nerve sheath tumors

Summary

Summary: Neoplasms which arise from nerve sheaths formed by SCHWANN CELLS in the PERIPHERAL NERVOUS SYSTEM or by OLIGODENDROCYTES in the CENTRAL NERVOUS SYSTEM. Malignant peripheral nerve sheath tumors, NEUROFIBROMA, and NEURILEMMOMA are relatively common tumors in this category.

Top Publications

  1. Stucky C, Johnson K, Gray R, Pockaj B, Ocal I, Rose P, et al. Malignant peripheral nerve sheath tumors (MPNST): the Mayo Clinic experience. Ann Surg Oncol. 2012;19:878-85 pubmed publisher
    Malignant peripheral nerve sheath tumors (MPNST) are a rare form of soft tissue sarcoma with few studies reporting on patient outcomes and prognostic variables.
  2. Bottillo I, Ahlquist T, Brekke H, Danielsen S, van den Berg E, Mertens F, et al. Germline and somatic NF1 mutations in sporadic and NF1-associated malignant peripheral nerve sheath tumours. J Pathol. 2009;217:693-701 pubmed publisher
    ..However, the somatic events share common characteristics with the NF1-related and the sporadic tumours...
  3. Endo M, Kobayashi C, Setsu N, Takahashi Y, Kohashi K, Yamamoto H, et al. Prognostic significance of p14ARF, p15INK4b, and p16INK4a inactivation in malignant peripheral nerve sheath tumors. Clin Cancer Res. 2011;17:3771-82 pubmed publisher
    ..Here we carried out a genetic, epigenetic, and expression analysis of p14(ARF), p15(INK4b), and p16(INK4a), and clarified the prognostic significance of their inactivation in MPNSTs...
  4. Longhi A, Errani C, Magagnoli G, Alberghini M, Gambarotti M, Mercuri M, et al. High grade malignant peripheral nerve sheath tumors: outcome of 62 patients with localized disease and review of the literature. J Chemother. 2010;22:413-8 pubmed
    ..New drugs employed successfully in advanced mpNSt should be employed also in the adjuvant setting...
  5. Nielsen A, Jensen H, Leifsson P. Immunohistochemistry for 2',3'-cyclic nucleotide-3'-phosphohydrolase in 63 bovine peripheral nerve sheath tumors. Vet Pathol. 2011;48:796-802 pubmed publisher
    To establish a simple and uniform classification of bovine peripheral nerve sheath tumors (PNSTs), 63 tumors from 44 cattle were examined histologically and immunohistochemically with antibodies against S100 protein and 2',3'-cyclic ..
  6. Zou C, Smith K, Zhu Q, Liu J, McCutcheon I, Slopis J, et al. Dual targeting of AKT and mammalian target of rapamycin: a potential therapeutic approach for malignant peripheral nerve sheath tumor. Mol Cancer Ther. 2009;8:1157-68 pubmed publisher
    ..of rapamycin (mTOR) pathway may constitute a potential target for the treatment of malignant peripheral nerve sheath tumors (MPNST)...
  7. Subramanian S, Thayanithy V, West R, Lee C, Beck A, Zhu S, et al. Genome-wide transcriptome analyses reveal p53 inactivation mediated loss of miR-34a expression in malignant peripheral nerve sheath tumours. J Pathol. 2010;220:58-70 pubmed publisher
    ..Collectively, our findings suggest that deregulation of miRNAs has a potential role in the malignant transformation process in peripheral nerve sheath tumours...
  8. Kroep J, Ouali M, Gelderblom H, Le Cesne A, Dekker T, van Glabbeke M, et al. First-line chemotherapy for malignant peripheral nerve sheath tumor (MPNST) versus other histological soft tissue sarcoma subtypes and as a prognostic factor for MPNST: an EORTC soft tissue and bone sarcoma group study. Ann Oncol. 2011;22:207-14 pubmed publisher
    ..the role of chemotherapy in advanced malignant peripheral nerve sheath tumor (MPNST) is unclear...
