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1. Molina AR, Brasch H, Tan ST: Malignant peripheral nerve sheath tumour of the cervical vagus nerve in a neurofibromatosis type 1 patient. J Plast Reconstr Aesthet Surg; 2006;59(12):1458-62
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  • [Title] Malignant peripheral nerve sheath tumour of the cervical vagus nerve in a neurofibromatosis type 1 patient.
  • One serious complication of neurofibromatosis type 1 (NF1) is the development of malignant peripheral nerve sheath tumours (MPNSTs).
  • Recent work demonstrates that the lifetime risk of malignant transformation is significantly greater than previously thought.
  • We present an NF1 patient who developed an MPNST of the cervical vagus nerve which was successfully treated with surgery.
  • Close monitoring of patients with NF and a high level of suspicion towards rapidly enlarging and painful swellings is merited as these features may signify malignant transformation.
  • Whether a positive history of MPNST in other affected family members predisposes the individual to a higher risk of malignant transformation is unclear.
  • [MeSH-major] Cranial Nerve Neoplasms / diagnosis. Nerve Sheath Neoplasms / diagnosis. Neurofibromatosis 1 / pathology. Vagus Nerve. Vagus Nerve Diseases / diagnosis
  • [MeSH-minor] Adult. Female. Humans. Magnetic Resonance Imaging


2. 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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3. 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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4. 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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  • [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] United States
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5. Warbey VS, Ferner RE, Dunn JT, Calonje E, O'Doherty MJ: [18F]FDG PET/CT in the diagnosis of malignant peripheral nerve sheath tumours in neurofibromatosis type-1. Eur J Nucl Med Mol Imaging; 2009 May;36(5):751-7
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  • [Title] [18F]FDG PET/CT in the diagnosis of malignant peripheral nerve sheath tumours in neurofibromatosis type-1.
  • PURPOSE: The detection of malignant peripheral nerve sheath tumours (MPNSTs) in patients with neurofibromatosis 1 (NF1) remains a clinical challenge.
  • The purpose of this study was to evaluate the use of [(18)F]2-fluoro-2-deoxy-D-glucose PET/CT (FDG PET/CT with early and delayed imaging) in patients with symptomatic neurofibromas, to revalidate current cut-off values for identification of malignant change within neurofibromas and to examine the relationship between SUV and tumour grade.
  • Sensitivity of FDG PET/CT in diagnosing NF1-associated MPNST was 0.97 (95% CI 0.81-0.99) and the specificity was 0.87 (CI 0.74-0.95).
  • There was a significant difference in SUVmax between early and delayed imaging and in SUVmax between tumours identified as benign and malignant on PET/CT.
  • CONCLUSION: FDG PET/CT is a highly sensitive and specific imaging modality for the diagnosis of MPNST in NF1 patients.
  • [MeSH-major] Fluorodeoxyglucose F18. Nerve Sheath Neoplasms / pathology. Neurofibromatosis 1 / diagnosis. Neurofibromatosis 1 / pathology. Positron-Emission Tomography / methods. Radiopharmaceuticals. Tomography, X-Ray Computed / methods
  • [MeSH-minor] Adolescent. Adult. Aged. Aged, 80 and over. Child. Humans. Middle Aged. Reproducibility of Results. Sensitivity and Specificity


6. Karube K, Nabeshima K, Ishiguro M, Harada M, Iwasaki H: cDNA microarray analysis of cancer associated gene expression profiles in malignant peripheral nerve sheath tumours. J Clin Pathol; 2006 Feb;59(2):160-5
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  • [Title] cDNA microarray analysis of cancer associated gene expression profiles in malignant peripheral nerve sheath tumours.
  • BACKGROUND: Malignant peripheral nerve sheath tumour (MPNST) is a highly aggressive malignancy that arises within peripheral nerves, and is associated with poor prognosis.
  • Little is known about the underlying biology of MPNST, especially the mechanisms involved in cell proliferation, invasion, or escape from apoptosis.
  • AIMS: To identify genes differentially expressed in MPNST compared with benign tumours, such as neurofibromas and schwannomas, by means of cDNA microarray analysis.
  • METHODS: Six MPNST cases and five benign cases (three schwannomas and two neurofibromas) were analysed.
  • RESULTS: Six genes (keratin 18, survivin, tenascin C, adenosine deaminase, collagen type VIa3, and collagen type VIIa1) were significantly upregulated in MPNST, whereas one gene, insulin-like growth factor binding protein 6, was downregulated in MPNST.
  • Immunohistochemistry confirmed upregulation of survivin in MPNST at the protein level in six of eight cases compared with benign tumours.
  • Tenascin C was also expressed at the invasive front and tumorous stroma in all MPNST cases.
  • MPNST cells expressed tenascin C in four of nine cases.
  • CONCLUSIONS: Survivin and tenascin C may be associated with the malignant potential of MPNST and could be considered as potential therapeutic targets.
  • [MeSH-major] Gene Expression Regulation, Neoplastic. Neoplasm Proteins / biosynthesis. Nerve Sheath Neoplasms / metabolism. Peripheral Nervous System Neoplasms / metabolism
  • [MeSH-minor] Adult. Aged. Aged, 80 and over. Female. Gene Expression Profiling / methods. Humans. Immunoenzyme Techniques. Inhibitor of Apoptosis Proteins. Male. Microtubule-Associated Proteins / biosynthesis. Microtubule-Associated Proteins / genetics. Middle Aged. Neurilemmoma / genetics. Neurilemmoma / metabolism. Neurilemmoma / pathology. Neurofibroma / genetics. Neurofibroma / metabolism. Neurofibroma / pathology. Oligonucleotide Array Sequence Analysis / methods. Reverse Transcriptase Polymerase Chain Reaction / methods. Tenascin / biosynthesis. Tenascin / genetics. Up-Regulation

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  • (PMID = 16443732.001).
  • [ISSN] 0021-9746
  • [Journal-full-title] Journal of clinical pathology
  • [ISO-abbreviation] J. Clin. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / BIRC5 protein, human; 0 / Inhibitor of Apoptosis Proteins; 0 / Microtubule-Associated Proteins; 0 / Neoplasm Proteins; 0 / Tenascin
  • [Other-IDs] NLM/ PMC1860323
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7. Storlazzi CT, Brekke HR, Mandahl N, Brosjö O, Smeland S, Lothe RA, Mertens F: Identification of a novel amplicon at distal 17q containing the BIRC5/SURVIVIN gene in malignant peripheral nerve sheath tumours. J Pathol; 2006 Aug;209(4):492-500
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Identification of a novel amplicon at distal 17q containing the BIRC5/SURVIVIN gene in malignant peripheral nerve sheath tumours.
  • Previous studies have suggested that amplification of genes, notably the TOP2A gene, on chromosome arm 17q may be important for the development of malignant peripheral nerve sheath tumour (MPNST).
  • Increased copy numbers were seen for the ERBB2 and TOP2A genes in eight and nine cases, respectively, supporting a potential role for these two genes in MPNST tumourigenesis.
  • Among the genes mapping to this region, BIRC5, which encodes the baculoviral IAP repeat-containing protein 5/survivin protein, is a strong candidate target gene for amplification, as it has been previously shown to be overexpressed in neurofibromatosis type 1-associated MPNST.
  • Three other genes that co-amplified with BIRC5 represent other potential candidate genes: PTDSR involved in apoptosis; SEPT9 overexpressed in human malignant brain tumours; and SOCS3 involved in cell survival and differentiation of neurons.
  • [MeSH-major] Chromosomes, Human, Pair 17. Genes, Neoplasm. Microtubule-Associated Proteins / genetics. Neoplasm Proteins / genetics. Nerve Sheath Neoplasms / genetics. Sarcoma / genetics
  • [MeSH-minor] Adolescent. Adult. Aged. Child. Chromosome Banding. DNA Topoisomerases, Type II / genetics. Female. Follow-Up Studies. Gene Amplification. Gene Dosage. Genes, erbB-2. Humans. In Situ Hybridization, Fluorescence. Inhibitor of Apoptosis Proteins. Interphase. Male. Metaphase. Middle Aged. Neurofibromatosis 1 / genetics. Neurofibromatosis 1 / pathology

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  • [Copyright] Copyright 2006 Pathological Society of Great Britain and Ireland.
  • (PMID = 16721726.001).
  • [ISSN] 0022-3417
  • [Journal-full-title] The Journal of pathology
  • [ISO-abbreviation] J. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / BIRC5 protein, human; 0 / Inhibitor of Apoptosis Proteins; 0 / Microtubule-Associated Proteins; 0 / Neoplasm Proteins; EC 5.99.1.3 / DNA Topoisomerases, Type II
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8. 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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9. 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


10. 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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11. Thanakit V, Shuangshoti S, Ruangvejvorachai P: Expression of CyclinD1, p27kp-1, and bcl-2 in plexiform neurofibroma with and without malignant transformation in neurofibromatosis type 1. J Med Assoc Thai; 2006 Sep;89 Suppl 3:S53-7
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  • [Title] Expression of CyclinD1, p27kp-1, and bcl-2 in plexiform neurofibroma with and without malignant transformation in neurofibromatosis type 1.
  • Plexiform neurofibroma (PNF) has a low potential to undergo malignant transformation.
  • Identification of markers associated with tumor progression is important since it may serve as prognostic indicators or adjuncts to standard pathological examination.
  • Six of the cases had progressed into malignant peripheral nerve sheath tumor (MPNST), and the transitional area of each sample was also stained separately in order to identify protein(s) associated with tumor progression.
  • The protein is, thus, likely to play a role in the malignant transformation.
  • There was no significant difference in the expression of p27kip-1 and bcl-2 during the malignant progression of PNF.
  • [MeSH-minor] Adolescent. Adult. Biomarkers, Tumor / metabolism. Cell Transformation, Neoplastic. Child. Cyclin-Dependent Kinase Inhibitor p27. Disease Progression. Female. Humans. Immunohistochemistry. Male. Middle Aged


