by MRI.
  • PURPOSE: The imaging discrimination between neurofibroma (NF) and malignant peripheral nerve sheath tumor (MPNST) is clinically very important.
  • The purpose of this study is to define the criteria for the differential diagnosis between NF and MPNST on MRI in neurofibromatosis 1 (NF1).
  • RESULTS: The MRI findings characteristic of MPNST (p < 0.05) were an irregular tumor shape (15/19 in MPNST vs. 5/18 in NF), unclear margin (13/19 in MPNST vs. 6/18 in NF), intra-tumoral lobulation (12/19 in MPNST vs. 3/18 in NF), presence of high signal-intensity area on T1-weighted images (T1WI) (12/19 in MPNST vs. 1/18 in NF), no target sign (0/19 in MPNST vs. 12/18 in NF), inhomogeneous enhancement on contract-enhanced T1WI (17/18 in MPNST vs. 9/16 in NF) and a lower rate of enhanced area (54% in MPNST vs. 87% in NF) were critical indicators to differentiate MPNST from NF.
  • A multivariate analysis showed that intra-tumoral lobulation and the presence of a high signal-intensity area on T1WI were considered to be diagnostic indicators of MPNST.
  • CONCLUSION: MRI shows features which were helpful for differentiating MPNST from NF.
  • [MeSH-major] Magnetic Resonance Imaging / methods. Nerve Sheath Neoplasms / diagnosis. Neurofibroma / diagnosis. Neurofibromatosis 1 / diagnosis. Peripheral Nervous System Neoplasms / diagnosis
  • [MeSH-minor] Adolescent. Adult. Aged. Aged, 80 and over. Diagnosis, Differential. Female. Gadolinium. Humans. Male. Middle Aged. Radioisotopes. Tomography, Emission-Computed / methods. Young Adult

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  • (PMID = 19101731.001).
  • [ISSN] 1432-1335
  • [Journal-full-title] Journal of cancer research and clinical oncology
  • [ISO-abbreviation] J. Cancer Res. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Comparative Study; Evaluation Studies; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Radioisotopes; AU0V1LM3JT / Gadolinium
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84. Murovic JA, Gibbs IC, Chang SD, Mobley BC, Park J, Adler JR Jr: Foraminal nerve sheath tumors: intermediate follow-up after cyberknife radiosurgery. Neurosurgery; 2009 Feb;64(2 Suppl):A33-43
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  • [Title] Foraminal nerve sheath tumors: intermediate follow-up after cyberknife radiosurgery.
  • OBJECTIVE: To conduct a retrospective review of outcomes in 15 patients with 18 foraminal tumors, including 17 benign peripheral nerve sheath tumors and 1 malignant peripheral nerve sheath tumor, who underwent CyberKnife (Accuray, Inc., Sunnyvale, CA) radiosurgery at Stanford University Medical Center from 1999 to 2006.
  • METHODS: Symptoms and findings, neurofibromatosis (NF) association, previous radiation, imaging, dosimetry, tumor volume, central necrosis, and the relation of these factors to outcomes were evaluated.
  • Three patients had NF1-associated neurofibromas, 1 patient with NF2 had a schwannoma, and 1 patient had a schwannomatosis-related lesion.
  • Two likely radiation-induced lesions, a neurofibroma and a malignant peripheral nerve sheath tumor, were observed.
  • Prescribed doses ranging from 16 to 24 Gy, delivered in 1 to 3 fractions of 6 to 20 Gy, resulted in maximum tumor doses ranging from 20.9 to 30 Gy.
  • Tumor volumes decreased in 12 (67%) of 18 tumors and increased in 3 tumors.
  • Tumor volumes decreased in 8 of 10 schwannomas and 3 of 7 neurofibromas.
  • CONCLUSION: CyberKnife radiosurgery resulted in pain relief and functional preservation in selected foraminal peripheral nerve sheath tumors and a malignant peripheral nerve sheath tumor.
  • [MeSH-major] Nerve Sheath Neoplasms / surgery. Radiosurgery. Spinal Neoplasms / surgery
  • [MeSH-minor] Adult. Female. Follow-Up Studies. Humans. Magnetic Resonance Imaging. Male. Middle Aged. Neurofibromatoses / complications. Retrospective Studies. Treatment Outcome