  9. Kabir S, Kapetanakis E, Shabbo F. Intracardiac malignant Triton tumor: a first presentation. Ann Thorac Surg. 2010;89:968-9 pubmed publisher

More Information

Publications74

  1. Upadhyaya M. Genetic basis of tumorigenesis in NF1 malignant peripheral nerve sheath tumors. Front Biosci (Landmark Ed). 2011;16:937-51 pubmed
    Malignant peripheral nerve sheath tumors (MPNSTs), often found associated with neurofibromatosis type 1 (NF1), are aggressive tumors that pose significant diagnostic and therapeutic challenges...
  2. Spurlock G, Knight S, Thomas N, Kiehl T, 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;136:1869-80 pubmed publisher
    ..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...
  3. Park H, Lee S, Sohn Y, Jin H, Han J, Kim Y, et al. NF1 deficiency causes Bcl-xL upregulation in Schwann cells derived from neurofibromatosis type 1-associated malignant peripheral nerve sheath tumors. Int J Oncol. 2013;42:657-66 pubmed publisher
    ..in Schwann cells (SCs) is common in both benign plexiform neurofibromas (PNs) and malignant peripheral nerve sheath tumors (MPNSTs) in patients with neurofibromatosis type 1 (NF1), other genetic alterations in SCs may be required ..
  4. Feber A, Wilson G, Zhang L, Presneau N, Idowu B, Down T, et al. Comparative methylome analysis of benign and malignant peripheral nerve sheath tumors. Genome Res. 2011;21:515-24 pubmed publisher
    ..By using MeDIP-seq, we report a sequencing-based comparative methylome analysis of malignant peripheral nerve sheath tumors (MPNSTs), benign neurofibromas, and normal Schwann cells...
  5. Brekke H, Kolberg M, Skotheim R, Hall K, Bjerkehagen B, Risberg B, et al. Identification of p53 as a strong predictor of survival for patients with malignant peripheral nerve sheath tumors. Neuro Oncol. 2009;11:514-28 pubmed publisher
    ..Patients in complete remission with a primary p53-positive MPNST diagnosis may be considered in a high-risk subgroup and candidates for adjuvant treatment...
  6. Endo M, Yamamoto H, Setsu N, Kohashi K, Takahashi Y, Ishii T, et al. Prognostic significance of AKT/mTOR and MAPK pathways and antitumor effect of mTOR inhibitor in NF1-related and sporadic malignant peripheral nerve sheath tumors. Clin Cancer Res. 2013;19:450-61 pubmed publisher
    ..Moreover, we assessed the antitumor effects of the inhibitor of candidate target...
  7. Byer S, Eckert J, Brossier N, Clodfelder Miller B, Turk A, Carroll A, et al. Tamoxifen inhibits malignant peripheral nerve sheath tumor growth in an estrogen receptor-independent manner. Neuro Oncol. 2011;13:28-41 pubmed publisher
    Few therapeutic options are available for malignant peripheral nerve sheath tumors (MPNSTs), the most common malignancy associated with neurofibromatosis type 1 (NF1)...
  8. Brossier N, Carroll S. Genetically engineered mouse models shed new light on the pathogenesis of neurofibromatosis type I-related neoplasms of the peripheral nervous system. Brain Res Bull. 2012;88:58-71 pubmed publisher
    ..plexiform, but not dermal, neurofibromas often undergo malignant progression to form malignant peripheral nerve sheath tumors (MPNSTs), the most common malignancy occurring in NF1 patients...
  9. Widemann B. Current status of sporadic and neurofibromatosis type 1-associated malignant peripheral nerve sheath tumors. Curr Oncol Rep. 2009;11:322-8 pubmed
    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)...
  10. Zehou O, Bularca S, Bastuji Garin S, Ortonne N, Valeyrie Allanore L, Wolkenstein P, et al. Neurofibromatosis 1 phenotype associated to malignant peripheral nerve sheath tumours: a case-control study. J Eur Acad Dermatol Venereol. 2013;27:1044-7 pubmed publisher
    ..Malignant peripheral nerve sheath tumours (MPNSTs) are the main cause of death in neurofibromatosis 1 adult patients...