12. Tucker T, Friedman JM, Friedrich RE, Wenzel R, Fünsterer C, Mautner VF: Longitudinal study of neurofibromatosis 1 associated plexiform neurofibromas. J Med Genet; 2009 Feb;46(2):81-5
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  • These tumours can cause serious complications and can also progress to malignant peripheral nerve sheath tumours (MPNSTs), one of the leading causes of death among NF1 patients.
  • Biopsy showed the presence of an MPNST.
  • [MeSH-minor] Adolescent. Adult. Child. Child, Preschool. Female. Humans. Infant. Longitudinal Studies. Magnetic Resonance Imaging. Male. Middle Aged

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  • (PMID = 18930997.001).
  • [ISSN] 1468-6244
  • [Journal-full-title] Journal of medical genetics
  • [ISO-abbreviation] J. Med. Genet.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
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13. Landy H, Feun L, Markoe A, Patchen S, Bruce J, Marcus J, Levi A: Extended remission of a recurrent median nerve malignant peripheral nerve sheath tumor after multimodal treatment. Case report. J Neurosurg; 2005 Oct;103(4):760-3
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  • [Title] Extended remission of a recurrent median nerve malignant peripheral nerve sheath tumor after multimodal treatment. Case report.
  • Malignant peripheral nerve sheath tumors (MPNSTs) are difficult to control despite aggressive treatment.
  • In this report the authors describe the treatment and follow-up review of a patient with neurofibromatosis Type I who harbored a recurrent median nerve MPNST.
  • 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


14. 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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15. Friedrich RE, Hartmann M, Mautner VF: Malignant peripheral nerve sheath tumors (MPNST) in NF1-affected children. Anticancer Res; 2007 Jul-Aug;27(4A):1957-60
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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 (MPNST) in NF1-affected children.
  • BACKGROUND: Malignant peripheral nerve sheath tumors (MPNST) constitute a heterogeneous group of malignant tumors that probably arise from cells of the peripheral nerve sheath.
  • Association of MPNST with neurofibromatosis type 1 (NF1) is frequently reported.
  • MPNST contribute significantly to the reduced life-span of NF1-patients.
  • At present there are only sparse data on MPNST in NF1-children.
  • The aim of this study was to determine the outcome of children affected with NF1 who developed an MPNST.
  • MATERIALS AND METHODS: Over the period of 1985 to 2005, we followed 52 NF1 patients with MPNST at our outpatient department.
  • RESULTS: Out of this cohort, 8 patients with MPNST were aged 1 to 17 years at the time of MPNST diagnosis (mean age: 12 years; 5 girls and 3 boys).
  • We noticed the following characteristics: MPNST arose from plexiform neurofibromas (PNF) with invasive or displacing growth pattern on MRI.
  • Diagnosis of MPNST in this age group took longer compared to adults.
  • This cohort did not show longer survival periods than adults with MPNST.
  • The longest survival of 112 months was achieved for a girl who presented with MPNST of the distal upper arm and underwent amputation.
  • The NF1 mutation analysis in the MPNST pediatric age group revealed the same mutational spectrum as the adult group.
  • CONCLUSION: Our data reveal MPNST in children with NF1.
  • [MeSH-major] Nerve Sheath Neoplasms / complications. Nerve Sheath Neoplasms / diagnosis. Neurofibromatosis 1 / complications

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  • (PMID = 17649804.001).
  • [ISSN] 0250-7005
  • [Journal-full-title] Anticancer research
  • [ISO-abbreviation] Anticancer Res.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Greece
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16. 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


17. 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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18. 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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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [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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19. 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


20. Minovi A, Basten O, Hunter B, Draf W, Bockmühl U: Malignant peripheral nerve sheath tumors of the head and neck: management of 10 cases and literature review. Head Neck; 2007 May;29(5):439-45
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Malignant peripheral nerve sheath tumors of the head and neck: management of 10 cases and literature review.
  • BACKGROUND: This study analyzes the management and outcomes of a series of 10 malignant peripheral nerve sheath tumors (MPNST) of the head and neck.
  • METHODS: From 1984 to 2004, 10 patients underwent surgical treatment of a MPNST.
  • RESULTS: Eight tumors were located at the lateral skull base; 2 involved the vagus nerve in isolation.
  • Negative prognostic indicators were advanced tumor stage, early recurrence, and presumably also the presence of von Recklinghausen's disease.
  • CONCLUSIONS: Although rare, MPNST is one of the most aggressive tumors in the head and neck area.
  • Complete tumor removal is the mainstay of treatment and most important prognostic factor of MPNST.
  • [MeSH-major] Head and Neck Neoplasms / mortality. Head and Neck Neoplasms / therapy. Neoplasm Recurrence, Local / mortality. Nerve Sheath Neoplasms / mortality. Nerve Sheath Neoplasms / therapy
  • [MeSH-minor] Adolescent. Adult. Aged. Chemotherapy, Adjuvant. Female. Humans. Male. Middle Aged. Prognosis. Radiotherapy, Adjuvant. Retrospective Studies. Survival Rate

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  • [Copyright] (c) 2006 Wiley Periodicals, Inc.
  • (PMID = 17163467.001).
  • [ISSN] 1043-3074
  • [Journal-full-title] Head & neck
  • [ISO-abbreviation] Head Neck
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 34
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21. 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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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [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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22. 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
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  • [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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23. 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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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [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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24. Radha S, Afroz T, Satyanarayana G: Paratesticular malignant peripheral nerve sheath tumour: a case report. Indian J Pathol Microbiol; 2006 Oct;49(4):614-5
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  • [Title] Paratesticular malignant peripheral nerve sheath tumour: a case report.
  • Primary malignant peripheral nerve sheath tumours (MPNST) of intrascrotal extra testicular site are extremely rare with only few cases reported in literature.
  • These are highly malignant tumours most often associated with neurofibromatosis.
  • The lesion was excised and was diagnosed as MPNST.
  • [MeSH-major] Nerve Sheath Neoplasms / diagnosis. Sarcoma / diagnosis. Scrotum / pathology
  • [MeSH-minor] Adult. Humans. Male

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  • (PMID = 17183877.001).
  • [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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25. Spurlock G, Griffiths S, Uff J, Upadhyaya M: Somatic alterations of the NF1 gene in an NF1 individual with multiple benign tumours (internal and external) and malignant tumour types. Fam Cancer; 2007;6(4):463-71
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  • [Title] Somatic alterations of the NF1 gene in an NF1 individual with multiple benign tumours (internal and external) and malignant tumour types.
  • We have carried out NF1 gene mutation analysis on DNA isolated from 25 tumours (dermal and plexiform neurofibromas, malignant peripheral nerve sheath tumour, MPNST), obtained at post-mortem from an NF1 patient.
  • [MeSH-minor] Adolescent. Adult. Alleles. Base Sequence. Child, Preschool. Chromatography, High Pressure Liquid. Exons / genetics. Gene Deletion. Humans. Microsatellite Instability. Molecular Sequence Data. Tumor Suppressor Protein p53 / genetics

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  • (PMID = 17551851.001).
  • [ISSN] 1389-9600
  • [Journal-full-title] Familial cancer
  • [ISO-abbreviation] Fam. Cancer
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Netherlands
  • [Chemical-registry-number] 0 / Neurofibromin 1; 0 / Tumor Suppressor Protein p53
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26. Pfeiffer J, Arapakis I, Boedeker CC, Ridder GJ: Malignant peripheral nerve sheath tumour of the paranasal sinuses and the anterior skull base. J Craniomaxillofac Surg; 2008 Jul;36(5):293-9
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  • [Title] Malignant peripheral nerve sheath tumour of the paranasal sinuses and the anterior skull base.
  • BACKGROUND: Malignant peripheral nerve sheath tumours (MPNSTs) are highly aggressive neoplasms with a marked propensity for local recurrence and metastatic spread.
  • PATIENTS AND METHODS: We present a case of MPNST of the anterior skull base and provide an overview of all MPNSTs reported since 1970, in which the tumour location was the anterior skull base or the paranasal sinuses.
  • CONCLUSIONS: Despite multimodal therapy and advances in surgical techniques, the prognosis of MPNST located in the paranasal sinuses and the anterior skull base remains dismal.
  • [MeSH-major] Nerve Sheath Neoplasms / pathology. Nose Neoplasms / pathology. Paranasal Sinus Neoplasms / pathology. Skull Base Neoplasms / pathology
  • [MeSH-minor] Adult. Humans. Male. Prognosis

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  • (PMID = 18362076.001).
  • [ISSN] 1010-5182
  • [Journal-full-title] Journal of cranio-maxillo-facial surgery : official publication of the European Association for Cranio-Maxillo-Facial Surgery
  • [ISO-abbreviation] J Craniomaxillofac Surg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] Scotland
  • [Number-of-references] 38
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27. Merimsky O, Soyfer V, Kovner F, Bickels J, Issakov J, Flusser G, Meller I, Ofer O, Kollender Y: Limb sparing approach: adjuvant radiation therapy in adults with intermediate or high-grade limb soft tissue sarcoma. Radiother Oncol; 2005 Dec;77(3):295-300
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  • [Title] Limb sparing approach: adjuvant radiation therapy in adults with intermediate or high-grade limb soft tissue sarcoma.
  • PATIENTS AND METHODS: Between October 1994 and October 2002, 133 adult patients with intermediate or high-grade limb STS were approached by LSS+RT.
  • DFS and OS were influenced by disease stage II vs I, primary site in the upper limb vs lower limb, MPNST vs other types, induction therapy vs no induction, adequate resection vs marginal resection or involved margins, and good response to induction therapy vs bad response.
  • DFS and OS were Patient's age and sex, tumor depth, acute or late toxicity of RT, or the interval of time between the date of definitive surgery and the start of RT did not affect DFS and or OS.
  • [MeSH-major] Limb Salvage. Sarcoma / radiotherapy. Sarcoma / surgery
  • [MeSH-minor] Adolescent. Adult. Aged. Aged, 80 and over. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Female. Humans. Male. Middle Aged. Radiotherapy, Adjuvant. Radiotherapy, Conformal. Treatment Outcome