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  • (PMID = 19165072.001).
  • [ISSN] 1524-4040
  • [Journal-full-title] Neurosurgery
  • [ISO-abbreviation] Neurosurgery
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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85. 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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  • [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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86. 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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87. 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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88. Friedrich RE, Kluwe L, Fünsterer C, Mautner VF: Malignant peripheral nerve sheath tumors (MPNST) in neurofibromatosis type 1 (NF1): diagnostic findings on magnetic resonance images and mutation analysis of the NF1 gene. Anticancer Res; 2005 May-Jun;25(3A):1699-702
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  • [Title] Malignant peripheral nerve sheath tumors (MPNST) in neurofibromatosis type 1 (NF1): diagnostic findings on magnetic resonance images and mutation analysis of the NF1 gene.
  • About 10% of patients with NF1 develop malignant peripheral nerve sheath tumors (MPNST), usually arising from PNF, and this is the major cause of poor prognosis.
  • Our objective was to establish magnetic resonance imaging (MRI) criteria for MPNST, and to test their usefulness in detecting early malignant changes in PNF and to correlate the findings with the mutations of the NF1 gene.
  • The study was approved by the Institutional Review Board and all patients gave informed consent to analyze clinical records and tumor material for scientific purposes.
  • RESULTS: MRI was performed on 50 patients with NF1 and nerve sheath tumors, of whom 7 had atypical pain, tumor growth or neurological deficits indicative of malignancy; the other 43 were asymptomatic.
  • All 3 asymptomatic patients with inhomogeneous lesions were shown to have MPNST.
  • Analysis of mutations of the NF1 gene of the 10 MPNST patients revealed a variety of mutations.
  • Concerning the correlation of genetic findings and MPNST in NF1, the sample size of this study group was too small to define genotype-phenotype relations.
  • CONCLUSION: This study provides evidence for certain radiographic findings on MRI in PNF of NF1 patients that have to be considered as signs of malignancy, in particular indicating an MPNST.
  • [MeSH-major] Genes, Neurofibromatosis 1. Mutation. Nerve Sheath Neoplasms / diagnosis
  • [MeSH-minor] Adolescent. Adult. Humans. Magnetic Resonance Imaging. Middle Aged

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  • (PMID = 16033085.001).
  • [ISSN] 0250-7005
  • [Journal-full-title] Anticancer research
  • [ISO-abbreviation] Anticancer Res.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Greece
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89. Hemalatha AL, Karthikeyan TM, Bharatnur SS, Kumar AS: Malignant peripheral nerve sheath tumor in oral cavity--a rare site. Indian J Pathol Microbiol; 2006 Jul;49(3):397-9
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  • [Title] Malignant peripheral nerve sheath tumor in oral cavity--a rare site.
  • MPNST occurring in oral cavity, which is a rare site for the tumour, in a 35 year old female patient with history of swelling underneath the tongue present since one year diagnosed clinically as ranula is presented here.
  • Histopathological examination of the excised mass showed features of spindle cell sarcoma following which a provisional diagnosis of MPNST was offered.
  • [MeSH-major] Mouth / pathology. Mouth Neoplasms / diagnosis. Nerve Sheath Neoplasms / diagnosis
  • [MeSH-minor] Adult. Diagnosis, Differential. Female. Humans

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  • (PMID = 17001897.001).
  • [ISSN] 0377-4929
  • [Journal-full-title] Indian journal of pathology & microbiology
  • [ISO-abbreviation] Indian J Pathol Microbiol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] India
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90. Brekke HR, Ribeiro FR, Kolberg M, Agesen TH, Lind GE, Eknaes M, Hall KS, Bjerkehagen B, van den Berg E, Teixeira MR, Mandahl N, Smeland S, Mertens F, Skotheim RI, Lothe RA: Genomic changes in chromosomes 10, 16, and X in malignant peripheral nerve sheath tumors identify a high-risk patient group. J Clin Oncol; 2010 Mar 20;28(9):1573-82
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Genomic changes in chromosomes 10, 16, and X in malignant peripheral nerve sheath tumors identify a high-risk patient group.
  • PURPOSE: The purpose of this study was to identify genetic aberrations contributing to clinical aggressiveness of malignant peripheral nerve sheath tumors (MPNSTs).
  • RESULTS: Forty-four (92%) MPNSTs displayed DNA copy number changes (median, 18 changes per tumor; range, 2 to 35 changes).
  • Multivariate analyses including NF1 status, tumor location, size, grade, sex, complete remission, and initial metastatic status showed that the genomic high-risk group was the most significant predictor of poor survival.
  • [MeSH-major] Chromosomes, Human, Pair 10 / genetics. Chromosomes, Human, Pair 16 / genetics. Chromosomes, Human, X / genetics. DNA Copy Number Variations. Nerve Sheath Neoplasms / genetics. Nervous System Neoplasms / genetics
  • [MeSH-minor] Adolescent. Adult. Aged. Female. Gene Expression. Genome. Humans. Male. Middle Aged. Neurofibromatosis 1 / genetics. Prognosis. Risk Factors. Survival Analysis. Young Adult