  11. Slomiany M, Dai L, Bomar P, Knackstedt T, Kranc D, Tolliver L, et al. Abrogating drug resistance in malignant peripheral nerve sheath tumors by disrupting hyaluronan-CD44 interactions with small hyaluronan oligosaccharides. Cancer Res. 2009;69:4992-8 pubmed publisher
    Malignant peripheral nerve sheath tumors (MPNST) develop in approximately 10% of neurofibromatosis type-1 patients and are a major contributing factor to neurofibromatosis-1 patient mortality and morbidity...
  12. Hakozaki M, Hojo H, Sato M, Tajino T, Yamada H, Kikuchi S, et al. Establishment and characterization of a novel human malignant peripheral nerve sheath tumor cell line, FMS-1, that overexpresses epidermal growth factor receptor and cyclooxygenase-2. Virchows Arch. 2009;455:517-26 pubmed publisher
    ..Thus, FMS-1 cells might be useful for investigating biological behaviors and developing new molecular-targeting antitumor drugs for MPNST expressing EGFR or COX-2...
  13. Lopez G, Torres K, Liu J, Hernandez B, Young E, Belousov R, et al. Autophagic survival in resistance to histone deacetylase inhibitors: novel strategies to treat malignant peripheral nerve sheath tumors. Cancer Res. 2011;71:185-96 pubmed publisher
    ..Here we report findings that strongly rationalize clinical evaluation of HDACis in malignant peripheral nerve sheath tumors (MPNST), a class of highly aggressive, therapeutically resistant, and commonly fatal malignancies that ..
  14. Wasa J, Nishida Y, Tsukushi S, Shido Y, Sugiura H, Nakashima H, et al. MRI features in the differentiation of malignant peripheral nerve sheath tumors and neurofibromas. AJR Am J Roentgenol. 2010;194:1568-74 pubmed publisher
    The objective of this study was to identify the MRI criteria that best differentiate malignant peripheral nerve sheath tumors from benign neurofibromas.
  15. Pasmant E, Masliah Planchon J, Lévy P, Laurendeau I, Ortonne N, Parfait B, et al. Identification of genes potentially involved in the increased risk of malignancy in NF1-microdeleted patients. Mol Med. 2011;17:79-87 pubmed publisher
    ..develop more neurofibromas at a younger age, and have an increased risk of malignant peripheral nerve sheath tumors (MPNSTs)...
  16. Brekke H, Ribeiro F, Kolberg M, Agesen T, Lind G, Eknaes M, et al. Genomic changes in chromosomes 10, 16, and X in malignant peripheral nerve sheath tumors identify a high-risk patient group. J Clin Oncol. 2010;28:1573-82 pubmed publisher
    The purpose of this study was to identify genetic aberrations contributing to clinical aggressiveness of malignant peripheral nerve sheath tumors (MPNSTs).
  17. Yu J, Deshmukh H, Payton J, Dunham C, Scheithauer B, Tihan T, et al. Array-based comparative genomic hybridization identifies CDK4 and FOXM1 alterations as independent predictors of survival in malignant peripheral nerve sheath tumor. Clin Cancer Res. 2011;17:1924-34 pubmed publisher
    Malignant peripheral nerve sheath tumors (MPNST) are highly aggressive sarcomas with variable patient survival and few known prognostically relevant genomic biomarkers...
  18. Ghosh A, Sastri S, Srinivas D, Mahadevan A, Anandappa C, Shankar S. Malignant triton tumor of cervical spine with hemorrhage. J Clin Neurosci. 2011;18:721-3 pubmed publisher
    ..He underwent surgery with decompression of the intradural, and a small part of the extradural, component. To our knowledge this is the first report of a cervical triton tumor presenting with an intratumoral bleed...
  19. Duong T, Sbidian E, Valeyrie Allanore L, Vialette C, Ferkal S, Hadj Rabia S, et al. Mortality associated with neurofibromatosis 1: a cohort study of 1895 patients in 1980-2006 in France. Orphanet J Rare Dis. 2011;6:18 pubmed publisher
    ..However, data on mortality in NF1 are limited. Our aim was to evaluate mortality in a large retrospective cohort of NF1 patients seen in France between 1980 and 2006...
  20. Spyra M, Kluwe L, Hagel C, Nguyen R, Panse J, Kurtz A, et al. Cancer stem cell-like cells derived from malignant peripheral nerve sheath tumors. PLoS ONE. 2011;6:e21099 pubmed publisher
    This study aims to examine whether or not cancer stem cells exist in malignant peripheral nerve sheath tumors (MPNST)...