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  • (PMID = 16300847.001).
  • [ISSN] 0167-8140
  • [Journal-full-title] Radiotherapy and oncology : journal of the European Society for Therapeutic Radiology and Oncology
  • [ISO-abbreviation] Radiother Oncol
  • [Language] eng
  • [Publication-type] Clinical Trial; Journal Article
  • [Publication-country] Ireland
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28. Fauth C, Kehrer-Sawatzki H, Zatkova A, Machherndl-Spandl S, Messiaen L, Amann G, Hainfellner JA, Wimmer K: Two sporadic spinal neurofibromatosis patients with malignant peripheral nerve sheath tumour. Eur J Med Genet; 2009 Nov-Dec;52(6):409-14
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  • [Title] Two sporadic spinal neurofibromatosis patients with malignant peripheral nerve sheath tumour.
  • We analyzed two unrelated male patients in whom neurofibromatosis type 1 (NF1) was not suspected until they presented with malignant peripheral nerve sheath tumours (MPNSTs) in their thirties and forties, respectively.
  • Patient A presented with progressive peroneus paresis due to a rapidly growing MPNST in the thigh.
  • MRI examination revealed multiple symmetrical spinal neurofibromas in this patient as well as in patient B who presented at the age of 42 with paraparesis and an MPNST at spinal level L4.
  • To our knowledge these are the first MPNST patients with the clinical phenotype of SNF.
  • The clinical course observed in these two patients suggests that nodular plexiform neurofibromas and spinal-nerve-root neurofibromas which may be asymptomatic for a long time and, hence, unrecognized in SNF patients bear the risk for malignant transformation.
  • [MeSH-major] Nerve Sheath Neoplasms / diagnosis. Neurofibromatosis 1 / diagnosis. Spinal Neoplasms / diagnosis
  • [MeSH-minor] Adult. Base Sequence. DNA Primers. Genes, p53. Humans. Hybrid Cells. Loss of Heterozygosity. Magnetic Resonance Imaging. Male. Mutation. Reverse Transcriptase Polymerase Chain Reaction

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  • (PMID = 19665063.001).
  • [ISSN] 1878-0849
  • [Journal-full-title] European journal of medical genetics
  • [ISO-abbreviation] Eur J Med Genet
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Netherlands
  • [Chemical-registry-number] 0 / DNA Primers
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29. 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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  • [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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30. 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


31. Rizvi S, Mehboob J, Asghar AH, Mateen A, Raza T, Hameed A: Omental caking: a rare manifestation of malignant peripheral nerve sheath tumour. J Coll Physicians Surg Pak; 2010 Aug;20(8):554-5
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  • [Title] Omental caking: a rare manifestation of malignant peripheral nerve sheath tumour.
  • Malignant peripheral nerve sheath tumour (MPNST) is a very rare tumour with an incidence of one per 100,000 and constitutes between 3 to 10% of all soft tissue sarcomas.
  • Most of the sarcoma involve the extremities and retroperitoneal regions.
  • [MeSH-major] Nerve Sheath Neoplasms / pathology. Nerve Sheath Neoplasms / radiography. Omentum. Peritoneal Neoplasms / pathology. Peritoneal Neoplasms / radiography. Tomography, X-Ray Computed
  • [MeSH-minor] Abdominal Neoplasms / pathology. Adult. Humans. Inguinal Canal. Male

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  • (PMID = 20688026.001).
  • [ISSN] 1022-386X
  • [Journal-full-title] Journal of the College of Physicians and Surgeons--Pakistan : JCPSP
  • [ISO-abbreviation] J Coll Physicians Surg Pak
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Pakistan
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32. 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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33. 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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34. 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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  • [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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35. 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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  • [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


36. 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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37. 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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38. 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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39. 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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  • [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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40. 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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41. Cavallaro G, Basile U, Polistena A, Giustini S, Arena R, Scorsi A, Zinnamosca L, Letizia C, Calvieri S, De Toma G: Surgical management of abdominal manifestations of type 1 neurofibromatosis: experience of a single center. Am Surg; 2010 Apr;76(4):389-96
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Abdominal manifestations of NF1 include five kinds of tumors: neurogenic tumors (neurofibromas, malignant peripheral nerve sheath tumors [MPNSTs], and ganglioneuromas); neuroendocrine tumors (pheochromocytomas and carcinoids); nonneurogenic gastrointestinal stromal tumors (GISTs); embryonal tumors; and miscellaneous.
  • Three patients underwent adrenalectomy for pheochromocytoma (in one case associated with jejunal wall neurofibroma); two patients were found to be affected by MPNST (recurrent and unresectable in one case).
  • Further studies are necessary to detect predictive factors of malignant tumor development of severe clinical conditions.
  • [MeSH-minor] Adolescent. Adrenal Gland Neoplasms / diagnosis. Adrenal Gland Neoplasms / surgery. Adult. Female. Gastrointestinal Neoplasms / diagnosis. Gastrointestinal Neoplasms / surgery. Humans. Magnetic Resonance Imaging. Male. Middle Aged. Peripheral Nervous System Neoplasms / diagnosis. Peripheral Nervous System Neoplasms / surgery. Pheochromocytoma / diagnosis. Pheochromocytoma / surgery. Retroperitoneal Neoplasms / diagnosis. Retroperitoneal Neoplasms / surgery. Tomography, X-Ray Computed. Treatment Outcome

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  • (PMID = 20420249.001).
  • [ISSN] 0003-1348
  • [Journal-full-title] The American surgeon
  • [ISO-abbreviation] Am Surg
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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42. 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


43. 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]
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  • (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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44. 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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45. Brenner W, Friedrich RE, Gawad KA, Hagel C, von Deimling A, de Wit M, Buchert R, Clausen M, Mautner VF: Prognostic relevance of FDG PET in patients with neurofibromatosis type-1 and malignant peripheral nerve sheath tumours. Eur J Nucl Med Mol Imaging; 2006 Apr;33(4):428-32
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  • [Title] Prognostic relevance of FDG PET in patients with neurofibromatosis type-1 and malignant peripheral nerve sheath tumours.
  • PURPOSE: In patients with neurofibromatosis type-1 (NF1) and malignant peripheral nerve sheath tumours (MPNSTs), survival rates are low and time to death is often less than 2 years.
  • Since histopathology and tumour grading are not well correlated with prognosis, we aimed to evaluate the potential of (18)F-fluorodeoxyglucose positron emission tomography (FDG PET) for prediction of patient outcome in MPNST.
  • [MeSH-major] Fluorodeoxyglucose F18. Nerve Sheath Neoplasms / diagnostic imaging. Nerve Sheath Neoplasms / mortality. Neurofibromatosis 1 / diagnostic imaging. Neurofibromatosis 1 / mortality. Positron-Emission Tomography / statistics & numerical data. Risk Assessment / methods
  • [MeSH-minor] Adolescent. Adult. Comorbidity. Female. Germany / epidemiology. Humans. Male. Middle Aged. Outcome Assessment (Health Care) / methods. Outcome Assessment (Health Care) / statistics & numerical data. Prognosis. Radiopharmaceuticals. Reproducibility of Results. Risk Factors. Sensitivity and Specificity. Survival Rate

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  • (PMID = 16404595.001).
  • [ISSN] 1619-7070
  • [Journal-full-title] European journal of nuclear medicine and molecular imaging
  • [ISO-abbreviation] Eur. J. Nucl. Med. Mol. Imaging
  • [Language] eng
  • [Publication-type] Clinical Trial; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Radiopharmaceuticals; 0Z5B2CJX4D / Fluorodeoxyglucose F18
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46. 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
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  • [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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47. 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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  • [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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48. Bottillo I, Ahlquist T, Brekke H, Danielsen SA, van den Berg E, Mertens F, Lothe RA, Dallapiccola B: Germline and somatic NF1 mutations in sporadic and NF1-associated malignant peripheral nerve sheath tumours. J Pathol; 2009 Apr;217(5):693-701
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  • [Title] Germline and somatic NF1 mutations in sporadic and NF1-associated malignant peripheral nerve sheath tumours.
  • Malignant peripheral nerve sheath tumours (MPNSTs) are a malignancy occurring with increased frequency in patients with neurofibromatosis type 1 (NF1).
  • In addition, DNA from peripheral blood and cutaneous neurofibroma biopsies from, respectively, 14/25 and 7/25 of the NF1 patients were analysed.
  • The spectrum of germline NF1 mutations in neurofibromatosis patients with MPNST is different from the spectrum of somatic mutations seen in MPNSTs.
  • [MeSH-major] Germ-Line Mutation. Nerve Sheath Neoplasms / genetics. Neurofibromatosis 1 / genetics
  • [MeSH-minor] Adolescent. Adult. Aged. Child. Chromatography, High Pressure Liquid / methods. DNA, Neoplasm / genetics. Female. Genetic Predisposition to Disease. Humans. Male. Middle Aged. Neoplasm Proteins / genetics. Point Mutation. Proto-Oncogene Proteins / genetics. Proto-Oncogene Proteins B-raf / genetics. ras Proteins / genetics