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  • (PMID = 20159821.001).
  • [ISSN] 1527-7755
  • [Journal-full-title] Journal of clinical oncology : official journal of the American Society of Clinical Oncology
  • [ISO-abbreviation] J. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
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91. Ghosh A, Talwar OP, Pradhan SV: Tumour and tumour-like conditions of peripheral nerve origin: ten years' experience. Kathmandu Univ Med J (KUMJ); 2010 Jan-Mar;8(29):97-101
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Tumour and tumour-like conditions of peripheral nerve origin: ten years' experience.
  • BACKGROUND: There are four major lesions which may arise in the peripheral nerve, namely neuroma, schwannoma, neurofibroma and malignant peripheral nerve sheath tumor.
  • OBJECTIVE: In the present study we look into the spectrum of peripheral neural tumors including their age distribution site distribution and histopathology.
  • All histopathologically diagnosed cases of primary lesions of peripheral nerve during the period Jan 2000 to Nov 2009 were reviewed and the data were analysed.
  • RESULT: A total of 114 cases of peripheral neural lesions were reported in the same period.
  • Total number of nonmalignant cases was 106 (93%) while malignant cases were 8 (7%).
  • Among the nonmalignant cases neurofibroma was the commonest (51 cases, 45% of all) closely followed by schwannoma (39 cases, 34% of all).
  • Among the schwannoma cases 4 were diagnosed as ancient schwannoma with presence of bizarre cells with hyperchromatic nuclei.
  • The commonest site involved for both schwannoma and neurofibroma was scalp-face-neck followed by back.
  • The age range for schwannoma was 16 to 75 years whereas the same for the neurofibroma cases was 2 to 82 years.
  • MPNST cases were seen in the age range of 40 to 72 with 3 cases in upper extremity, 3 in lower extremity and 1 each in lip and cheek.
  • CONCLUSION: The majority of the tumor are benign and the commonest benign tumor was neurofibroma of sporadic type, closely followed by schwannoma.
  • [MeSH-major] Nerve Sheath Neoplasms / epidemiology. Neurilemmoma / epidemiology. Neurofibroma / epidemiology. Neuroma / epidemiology. Peripheral Nervous System Neoplasms / epidemiology
  • [MeSH-minor] Adolescent. Adult. Age Distribution. Aged. Aged, 80 and over. Child. Child, Preschool. Female. Humans. Male. Middle Aged. Retrospective Studies. Young Adult

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  • (PMID = 21209517.001).
  • [ISSN] 1812-2078
  • [Journal-full-title] Kathmandu University medical journal (KUMJ)
  • [ISO-abbreviation] Kathmandu Univ Med J (KUMJ)
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Nepal
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92. Terzic A, Bode B, Gratz KW, Stoeckli SJ: Prognostic factors for the malignant triton tumor of the head and neck. Head Neck; 2009 May;31(5):679-88
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Prognostic factors for the malignant triton tumor of the head and neck.
  • BACKGROUND: Malignant triton tumors are rare neoplasias consisting of a malignant peripheral nerve sheath tumor with additional rhabdomyoblastic differentiation.
  • METHODS: From 1993 to 2005, 7 patients with a malignant triton tumor of the head and neck were treated at our institution.
  • CONCLUSION: Location of the primary tumor is a key factor for prognosis.
  • [MeSH-major] Head and Neck Neoplasms / mortality. Nerve Sheath Neoplasms / mortality
  • [MeSH-minor] Adult. Aged. Female. Humans. Male. Middle Aged. Prognosis. Survival Analysis

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  • (PMID = 19283843.001).
  • [ISSN] 1097-0347
  • [Journal-full-title] Head & neck
  • [ISO-abbreviation] Head Neck
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 47
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93. Flores LP, Carneiro JZ: Peripheral nerve compression secondary to adjacent lipomas. Surg Neurol; 2007 Mar;67(3):258-62; discussion 262-3