  21. Ingham S, Huson S, Moran A, Wylie J, Leahy M, Evans D. Malignant peripheral nerve sheath tumours in NF1: improved survival in women and in recent years. Eur J Cancer. 2011;47:2723-8 pubmed publisher
    ..These uncommon tumours are known to occur at high frequency and lead to poor survival. Our aim was to determine risk of MPNST in NF1 patients, and survival rates...
  22. Requena L, Sitthinamsuwan P, Fried I, Kaddu S, Schirren C, Schärer L, et al. A benign cutaneous plexiform hybrid tumor of perineurioma and cellular neurothekeoma. Am J Surg Pathol. 2013;37:845-52 pubmed publisher
    There are several recent reports describing hybrid peripheral nerve sheath tumors showing a biphasic component of neoplastic cells...
  23. Yang J, Ylipaa A, Sun Y, Zheng H, Chen K, Nykter M, et al. Genomic and molecular characterization of malignant peripheral nerve sheath tumor identifies the IGF1R pathway as a primary target for treatment. Clin Cancer Res. 2011;17:7563-73 pubmed publisher
    ..Malignant peripheral nerve sheath tumor (MPNST) is a rare sarcoma that lacks effective therapeutic strategies. We gain insight into the most recurrent genetically altered pathways with the purpose of scanning possible therapeutic targets...
  24. Mo W, Chen J, Patel A, Zhang L, Chau V, Li Y, et al. CXCR4/CXCL12 mediate autocrine cell- cycle progression in NF1-associated malignant peripheral nerve sheath tumors. Cell. 2013;152:1077-90 pubmed publisher
    Malignant peripheral nerve sheath tumors (MPNSTs) are soft tissue sarcomas that arise in connective tissue surrounding peripheral nerves. They occur sporadically in a subset of patients with neurofibromatosis type 1 (NF1)...
  25. Kolberg M, Høland M, Agesen T, Brekke H, Liestøl K, Hall K, et al. Survival meta-analyses for >1800 malignant peripheral nerve sheath tumor patients with and without neurofibromatosis type 1. Neuro Oncol. 2013;15:135-47 pubmed publisher
    ..These observations support the hypothesis that MPNSTs arising in NF1 and non-NF1 patients are not different per se. Consequently, we suggest that the choice of treatment for MPNST should be independent of NF1 status...
  26. Keng V, Rahrmann E, Watson A, Tschida B, Moertel C, Jessen W, et al. PTEN and NF1 inactivation in Schwann cells produces a severe phenotype in the peripheral nervous system that promotes the development and malignant progression of peripheral nerve sheath tumors. Cancer Res. 2012;72:3405-13 pubmed publisher
    ..Pten) and neurofibromatosis 1 (Nf1) genes recently were found to be comutated in high-grade peripheral nerve sheath tumors (PNST) in mice...
  27. Ghadimi M, Lopez G, Torres K, Belousov R, Young E, Liu J, et al. Targeting the PI3K/mTOR axis, alone and in combination with autophagy blockade, for the treatment of malignant peripheral nerve sheath tumors. Mol Cancer Ther. 2012;11:1758-69 pubmed publisher
    ..for efficacious therapeutic strategies to improve the outcome of patients afflicted by malignant peripheral nerve sheath tumors (MPNST)...
  28. Verdijk R, Den Bakker M, Dubbink H, Hop W, Dinjens W, Kros J. TP53 mutation analysis of malignant peripheral nerve sheath tumors. J Neuropathol Exp Neurol. 2010;69:16-26 pubmed publisher
    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...
  29. Ferrari A, Miceli R, Rey A, Oberlin O, Orbach D, Brennan B, et al. Non-metastatic unresected paediatric non-rhabdomyosarcoma soft tissue sarcomas: results of a pooled analysis from United States and European groups. Eur J Cancer. 2011;47:724-31 pubmed publisher
    ..Various international research groups pooled their data in a joint study in order to investigate prognostic variables and treatment modalities...