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  • (PMID = 19142971.001).
  • [ISSN] 1096-9896
  • [Journal-full-title] The Journal of pathology
  • [ISO-abbreviation] J. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / DNA, Neoplasm; 0 / KRAS protein, human; 0 / Neoplasm Proteins; 0 / Proto-Oncogene Proteins; EC 2.7.11.1 / BRAF protein, human; EC 2.7.11.1 / Proto-Oncogene Proteins B-raf; EC 3.6.5.2 / ras Proteins
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49. Hatori M, Hosaka M, Watanabe M, Moriya T, Sasano H, Kokubun S: Osteosarcoma in a patient with neurofibromatosis type 1: a case report and review of the literature. Tohoku J Exp Med; 2006 Apr;208(4):343-8
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  • Malignant tumors of the nervous system, such as malignant schwannomas, gliomas, or astrocytomas, have been well known to coexist with neurofibromatosis.
  • However, occurrence of malignant tumors unrelated to the nervous system is rare.
  • We report an unusual case of a 29-year-old NF1 female suffering from malignant peripheral nerve sheath tumor (MPNST) that eventually developed osteosarcoma in the proximal femur.
  • Osteosarcoma is the most common high-grade malignant bone tumor in which the neoplastic cells produce osteoid.
  • At 23 and 24 years old, she underwent excision of MPNST in the left posterior thigh.
  • Computed tomography demonstrated cortical bone destruction in the left proximal femur where MPNST occurred.
  • Magnetic resonance imaging revealed extraskeletal growth of the tumor.
  • The removed tumor was composed of highly anaplastic cells.
  • Lace-like irregular osteoid formation was observed among the tumor cells.
  • MPNST component was totally absent.
  • The tumor was diagnosed as osteoblastic type osteosarcoma.
  • The correlation between the histogenesis of osteosarcoma and the genetic abnormality in NF1 patients has not been elucidated, but the finding of osteosarcomatous transformation in this case suggests the divergent cellular differentiation to mesenchymal malignant tumors of neuroectodermal tissue in NF1 patients.
  • [MeSH-minor] Adult. Cell Transformation, Neoplastic. Combined Modality Therapy. Fatal Outcome. Female. Femur / pathology. Femur / radiography. Humans. Magnetic Resonance Imaging. Nerve Sheath Neoplasms / pathology. Nerve Sheath Neoplasms / therapy


50. Mautner VF, Brenner W, Fünsterer C, Hagel C, Gawad K, Friedrich RE: Clinical relevance of positron emission tomography and magnetic resonance imaging in the progression of internal plexiform neurofibroma in NF1. Anticancer Res; 2007 Jul-Aug;27(4A):1819-22
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  • Neurofibromatosis type 1 (NF1) is a frequent and inherited disease with a predisposition for malignant peripheral nerve sheath tumor (MPNST) development.
  • MPNST are soft tissue sarcomas that arise from peripheral nerves, being one of the most aggressive malignancies in humans with extremely poor prognosis.
  • MPNST frequently arise from a previously undetected plexiform neurofibroma (PNF).
  • The malignant transformation of an internal PNF to an MPNST is difficult to assess and requires advanced imaging techniques like magnetic resonance imaging or positron emission tomography.
  • Despite the high quality of current diagnostics, the changing tumor biology inside a plexiform neurofibroma cannot currently be visualized accurately.
  • We report 4 cases of NF1 patients with PNF who showed imaging findings suspicious for malignant degeneration, but proved to have MPNST in only one case.
  • Three tumors might represent an intermediate type between PNF and MPNST.
  • Ablative surgery and complete histological work-up of specimens is the only way to clarify tumor status, thereby enabling provision of adequate local treatment.
  • [MeSH-major] Nerve Sheath Neoplasms / diagnosis. Neurofibroma, Plexiform / diagnosis. Neurofibromatosis 1 / complications
  • [MeSH-minor] Adult. Child. Diagnosis, Differential. Disease Progression. Female. Humans. Magnetic Resonance Imaging. Male. Middle Aged. Positron-Emission Tomography

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  • (PMID = 17649778.001).
  • [ISSN] 0250-7005
  • [Journal-full-title] Anticancer research
  • [ISO-abbreviation] Anticancer Res.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Greece
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51. 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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  • [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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52. Upadhyaya M, Spurlock G, Majounie E, Griffiths S, Forrester N, Baser M, Huson SM, Gareth Evans D, Ferner R: The heterogeneous nature of germline mutations in NF1 patients with malignant peripheral serve sheath tumours (MPNSTs). Hum Mutat; 2006 Jul;27(7):716
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  • [Title] The heterogeneous nature of germline mutations in NF1 patients with malignant peripheral serve sheath tumours (MPNSTs).
  • Malignant peripheral nerve sheath tumours (MPNSTs) are a major cause of mortality in patients with neurofibromatosis 1 (NF1).
  • Several studies have suggested that NF1 patients with a constitutional 1.5 Mb deletion of the NF1 gene have an increased risk of developing malignant peripheral nerve sheath tumours (MPNSTs).
  • Larger prospective studies are needed to ascertain whether there is a different spectrum of NF1 mutations in NF1 patients with high grade compared to low grade MPNSTs and of patients with the 1.5Mb deletion, in order to determine the true frequency of MPNST in this sub-group of NF1 patients.
  • [MeSH-major] Genes, Neurofibromatosis 1. Genetic Heterogeneity. Germ-Line Mutation. Nerve Sheath Neoplasms / genetics. Neurofibromatosis 1 / genetics
  • [MeSH-minor] Adolescent. Adult. DNA Mutational Analysis. Humans. Middle Aged

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  • [Copyright] Copyright 2006 Wiley-Liss, Inc.
  • (PMID = 16786508.001).
  • [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
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53. 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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54. Friedrich RE, Derlin T, Hagel C: Atypical plexiform neurofibroma in NF1 with high standardised uptake value (SUV) in positron-emission tomography (PET) expressing podoplanin. In Vivo; 2010 Nov-Dec;24(6):871-6
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  • A 19-year-old female with established neurofibromatosis type 1 (NF1) diagnosis and history of malignant peripheral nerve sheath tumour (MPNST) of the lower extremities showed a tumour of her right upper extremity with a maximum standardised uptake value (SUV) of 5.7 on positron emission/computerised tomography (PET/CT) scan.
  • Scattered nerve fibres were labelled with neurofilament antibodies within the tumour.
  • This atypical neurofibroma showed a high SUV on PET that is indicative for MPNST.
  • [MeSH-minor] Biomarkers, Tumor / biosynthesis. Female. Humans. Positron-Emission Tomography. Young Adult

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  • (PMID = 21164047.001).
  • [ISSN] 1791-7549
  • [Journal-full-title] In vivo (Athens, Greece)
  • [ISO-abbreviation] In Vivo
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Greece
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Membrane Glycoproteins; 0 / PDPN protein, human
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55. 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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56. Katenkamp K, Katenkamp D: [Low-malignant peripheral nerve sheath tumors of nasal and sinonasal mucous membranes]. Pathologe; 2005 Mar;26(2):90-5
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  • [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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57. Schittenhelm J, Thiericke J, Nagel C, Meyermann R, Beschorner R: WT1 expression in normal and neoplastic cranial and peripheral nerves is independent of grade of malignancy. Cancer Biomark; 2010;7(2):73-7
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] WT1 expression in normal and neoplastic cranial and peripheral nerves is independent of grade of malignancy.
  • OBJECTIVE: Wilms' tumor protein (WT1) expression is usually absent in normal glial cells of the CNS but is highly upregulated in brain tumor cells and its expression correlates with tumor grade.
  • However, knowledge on WT1 expression in tumors of the peripheral nerve system (PNS) is limited.
  • As WT1 antibodies not only serve as biomarker for cancerous tissue but also are considered for cancer immunotherapy, knowledge of WT1 expression in tumorous and normal peripheral nerve tissue is important for therapeutical purposes.
  • METHODS: We analyze the immunohistochemical expression of WT1 in 101 samples consisting of 13 normal nerves, 10 neurofibromas, 69 schwannomas and 9 malignant peripheral nerve sheath tumors (MPNST).
  • Tumor samples included 14 specimen from patients with a proven neurocutaneous disorder (neurofibromatosis type 1 and 2) and 3 cases of schwannomatosis.
  • In 50 vestibular schwannomas tumor growth extension was correlated to WT1 expression.
  • In peripheral nerve sheath tumors, cytoplasmic WT1 protein is expressed in the cytoplasm of the neoplastic cells in all tumors, including MPNST, neurofibromas and schwannomas.
  • The WT1 expression is independent of tumor malignancy or tumor growth extension and is not associated with a neurocutaneous disorder.
  • CONCLUSION: WT1 expression in normal and neoplastic tissue differs in the peripheral and the central nervous system.
  • [MeSH-major] Cranial Nerve Neoplasms / metabolism. Cranial Nerves / metabolism. Nerve Sheath Neoplasms / metabolism. Peripheral Nerves / metabolism. WT1 Proteins / metabolism
  • [MeSH-minor] Adolescent. Adult. Aged. Case-Control Studies. Child. Female. Humans. Male. Middle Aged. Neurilemmoma / metabolism. Neurofibroma / metabolism. Young Adult

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  • (PMID = 21178265.001).
  • [ISSN] 1875-8592
  • [Journal-full-title] Cancer biomarkers : section A of Disease markers
  • [ISO-abbreviation] Cancer Biomark
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article
  • [Publication-country] Netherlands
  • [Chemical-registry-number] 0 / WT1 Proteins
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58. 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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59. 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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60. Hummel TR, Jessen WJ, Miller SJ, Kluwe L, Mautner VF, Wallace MR, Lázaro C, Page GP, Worley PF, Aronow BJ, Schorry EK, Ratner N: Gene expression analysis identifies potential biomarkers of neurofibromatosis type 1 including adrenomedullin. Clin Cancer Res; 2010 Oct 15;16(20):5048-57
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  • pNF can undergo transformation to malignant peripheral nerve sheath tumors (MPNST).
  • There are no identified serum biomarkers of pNF tumor burden or transformation to MPNST.
  • Serum biomarkers would be useful to verify NF1 diagnosis, monitor tumor burden, and/or detect transformation.
  • EXPERIMENTAL DESIGN: We used microarray gene expression analysis to define 92 genes that encode putative secreted proteins in neurofibroma Schwann cells, neurofibromas, and MPNST.
  • ADM protein concentrati on was further elevated in serum of a small sampling of NF1 patients with MPNST.
  • MPNST cell conditioned medium, containing ADM and hepatocyte growth factor, stimulated MPNST migration and endothelial cell proliferation.
  • ADM serum levels do not seem to correlate with the presence of pNFs but may be a biomarker of transformation to MPNST.