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Peripheral nerve compression secondary to adjacent lipomas.
  • BACKGROUND: Nonneural sheath origin tumors include some rare benign and malignant lesions, and the compression of peripheral nerves by benign fatty tumors is infrequently reported in the medical literature.
  • This study aims to review the authors' experience in treating patients with peripheral nerve compressions secondary to adjacent lipomas.
  • METHODS: This study is a retrospective analysis of data about the patients who presented peripheral nerve compressive symptoms secondary to lipomas in the upper and lower limbs, treated during the period of 1999 to 2006.
  • Included in the cases were those wherein the tumor was in contact with the nerve and the symptoms matched a respective nerve sensitive and/or motor innervation pattern.
  • RESULTS: The upper extremity was the site of 5 (62.5%) of 8 lipomas, followed by the lower limb (2 lesions, 25%), and 1 tumor involved the brachial plexus (12.5%).
  • Ultrasound imaging was useful to define the mass as a nonneural sheath origin tumor; MRI allowed a better analysis of the relationship of the tumor with other vascular, bony, or ligamentous structures.
  • CONCLUSIONS: The surgical treatment offers good outcomes in pain relief and neurological recovery, but one should not expect a real compression effect of the lipoma on the nerve during surgery.
  • [MeSH-major] Lipoma / complications. Lipoma / diagnosis. Nerve Compression Syndromes / diagnosis. Nerve Compression Syndromes / etiology. Peripheral Nervous System Neoplasms / complications
  • [MeSH-minor] Adult. Brachial Plexus Neuropathies / diagnosis. Brachial Plexus Neuropathies / etiology. Female. Humans. Lower Extremity / pathology. Lower Extremity / ultrasonography. Male. Middle Aged. Pain / diagnosis. Pain / etiology. Retrospective Studies. Upper Extremity / pathology. Upper Extremity / ultrasonography

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  • (PMID = 17320633.001).
  • [ISSN] 0090-3019
  • [Journal-full-title] Surgical neurology
  • [ISO-abbreviation] Surg Neurol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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94. Jeong SY, Park SJ, Lee SJ, Park HJ, Kim HJ: Loss of Y chromosome in the malignant peripheral nerve sheet tumor of a patient with Neurofibromatosis type 1. J Korean Med Sci; 2010 May;25(5):804-8
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  • [Title] Loss of Y chromosome in the malignant peripheral nerve sheet tumor of a patient with Neurofibromatosis type 1.
  • In order to determine whether genomic alterations and/or chromosomal aberrations involved in the malignant progression of NF1 were present in a Korean patient with NF1, molecular and cytogenetic analyses were performed on the pathologically normal, benign, and malignant tissues and primary cells cultured from those tissues of the patient.
  • The comparative genomic hybridization (CGH) array revealed a Y chromosome loss in the malignant peripheral nerve sheet tumor (MPNST) tissue.
  • G-banding analysis of 50 metaphase cells showed normal chromosomal patterns in the histopathologically normal and benign cultured cells, but a mosaic Y chromosome loss in the malignant cells.
  • The final karyotype for the malignant cells from MPNST tissue was 45,X,-Y[28]/46,XY[22].
  • [MeSH-major] Chromosomes, Human, Y / genetics. Nerve Sheath Neoplasms / genetics. Neurofibromatosis 1 / genetics
  • [MeSH-minor] Humans. Young Adult

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  • (PMID = 20436723.001).
  • [ISSN] 1598-6357
  • [Journal-full-title] Journal of Korean medical science
  • [ISO-abbreviation] J. Korean Med. Sci.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Korea (South)
  • [Other-IDs] NLM/ PMC2858846
  • [Keywords] NOTNLM ; CGH Array / Chromosome Loss / G-banding / Nerve Sheath Neoplasms / Neurofibromatosis 1, Y chromosome
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95. 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
NCI CPTC Antibody Characterization Program. NCI CPTC Antibody Characterization Program .

  • [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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96. Gonzalez LF, Lekovic GP, Eschbacher J, Coons S, Spetzler RF: A true malignant schwannoma of the eighth cranial nerve: case report. Neurosurgery; 2007 Aug;61(2):E421-2; discussion E422
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] A true malignant schwannoma of the eighth cranial nerve: case report.
  • OBJECTIVE: The clinical presentation, pathology, treatment, and outcome of a 43-year-old woman with a malignant peripheral nerve sheath tumor arising from a benign schwannoma of the eighth cranial nerve are presented.
  • CLINICAL PRESENTATION: Initially, the tumor was debulked.
  • After finding malignant areas within the benign tumor, it was considered to be a malignant transformation of a previously benign tumor.
  • Postoperatively, the tumor bed was radiated for palliation.
  • [MeSH-major] Cranial Nerve Neoplasms / pathology. Neuroma, Acoustic / secondary. Vestibulocochlear Nerve / pathology
  • [MeSH-minor] Adult. Dura Mater / pathology. Fatal Outcome. Female. Humans. Magnetic Resonance Imaging. Meningeal Neoplasms / secondary