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  • [Copyright] ©2010 AACR.
  • (PMID = 20739432.001).
  • [ISSN] 1078-0432
  • [Journal-full-title] Clinical cancer research : an official journal of the American Association for Cancer Research
  • [ISO-abbreviation] Clin. Cancer Res.
  • [Language] ENG
  • [Grant] United States / NINDS NIH HHS / NS / K01 NS049191; United States / NINDS NIH HHS / NS / R01 NS028840; United States / NINDS NIH HHS / NS / K01-NS049191
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, Non-P.H.S.
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Culture Media; 0 / RNA, Messenger; 148498-78-6 / Adrenomedullin
  • [Other-IDs] NLM/ NIHMS763673; NLM/ PMC4837895
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61. 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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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [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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62. 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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63. Gudena V, Verma N, Post G, Kizziah M, Fenning R, Montero AJ: Metastatic chest wall malignant schwannoma responding to sorafenib: case report and literature review. Cancer Biol Ther; 2008 Jun;7(6):810-3
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  • [Title] Metastatic chest wall malignant schwannoma responding to sorafenib: case report and literature review.
  • Malignant schwannomas or malignant peripheral nerve sheath tumors (MPNST) represent approximately 10% of all soft tissue sarcomas.
  • Metastatic disease from chest wall MPNST is very rare.
  • We present a case of a major clinical response to the tyrosine kinase inhibitor (TKI) sorafenib in a patient with metastatic MPNST.
  • A 42-year-old female with a prior history of neurofibromas developed MPNST, which later metastasized to the lungs and brain.
  • MPNST show high levels of Ras activity and hence these tumors are promising targets for TKIs.
  • [MeSH-major] Antineoplastic Agents / therapeutic use. Benzenesulfonates / therapeutic use. Nerve Sheath Neoplasms / diagnosis. Nerve Sheath Neoplasms / drug therapy. Neurilemmoma / diagnosis. Neurilemmoma / drug therapy. Pyridines / therapeutic use. Thoracic Wall / pathology
  • [MeSH-minor] Adult. Brain Neoplasms / drug therapy. Brain Neoplasms / secondary. Female. Humans. Lung Neoplasms / drug therapy. Lung Neoplasms / secondary. Magnetic Resonance Imaging. Neoplasm Metastasis. Niacinamide / analogs & derivatives. Phenylurea Compounds. Protein Kinase Inhibitors / therapeutic use. Radiography, Thoracic. Treatment Outcome

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  • (PMID = 18376142.001).
  • [ISSN] 1555-8576
  • [Journal-full-title] Cancer biology & therapy
  • [ISO-abbreviation] Cancer Biol. Ther.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Benzenesulfonates; 0 / Phenylurea Compounds; 0 / Protein Kinase Inhibitors; 0 / Pyridines; 25X51I8RD4 / Niacinamide; 9ZOQ3TZI87 / sorafenib
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64. 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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65. Nabeshima K, Iwasaki H, Nishio J, Koga K, Shishime M, Kikuchi M: Expression of emmprin and matrix metalloproteinases (MMPs) in peripheral nerve sheath tumors: emmprin and membrane-type (MT)1-MMP expressions are associated with malignant potential. Anticancer Res; 2006 Mar-Apr;26(2B):1359-67
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  • [Title] Expression of emmprin and matrix metalloproteinases (MMPs) in peripheral nerve sheath tumors: emmprin and membrane-type (MT)1-MMP expressions are associated with malignant potential.
  • However, their roles in peripheral nerve sheath tumors (PNSTs) have rarely been investigated.
  • MATERIALS AND METHODS: In this study, the immunohistochemical expression of 6 MMPs, their 3 inhibitors and emmprin, an MMP inducer, was examined in 14 schwannomas, 14 neurofibromas and 12 malignant peripheral nerve sheath tumors (MPNSTs) in relation to malignant potentials.
  • Moreover, the expression patterns of MMP-1 and gelatinase B (MMP-9) could divide PNSTs into two groups: schwannoma versus neurofibroma/MPNST.
  • CONCLUSION: These results suggest that emmprin and MT1-MMP may be malignant potential-related proteins in PNSTs, and that MMP-1 and 9 may help differentiation between schwannoma and neurofibroma, especially in their plexiform types.
  • [MeSH-major] Antigens, CD147 / biosynthesis. Matrix Metalloproteinases / biosynthesis. Nerve Sheath Neoplasms / metabolism
  • [MeSH-minor] Adolescent. Adult. Aged. Aged, 80 and over. Child. Female. Humans. Immunohistochemistry. Isoenzymes / biosynthesis. Male. Matrix Metalloproteinases, Membrane-Associated. Middle Aged. Neurilemmoma / enzymology. Neurilemmoma / metabolism. Neurilemmoma / pathology. Neurofibroma / enzymology. Neurofibroma / metabolism. Neurofibroma / pathology

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  • (PMID = 16619545.001).
  • [ISSN] 0250-7005
  • [Journal-full-title] Anticancer research
  • [ISO-abbreviation] Anticancer Res.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Greece
  • [Chemical-registry-number] 0 / BSG protein, human; 0 / Isoenzymes; 136894-56-9 / Antigens, CD147; EC 3.4.24.- / Matrix Metalloproteinases; EC 3.4.24.- / Matrix Metalloproteinases, Membrane-Associated
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66. 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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67. Di Giovannantonio L, Bellocci R, Zappacosta R, Zappacosta B, Castrataro A, Liberatore M, Liberati M, Angelucci D: [Primary malignant schwannoma of the uterine cervix: a malignant tumor with unusual behaviour. A case report]. Pathologica; 2005 Feb;97(1):7-9
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  • [Title] [Primary malignant schwannoma of the uterine cervix: a malignant tumor with unusual behaviour. A case report].
  • [Transliterated title] Schwannoma maligno primitivo della cervice uterina: un tumore maligno a comportamento inusuale. Caso clinico.
  • Malignant tumors of peripheral nerves (MPNST) represent approximately 5-10% of all soft tissue sarcomas and usually are in relationship with a major nerve.
  • Primary cervical malignant Schwannomas are very rare neural sheat tumors that, grossly and clinically, are misdiagnosed for other more frequent lesions of the uterine cervix.
  • We report a case of primary cervical malignant Schwannoma in a 27 years old female with atypical bleeding.
  • [MeSH-major] Neurilemmoma / pathology. Uterine Cervical Neoplasms / pathology
  • [MeSH-minor] Adult. Female. Humans


68. Zambrana F, Vicente F, García-Manrique T, Pereira S, Sáinz De Zaitigui J, De La Cruz Merino L: Primary intracranial malignant peripheral nerve sheath tumour responding to chemotherapy. Clin Transl Oncol; 2010 Mar;12(3):231-3
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  • [Title] Primary intracranial malignant peripheral nerve sheath tumour responding to chemotherapy.
  • Malignant peripheral nerve sheath tumours (MPNST) are a rare variety of soft tissue sarcomas (STS) arising from major peripheral nerve branches and typically located in the lower extremity, chest wall or the retroperitoneum.
  • It is a biologically aggressive neoplasm for which the treatment of choice is surgery, but usually requires a multimodality approach, having been generally labelled as chemoresistant.
  • We present a case of MPNST located intracranially with a good response to chemotherapy.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Brain Neoplasms / drug therapy. Brain Neoplasms / pathology. Nerve Sheath Neoplasms / drug therapy
  • [MeSH-minor] Adult. Doxorubicin / administration & dosage. Humans. Ifosfamide / administration & dosage. Male

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  • (PMID = 20231129.001).
  • [ISSN] 1699-3055
  • [Journal-full-title] Clinical & translational oncology : official publication of the Federation of Spanish Oncology Societies and of the National Cancer Institute of Mexico
  • [ISO-abbreviation] Clin Transl Oncol
  • [Language] eng
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  • [Chemical-registry-number] 80168379AG / Doxorubicin; UM20QQM95Y / Ifosfamide
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69. 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 Protein p73. 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 Protein p73; 0 / Tumor Suppressor Protein p53; 0 / Tumor Suppressor Proteins; 0 / Vascular Endothelial Growth Factor A; EC 2.7.10.1 / Receptor, Epidermal Growth Factor
  • [Number-of-references] 80
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70. Dang I, Nelson JK, DeVries GH: c-Kit receptor expression in normal human Schwann cells and Schwann cell lines derived from neurofibromatosis type 1 tumors. J Neurosci Res; 2005 Nov 15;82(4):465-71
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  • Its role in Schwann cells has been described in transformed cells derived from malignant peripheral nerve sheath tumors from patients with neurofibromatosis type 1 (NF1 MPNST; Badache et al. [1998] Oncogene 17:795-800).
  • We report here that neonatal rat Schwann cells express low c-Kit levels, whereas expression levels for c-Kit are high for Schwann cells derived from MPNST of NF1 patients.
  • In addition, c-Kit expression is not detectable in normal adult human Schwann cells.

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  • [Copyright] Copyright 2005 Wiley-Liss, Inc.
  • (PMID = 16235251.001).
  • [ISSN] 0360-4012
  • [Journal-full-title] Journal of neuroscience research
  • [ISO-abbreviation] J. Neurosci. Res.
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article; Research Support, U.S. Gov't, Non-P.H.S.
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Chromones; 0 / Enzyme Inhibitors; 0 / Morpholines; 0 / Neuregulin-1; 0 / Neurofibromin 1; 0 / RNA, Messenger; 154447-36-6 / 2-(4-morpholinyl)-8-phenyl-4H-1-benzopyran-4-one; EC 2.7.10.1 / Proto-Oncogene Proteins c-kit; EC 2.7.11.1 / Proto-Oncogene Proteins c-akt; G34N38R2N1 / Bromodeoxyuridine
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71. Melean G, Hernández AM, Valero MC, Hernández-Imaz E, Martín Y, Hernández-Chico C: Monozygotic twins with Neurofibromatosis type 1, concordant phenotype and synchronous development of MPNST and metastasis. BMC Cancer; 2010;10:407
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  • [Title] Monozygotic twins with Neurofibromatosis type 1, concordant phenotype and synchronous development of MPNST and metastasis.
  • The condition predisposes individuals to the development of malignant nervous system tumours, most frequently Malignant Peripheral Nerve Sheath Tumours (MPNSTs).
  • Both twins developed a left sciatic plexiform neurofibroma that evolved into MPNST at a similar age and they also developed pulmonary metastasis at the same age.
  • [MeSH-major] Lung Neoplasms / secondary. Nerve Sheath Neoplasms / pathology. Neurofibromatosis 1 / pathology. Twins, Monozygotic
  • [MeSH-minor] Adult. Genotype. Humans. Male. Mutation / genetics. Neurofibromin 1 / genetics. Phenotype. Polymerase Chain Reaction. Prognosis

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  • (PMID = 20687928.001).
  • [ISSN] 1471-2407
  • [Journal-full-title] BMC cancer
  • [ISO-abbreviation] BMC Cancer
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Neurofibromin 1
  • [Other-IDs] NLM/ PMC2924852
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72. Bensaid B, Giammarile F, Mognetti T, Galoisy-Guibal L, Pinson S, Drouet A, Combemale P: [Utility of 18 FDG positon emission tomography in detection of sarcomatous transformation in neurofibromatosis type 1]. Ann Dermatol Venereol; 2007 Oct;134(10 Pt 1):735-41
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • BACKGROUND: The appearance of malignant peripheral nerve sheath tumours (MPNST) marks a critical stage in the course of neurofibromatosis type 1 (NF1).
  • We assessed the value of 18 FDG positron emission tomoscintigraphy (PET scan) in screening for such malignant tumours.
  • PATIENTS AND METHODS: Between October 2000 and August 2006, all of our patients with NF1 and suspected MPNST underwent PET scan.
  • In 8 patients, PET scan showed suspect lesions (12 tumours), and histological analysis of these tumours revealed 6 MPNST.
  • In 30 patients (37 tumours) PET scan showed non-suspect binding, and no malignant tumours were demonstrated either on histological examination or after mean follow-up of 33.5 months.
  • DISCUSSION: Our study, to our knowledge the most extensive yet performed, demonstrates the value of PET scan in detecting MPNST, particularly based on its 100% negative predictive value.
  • The first involved 18 NF1 patients with 23 plexiform neurofibromas: of 7 tumours with hyperbinding, 5 were MPNST.
  • The second study concerned 5 NF1 patients with a total of 15 tumours: 7 tumours showed hyperbinding, of which 6 were MPNST.
  • [MeSH-major] Fluorodeoxyglucose F18. Neurofibromatosis 1 / complications. Positron-Emission Tomography. Radiopharmaceuticals. Sarcoma / radionuclide imaging
  • [MeSH-minor] Adolescent. Adult. Aged. Cell Transformation, Neoplastic. Child. Decision Trees. Female. Humans. Male. Middle Aged

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  • (PMID = 17978710.001).
  • [ISSN] 0151-9638
  • [Journal-full-title] Annales de dermatologie et de vénéréologie
  • [ISO-abbreviation] Ann Dermatol Venereol
  • [Language] fre
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] France
  • [Chemical-registry-number] 0 / Radiopharmaceuticals; 0Z5B2CJX4D / Fluorodeoxyglucose F18
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73. Li H, Zhang X, Fishbein L, Kweh F, Campbell-Thompson M, Perrin GQ, Muir D, Wallace M: Analysis of steroid hormone effects on xenografted human NF1 tumor schwann cells. Cancer Biol Ther; 2010 Oct 15;10(8):758-64
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  • [Title] Analysis of steroid hormone effects on xenografted human NF1 tumor schwann cells.
  • The neurofibroma, a common feature of neurofibromatosis type 1 (NF1), is a benign peripheral nerve sheath tumor that contains predominantly Schwann cells (SC).
  • This study examined the effects of estrogen and progesterone on proliferation and apoptosis in a panel of NF1 tumor xenografts.
  • SC-enriched cultures derived from three human NF1 tumor types (dermal neurofibroma, plexiform neurofibroma, and malignant peripheral nerve sheath tumor (MPNST)) were xenografted in sciatic nerves of ovariectomized scid /Nf1-/+ mice.
  • Estrogen was found to increase the growth of all three MPNST xenografts.

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  • (PMID = 20699653.001).
  • [ISSN] 1555-8576
  • [Journal-full-title] Cancer biology & therapy
  • [ISO-abbreviation] Cancer Biol. Ther.
  • [Language] ENG
  • [Grant] United States / NINDS NIH HHS / NS / F30 NS043951; United States / NINDS NIH HHS / NS / 1F30NS43951
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, Non-P.H.S.
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Drug Implants; 0 / Estrogens; 0 / Ki-67 Antigen; 4G7DS2Q64Y / Progesterone
  • [Other-IDs] NLM/ PMC3093914
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74. 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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75. Mautner VF, Asuagbor FA, Dombi E, Fünsterer C, Kluwe L, Wenzel R, Widemann BC, Friedman JM: Assessment of benign tumor burden by whole-body MRI in patients with neurofibromatosis 1. Neuro Oncol; 2008 Aug;10(4):593-8
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  • [Title] Assessment of benign tumor burden by whole-body MRI in patients with neurofibromatosis 1.
  • People with neurofibromatosis 1 (NF1) have multiple benign neurofibromas and a 10% lifetime risk of developing malignant peripheral nerve sheath tumors (MPNSTs).
  • Most MPNSTs develop from benign plexiform neurofibromas, so the burden of benign tumors may be a risk factor for developing MPNST.
  • Both the median number of plexiform neurofibromas (p < 0.05) and the median neurofibroma volume (p < 0.01) on whole-body MRI were significantly greater among MPNST patients younger than 30 years of age than among controls.
  • Whole-body imaging of young NF1 patients may allow those at highest risk for developing MPNST to be identified early in life.
  • [MeSH-major] Magnetic Resonance Imaging. Neurofibromatosis 1 / pathology. Tumor Burden. Whole Body Imaging
  • [MeSH-minor] Adolescent. Adult. Child. Child, Preschool. Female. Humans. Infant. Male. Middle Aged

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  • (PMID = 18559970.001).
  • [ISSN] 1522-8517
  • [Journal-full-title] Neuro-oncology
  • [ISO-abbreviation] Neuro-oncology
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Other-IDs] NLM/ PMC2666233
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76. 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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  • [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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77. 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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78. Upadhyaya M, Spurlock G, Monem B, Thomas N, Friedrich RE, Kluwe L, Mautner V: Germline and somatic NF1 gene mutations in plexiform neurofibromas. Hum Mutat; 2008 Aug;29(8):E103-11
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Plexiform neurofibroma (PNF) is a congenital benign tumour present in 30-50% of NF1 patients, which in about 10-15% of cases, can develop into a malignant peripheral nerve sheath tumour (MPNST).
  • LOH of the NF1 gene region, as identified by marker analysis and/or MLPA, was detected in only 20/29 (69%) PNFs, compared to the >90% LOH previously found in MPNST.
  • [MeSH-minor] Adolescent. Adult. Child. DNA Mutational Analysis. Genes, p53. Humans. Loss of Heterozygosity. Male. Microsatellite Repeats. Middle Aged. Tumor Suppressor Protein p53 / genetics