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  • (PMID = 17762727.001).
  • [ISSN] 1524-4040
  • [Journal-full-title] Neurosurgery
  • [ISO-abbreviation] Neurosurgery
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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97. 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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98. 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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99. Demetriades AK, Saunders N, Rose P, Fisher C, Rowe J, Tranter R, Hardwidge C: Malignant transformation of acoustic neuroma/vestibular schwannoma 10 years after gamma knife stereotactic radiosurgery. Skull Base; 2010 Sep;20(5):381-7

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Malignant transformation of acoustic neuroma/vestibular schwannoma 10 years after gamma knife stereotactic radiosurgery.
  • Only a handful of cases of de-novo malignancies of the vestibulocochlear nerve have been reported.
  • Even rarer is the malignant transformation of a previously histologically diagnosed benign vestibular schwannoma.
  • We present the case of a young adult who had combined operative/Gamma knife treatment for a benign vestibular schwannoma, followed by further surgery 2 years later.
  • He represented 10 years after original diagnosis with facial numbness and ataxia, MRI showing gross tumor recurrence.
  • After radical resection, histology showed malignant transformation to a malignant peripheral nerve sheath tumor.
  • Histology confirmed further de-differentiation to an anaplastic sarcoma.
  • While awaiting radiotherapy the tumor recurred again, the patient succumbing.
  • In the literature there are 13 other cases of malignant vestibular schwannomata.
  • The tumor biology of vestibular schwannomata as well as the radiobiology in the context of malignant transformation is discussed.

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  • [Cites] Nucleic Acids Res. 1983 Oct 25;11(20):7191-203 [6634412.001]
  • (PMID = 21359005.001).
  • [ISSN] 1532-0065
  • [Journal-full-title] Skull base : official journal of North American Skull Base Society ... [et al.]
  • [ISO-abbreviation] Skull Base
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Other-IDs] NLM/ PMC3023338
  • [Keywords] NOTNLM ; Gamma knife radiosurgery / Vestibular schwannoma / acoustic neuroma / anaplastic sarcoma / malignant peripheral nerve sheath tumor (MPNST) / malignant transformation / radiotherapy
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100. Guo H, Xiong Y, Nong L, Zhang S, Li T: [Reassessment of the pathological diagnosis in 33 cases of malignant fibrous histiocytoma]. Beijing Da Xue Xue Bao; 2008 Aug 18;40(4):374-9
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Reassessment of the pathological diagnosis in 33 cases of malignant fibrous histiocytoma].
  • OBJECTIVE: Since malignant fibrous histiocytoma (MFH) may be taken as an undifferentiated pleomorphic sarcoma (UPS), this study was conducted to reassess 33 previously diagnosed MFH cases in the past 10 years based on the latest WHO concept.
  • METHODS: Thirty-three cases in tissue microarray were studied by immunohistochemistry with panels of neurogenic, myogenic, and lipogenic antibodies.
  • RESULTS: Among the 33 cases, 17 cases (51.5%) of MFH had their diagnoses changed, including 5 leiomyosarcomas, 3 malignant peripheral nerve sheath tumors, 1 fibrosarcoma, 1 inflammatory myofibrosarcoma, 1 giant cell tumor and 1 angiomatoid fibrous histiocytoma.
  • The median tumor size was 6.0 cm (range: 3.0 to 14.0 cm), 8 cases (50%) located in lower limb and 5 cases (31.3%) located in thigh.
  • Eleven cases (68.8%) variously expressed CD68 (KP1) and 7 cases (43.8%) expressed CD68 (PG-M1), which were much higher than leiomyosarcoma, malignant peripheral nerve sheath tumor and liposarcoma with significant difference.
  • CONCLUSION: MFH/UPS often show marked histological pleomorphism, and the diagnosis must be made by exclusion of other definitive sarcomas, especially myogenic and neurogenic sarcoma.
  • [MeSH-minor] Adult. Aged. Diagnosis, Differential. Humans. Immunohistochemistry. Middle Aged

  • Genetic Alliance. consumer health - Malignant fibrous histiocytoma.
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  • (PMID = 18677383.001).
  • [ISSN] 1671-167X
  • [Journal-full-title] Beijing da xue xue bao. Yi xue ban = Journal of Peking University. Health sciences
  • [ISO-abbreviation] Beijing Da Xue Xue Bao
  • [Language] chi
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] China
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