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  • (PMID = 18484666.001).
  • [ISSN] 1098-1004
  • [Journal-full-title] Human mutation
  • [ISO-abbreviation] Hum. Mutat.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Neurofibromin 1; 0 / TP53 protein, human; 0 / Tumor Suppressor Protein p53
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79. Siqueira MG, Martins RS, Teixeira MJ: Management of brachial plexus region tumours and tumour-like conditions: relevant diagnostic and surgical features in a consecutive series of eighteen patients. Acta Neurochir (Wien); 2009 Sep;151(9):1089-98
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • FINDINGS: The tumours comprised a heterogeneous group of lesions, including schwannomas, neurofibromas, malignant peripheral nerve sheath tumour (MPNST), sarcomas, metastases, desmoids and an aneurysmal bone cyst.
  • Eleven tumours were benign and 7 were malignant.
  • Some of the malignant tumours could be controlled by surgery plus adjuvant therapy, but this category is still associated with high morbidity and mortality rates.
  • [MeSH-major] Brachial Plexus / pathology. Brachial Plexus / surgery. Brachial Plexus Neuropathies / diagnosis. Brachial Plexus Neuropathies / surgery. Peripheral Nervous System Neoplasms / diagnosis. Peripheral Nervous System Neoplasms / surgery
  • [MeSH-minor] Adolescent. Adult. Bone Cysts, Aneurysmal / diagnosis. Bone Cysts, Aneurysmal / pathology. Bone Cysts, Aneurysmal / surgery. Child. Female. Fibromatosis, Aggressive / diagnosis. Fibromatosis, Aggressive / physiopathology. Fibromatosis, Aggressive / surgery. Humans. Magnetic Resonance Imaging. Male. Middle Aged. Neoplasm Metastasis / diagnosis. Neoplasm Metastasis / physiopathology. Neoplasm Recurrence, Local / epidemiology. Nerve Sheath Neoplasms / diagnosis. Nerve Sheath Neoplasms / physiopathology. Nerve Sheath Neoplasms / surgery. Neurilemmoma / diagnosis. Neurilemmoma / physiopathology. Neurilemmoma / surgery. Neurofibroma / diagnosis. Neurofibroma / physiopathology. Neurofibroma / surgery. Neurosurgical Procedures. Pain / etiology. Paresthesia / etiology. Postoperative Complications / epidemiology. Retrospective Studies. Sarcoma / diagnosis. Sarcoma / physiopathology. Sarcoma / surgery. Tomography, X-Ray Computed. Treatment Outcome. Young Adult

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  • (PMID = 19448970.001).
  • [ISSN] 0942-0940
  • [Journal-full-title] Acta neurochirurgica
  • [ISO-abbreviation] Acta Neurochir (Wien)
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Austria
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80. 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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81. Okoshi A, Shiga K, Sasaki K, Asada Y, Nishikawa H, Kobayashi T, Watanabe M: [Two cases of radiation-induced sarcoma after radiation therapy in nasopharyngeal carcinoma]. Nihon Jibiinkoka Gakkai Kaiho; 2008 Jul;111(7):533-6
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  • [Title] [Two cases of radiation-induced sarcoma after radiation therapy in nasopharyngeal carcinoma].
  • We report two cases of radiation-induced sarcoma after chemoradiation therapy in nasopharyngeal carcinoma.
  • Case 1: A 40-year-old man developed a malignant peripheral nerve sheath tumor (MPNST) in the posterior floor of the nasal cavity 10 years after treatment for nasopharyngeal cancer.
  • Case 2: A 64 year-old man developed a malignant fibrous histiocytoma (MFH) of the lower gum 10 years after treatment of nasopharyngeal cancer.
  • Despite radical surgery, the man with MPNST had a recurrent tumor and died of the disease.
  • [MeSH-major] Histiocytoma, Malignant Fibrous / etiology. Nasopharyngeal Neoplasms / etiology. Neoplasms, Radiation-Induced / etiology. Nerve Sheath Neoplasms / etiology. Radiotherapy / adverse effects
  • [MeSH-minor] Adult. Antineoplastic Combined Chemotherapy Protocols / administration & dosage. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Carcinoma, Squamous Cell / therapy. Cisplatin / administration & dosage. Cisplatin / therapeutic use. Combined Modality Therapy. Fatal Outcome. Fluorouracil / administration & dosage. Humans. Male. Middle Aged. Radiotherapy Dosage. Treatment Outcome

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  • (PMID = 18697477.001).
  • [ISSN] 0030-6622
  • [Journal-full-title] Nihon Jibiinkoka Gakkai kaiho
  • [ISO-abbreviation] Nippon Jibiinkoka Gakkai Kaiho
  • [Language] jpn
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Japan
  • [Chemical-registry-number] Q20Q21Q62J / Cisplatin; U3P01618RT / Fluorouracil; CF regimen
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82. Nepka C, Karadana M, Karasavvidou F, Barbanis S, Kalodimos G, Koukoulis G: Fine needle aspiration cytology of a primary malignant peripheral nerve sheath tumor arising in the parotid gland: a case report. Acta Cytol; 2009 Jul-Aug;53(4):423-6
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  • [Title] Fine needle aspiration cytology of a primary malignant peripheral nerve sheath tumor arising in the parotid gland: a case report.
  • BACKGROUND: Malignant peripheral nerve sheath tumor (MPNST) is an uncommon mesenchymal neoplasm showing nerve sheath differentiation, usually arising in large nerves of the trunk and extremities.
  • We describe fine needle aspiration (FNA) findings in a case of MPNST in the parotid gland.
  • Smears were cellular, with clusters of tightly packed spindle or oval cells arranged in a storiform or whorled pattern, showing clearly malignant features.
  • The background contained abundant necrotic material with dispersed malignant nuclei.
  • Cytologic diagnosis was positive for malignant cells consistent with a spindle cell sarcoma, with morphologic features compatible to neural differentiation, confirmed by histologic examination.
  • CONCLUSION: This case illustrates that attention to moiphologic criteria suggestive of nerve sheath phenotype supported by immunocytochemical data is extremely helpful and reliable in the diagnostic approach to MPNSTs, even in rare locations.
  • [MeSH-major] Biopsy, Fine-Needle. Nerve Sheath Neoplasms / pathology. Parotid Neoplasms / pathology
  • [MeSH-minor] Adult. Diagnosis, Differential. Humans. Male

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  • [CommentIn] Acta Cytol. 2010 Sep-Oct;54(5 Suppl):1073-4 [21053609.001]
  • (PMID = 19697728.001).
  • [ISSN] 0001-5547
  • [Journal-full-title] Acta cytologica
  • [ISO-abbreviation] Acta Cytol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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83. Prayson RA, Yoder BJ, Barnett GH: Epidermal growth factor receptor is not amplified in schwannomas. Ann Diagn Pathol; 2007 Oct;11(5):326-9
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  • Both neurofibromas and schwannomas are capable of progression to malignant peripheral nerve sheath tumors (MPNSTs).
  • The mitogenic signaling for schwannomas is unlikely to be related to overexpression or amplification of EGFR; however, acquiring this signaling pathway might contribute to the progression of a subset of benign peripheral nerve sheath tumors to MPNST.
  • [MeSH-major] Nerve Sheath Neoplasms / metabolism. Neurilemmoma / metabolism. Receptor, Epidermal Growth Factor / metabolism
  • [MeSH-minor] Adult. Aged. DNA, Neoplasm / genetics. Disease Progression. Female. Gene Amplification. Gene Expression Regulation, Neoplastic. Humans. Immunohistochemistry. In Situ Hybridization, Fluorescence. Male. Middle Aged

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  • (PMID = 17870017.001).
  • [ISSN] 1092-9134
  • [Journal-full-title] Annals of diagnostic pathology
  • [ISO-abbreviation] Ann Diagn Pathol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / DNA, Neoplasm; EC 2.7.10.1 / Receptor, Epidermal Growth Factor
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84. Wasa J, Nishida Y, Suzuki Y, Tsukushi S, Shido Y, Hosono K, Shimoyama Y, Nakamura S, Ishiguro N: Differential expression of angiogenic factors in peripheral nerve sheath tumors. Clin Exp Metastasis; 2008;25(7):819-25
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  • [Title] Differential expression of angiogenic factors in peripheral nerve sheath tumors.
  • It is difficult to differentiate some malignant peripheral nerve sheath tumors (MPNST) from benign peripheral nerve sheath tumors (BPNST) histologically, and to predict the clinical outcome of patients with MPNST.
  • In this study, the expression of VEGF and MVD were evaluated immunohistochemically in 22 cases of MPNST, 14 of neurofibroma and 19 of schwannoma and correlation of the staining grade of VEGF or MVD and the various clinical factors were analyzed, and statistically evaluated.
  • Statistically higher positive staining for VEGF was observed in MPNST compared to neurofibroma (P=0.004) and schwannoma (P<0.001).
  • Even low grade MPNST showed higher VEGF positive staining than neurofibroma.
  • Moreover, high VEGF expression statistically correlated with the poor prognosis of the patients with MPNST (P=0.015).
  • Although MVD in MPNST was significantly higher than that in neurofibroma (P=0.038) and schwannoma (P<0.001), MVD could not predict the prognosis of the patients with MPNST.
  • Although VEGF mRNA expression tended to be higher in MPNST compared to neurofibroma, the difference was not significant.
  • Levels of VEGF protein expression serve as a novel diagnostic and prognostic tools for peripheral nerve sheath tumors.
  • [MeSH-major] Nerve Sheath Neoplasms / chemistry. Vascular Endothelial Growth Factor A / analysis
  • [MeSH-minor] Adolescent. Adult. Aged. Aged, 80 and over. Child. Female. Humans. Immunohistochemistry. Male. Middle Aged. Neurilemmoma / blood supply. Neurilemmoma / chemistry. Neurofibroma / blood supply. Neurofibroma / chemistry. RNA, Messenger / analysis

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  • (PMID = 18679811.001).
  • [ISSN] 0262-0898
  • [Journal-full-title] Clinical & experimental metastasis
  • [ISO-abbreviation] Clin. Exp. Metastasis
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Netherlands
  • [Chemical-registry-number] 0 / RNA, Messenger; 0 / Vascular Endothelial Growth Factor A
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85. 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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86. Aron M, Kapila K, Verma K: Neural tumours of the neck presenting as thyroid nodules: a report of three cases. Cytopathology; 2005 Aug;16(4):206-9
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • We report two cases of schwannoma and one case of a malignant peripheral nerve sheath tumour (MPNST) of the neck, which presented as thyroid nodules and evaluate the role of cytology in identifying these tumours.
  • RESULTS: Both the cases of schwannoma were correctly identified on cytology while the case of MPNST could only be typed as a spindle cell tumour.
  • CONCLUSIONS: Schwannomas of the neck are easily identifiable on cytology compared with MPNST.
  • [MeSH-major] Head and Neck Neoplasms / diagnosis. Nerve Sheath Neoplasms / diagnosis. Neurilemmoma / diagnosis. Thyroid Gland / pathology
  • [MeSH-minor] Adult. Cytodiagnosis. Diagnosis, Differential. Female. Goiter, Nodular / diagnosis. Humans. Male

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  • (PMID = 16048507.001).
  • [ISSN] 0956-5507
  • [Journal-full-title] Cytopathology : official journal of the British Society for Clinical Cytology
  • [ISO-abbreviation] Cytopathology
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
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87. 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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88. 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
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  • [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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89. Shah H, Pervez S: Immunophenotypic characterization of high grade pleomorphic sarcomas: a demographic and immunohistochemical study in a major referral center of Pakistan. J Pak Med Assoc; 2005 Mar;55(3):101-4
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  • RESULTS: Of the 134 cases which were characterized, 38.1% were pleomorphic leiomyosarcoma, followed by pleomorphic rhabdomyosarcoma 14.9%, Malignant Peripheral Nerve Sheath Tumour 9%, pleomorphic liposarcoma 3.7% and pleomorphic storiform Malignant Fibrous Histiocytoma 0.7%.
  • Mean/ median age for Leiomyosarcoma was 50/50, for Rhabdomyosarcomas 33/22, for MPNST 42/41, for Liposarcoma 52/50 and for Malignant Fibrous Histiocytoma 46/46 respectively.
  • CONCLUSION: It was concluded that the most common pleomorphic sarcoma occurring in our adult population was Leiomyosarcoma, and that immunohistochemical stains are essential in most cases for further characterization of pleomorphic high grade sarcoma.
  • [MeSH-major] Sarcoma / pathology. Soft Tissue Neoplasms / pathology
  • [MeSH-minor] Adult. Age Distribution. Aged. Antigens, Neoplasm / immunology. Biomarkers, Tumor / analysis. Cross-Sectional Studies. Humans. Immunoenzyme Techniques. Immunohistochemistry. Leiomyosarcoma / pathology. Liposarcoma / genetics. Liposarcoma / pathology. Middle Aged. Nerve Sheath Neoplasms / genetics. Nerve Sheath Neoplasms / pathology. Pakistan. Phenotype. Rhabdomyosarcoma / genetics. Rhabdomyosarcoma / pathology. Surveys and Questionnaires

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  • (PMID = 15852744.001).
  • [ISSN] 0030-9982
  • [Journal-full-title] JPMA. The Journal of the Pakistan Medical Association
  • [ISO-abbreviation] J Pak Med Assoc
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Pakistan
  • [Chemical-registry-number] 0 / Antigens, Neoplasm; 0 / Biomarkers, Tumor
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90. 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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91. Oda Y, Saito T, Tateishi N, Ohishi Y, Tamiya S, Yamamoto H, Yokoyama R, Uchiumi T, Iwamoto Y, Kuwano M, Tsuneyoshi M: ATP-binding cassette superfamily transporter gene expression in human soft tissue sarcomas. Int J Cancer; 2005 May 10;114(6):854-62
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  • The phenomenon of multidrug resistance (MDR) in various malignant neoplasms has been reported as being caused by one or multiple expressions of ATP-binding cassette (ABC) superfamily protein, including P-glycoprotein/multidrug resistance (MDR) 1 and the MDR protein (MRP) family.
  • In 86 cases of surgically resected soft tissue sarcoma, intrinsic mRNA levels of MDR1, MRP1, MRP2 and MRP3 were assessed using a quantitative reverse transcriptase-PCR (RT-PCR) method.
  • Among the various histologic types, malignant peripheral nerve sheath tumor (MPNST) showed significantly high levels of MDR1 (p=0.017) and MRP3 (p=0.0384) mRNA expression, compared to the other tumor types.
  • P-gp expression was significantly correlated with large tumor size (> or =5 cm, p=0.041) and high AJCC stage (stages III and IV) (p=0.0365).
  • Our results suggest that MDR1/P-gp expression may have an important role to play in tumor progression in the cases of soft tissue sarcoma, and p53 may be one of the active regulators of the MDR1 transcript.
  • In addition, the high levels of both MDR1 and MRP3 mRNA expression in MPNST may help to explain the poor response of this tumor to anticancer-drugs.
  • [MeSH-major] ATP-Binding Cassette Transporters / biosynthesis. ATP-Binding Cassette Transporters / genetics. Drug Resistance, Multiple. Gene Expression Regulation, Neoplastic. Genes, p53. Sarcoma / drug therapy. Sarcoma / genetics
  • [MeSH-minor] Adolescent. Adult. Disease Progression. Drug Resistance, Neoplasm / genetics. Female. Gene Expression Profiling. Humans. Immunohistochemistry. Male. Reverse Transcriptase Polymerase Chain Reaction

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  • (PMID = 15609299.001).
  • [ISSN] 0020-7136
  • [Journal-full-title] International journal of cancer
  • [ISO-abbreviation] Int. J. Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / ATP-Binding Cassette Transporters
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92. Agaram NP, Prakash S, Antonescu CR: Deep-seated plexiform schwannoma: a pathologic study of 16 cases and comparative analysis with the superficial variety. Am J Surg Pathol; 2005 Aug;29(8):1042-8
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  • [Title] Deep-seated plexiform schwannoma: a pathologic study of 16 cases and comparative analysis with the superficial variety.
  • Plexiform schwannoma (PS) is one of the least common histologic variants of schwannoma.
  • However, the frequent cellular morphology associated with hyperchromatic nuclei, increased mitoses, and plexiform growth can suggest a malignant process, mainly a high-grade malignant peripheral nerve sheath tumor (MPNST).
  • Fifteen tumors were located in the deep somatic soft tissue (extremities, 8; retroperitoneum/pelvis, 3; trunk, 2; parotid, 1; vulva, 1) and 1 tumor was located in the thoracic esophagus.
  • The 8 superficial PSs showed increased cellularity and mild to moderate pleomorphism in 62% of cases but lacked tumor necrosis.
  • [MeSH-major] Neurilemmoma / pathology. Soft Tissue Neoplasms / pathology
  • [MeSH-minor] Adolescent. Adult. Child, Preschool. Female. Humans. Immunohistochemistry. Infant. Male. Middle Aged. S100 Proteins / analysis

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  • (PMID = 16006798.001).
  • [ISSN] 0147-5185
  • [Journal-full-title] The American journal of surgical pathology
  • [ISO-abbreviation] Am. J. Surg. Pathol.
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / S100 Proteins
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93. 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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94. 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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95. 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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  • [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] United States
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96. 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
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  • [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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97. Jokinen CH, Argenyi ZB: Atypical neurofibroma of the skin and subcutaneous tissue: clinicopathologic analysis of 11 cases. J Cutan Pathol; 2010 Jan;37(1):35-42
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • BACKGROUND: Neurofibroma (NF) is a relatively common cutaneous tumor, which typically presents little diagnostic difficulty.
  • Occasionally, however, pleomorphic cells may be present in NF raising consideration of other neoplasms like malignant peripheral nerve sheath tumor (MPNST).
  • Some tumors were hypercellular, but marked density characteristic of MPNST was not observed.
  • Of six patients with available follow-up, no tumor recurred and none developed malignancy (range 6-63, mean 33 months).
  • CONCLUSIONS: Superficial atypical NF, while morphologically unusual, has no apparent association with neurofibromatosis-1 or short-term risk of recurrence or malignant transformation.
  • Awareness of this variant is important in order to avoid misdiagnosis of a more aggressive neoplasm.
  • [MeSH-minor] Adult. Aged. Biomarkers, Tumor / metabolism. Child. Diagnosis, Differential. Disease-Free Survival. Female. Humans. Male. Middle Aged. Neoplasm Recurrence, Local. Nerve Sheath Neoplasms / pathology. S100 Proteins / metabolism. Young Adult

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  • [Copyright] Copyright © 2009 John Wiley & Sons A/S.
  • (PMID = 19469864.001).
  • [ISSN] 1600-0560
  • [Journal-full-title] Journal of cutaneous pathology
  • [ISO-abbreviation] J. Cutan. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Denmark
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / S100 Proteins
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98. 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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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [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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99. 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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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Malignant peripheral nerve sheath tumors: prognostic factors and survival in a series of patients treated at a single institution.
  • BACKGROUND: The authors explored the prognostic factors and clinical outcomes of patients who had malignant peripheral nerve sheath tumors (MPNST) with and without neurofibromatosis type 1 (NF-1).
  • METHODS: Two hundred five patients with localized MPNST who underwent surgery at the Istituto Nazionale per lo Studio e la Cura dei Tumori (Milan, Italy) over 25 years were reviewed.
  • Presentation with either primary or recurrent disease, tumor size, and tumor site (trunk vs. extremity) were the strongest independent predictors of survival.
  • The results confirmed that patients with MPNST share similar prognostic factors with patients who have other soft tissue sarcomas and have some of the worst clinical outcomes.
  • [MeSH-major] Nerve Sheath Neoplasms / mortality. Neurofibromatosis 1 / complications
  • [MeSH-minor] Adolescent. Adult. Combined Modality Therapy. Disease-Free Survival. Female. Humans. Male. Neoplasm Metastasis. Neoplasm Recurrence, Local. Prognosis. Survival Rate

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  • (PMID = 16881077.001).
  • [ISSN] 0008-543X
  • [Journal-full-title] Cancer
  • [ISO-abbreviation] Cancer
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article
  • [Publication-country] United States
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100. 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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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [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






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