  30. Zietsch J, Ziegenhagen N, Heppner F, Reuss D, von Deimling A, Holtkamp N. The 4q12 amplicon in malignant peripheral nerve sheath tumors: consequences on gene expression and implications for sunitinib treatment. PLoS ONE. 2010;5:e11858 pubmed publisher
    Malignant peripheral nerve sheath tumors (MPNST) are highly aggressive tumors which originate from Schwann cells and develop in about 10% of neurofibromatosis type 1 (NF1) patients...
  31. McConnell Y, Giacomantonio C. Malignant triton tumors--complete surgical resection and adjuvant radiotherapy associated with improved survival. J Surg Oncol. 2012;106:51-6 pubmed publisher
    Malignant triton tumors (MTT) are a rare form of peripheral nerve sheath tumors that follows a particularly aggressive course. Given its rarity, only case reports and small series of patients have been published.
  32. Naber U, Friedrich R, Glatzel M, Mautner V, Hagel C. Podoplanin and CD34 in peripheral nerve sheath tumours: focus on neurofibromatosis 1-associated atypical neurofibroma. J Neurooncol. 2011;103:239-45 pubmed publisher
    ..Podoplanin expression in atypical neurofibroma was not associated with tumour progression towards MPNST...
  33. Demir H, Varan A, Yalçn B, Akyuz C, Kutluk T, Buyukpamukcu M. Malignant peripheral nerve sheath tumors in childhood: 13 cases from a single center. J Pediatr Hematol Oncol. 2012;34:204-7 pubmed publisher
    We aimed to review clinical characteristics, treatment results, and outcome for pediatric patients with malignant peripheral nerve sheath tumors (MPNSTs).
  34. Torres K, Zhu Q, Bill K, Lopez G, Ghadimi M, Xie X, et al. Activated MET is a molecular prognosticator and potential therapeutic target for malignant peripheral nerve sheath tumors. Clin Cancer Res. 2011;17:3943-55 pubmed publisher
    MET signaling has been suggested a potential role in malignant peripheral nerve sheath tumors (MPNST). Here, MET function and blockade were preclinically assessed.
  35. Potocka M, Skorek A, Izycka Swieszewska E. [Malignant Triton tumor of the nose--case report]. Otolaryngol Pol. 2009;63:165-7 pubmed publisher
    ..Histopathological diagnosis of the tumor was made on the surgical specimen, based on immunohistochemical analysis. Radiation therapy as the next step of treatment of choice was performed in presented case...
  36. Schulman F, Johnson T, Facemire P, Fanburg Smith J. Feline peripheral nerve sheath tumors: histologic, immunohistochemical, and clinicopathologic correlation (59 tumors in 53 cats). Vet Pathol. 2009;46:1166-80 pubmed publisher
    Feline peripheral nerve sheath tumors are uncommonly reported, and their clinical behavior has not been well documented. Fifty-nine peripheral nerve sheath tumors were collected from 53 cats...
  37. Zou C, Smith K, Liu J, Lahat G, Myers S, Wang W, et al. Clinical, pathological, and molecular variables predictive of malignant peripheral nerve sheath tumor outcome. Ann Surg. 2009;249:1014-22 pubmed publisher
    ..Consequently, we sought to identify clinical, pathologic, and molecular predictors of outcome in patients with/without neurofibromatosis type 1 (NF-1) associated MPNST...
  38. Keizman D, Issakov J, Meller I, Maimon N, Meimon N, Ish Shalom M, et al. Expression and significance of EGFR in malignant peripheral nerve sheath tumor. J Neurooncol. 2009;94:383-8 pubmed publisher
    ..EGFR appeared to play a role in MPNST progression. EGFR overexpression was correlated with worse prognostic variables and course. Clinical trials of targeting EGFR in MPNST are warranted...
  39. Perrone F, Da Riva L, Orsenigo M, Losa M, Jocollè G, Millefanti C, et al. PDGFRA, PDGFRB, EGFR, and downstream signaling activation in malignant peripheral nerve sheath tumor. Neuro Oncol. 2009;11:725-36 pubmed publisher
    ..B (PDGFRB), epidermal growth factor receptor (EGFR), and their downstream pathways in malignant peripheral nerve sheath tumors (MPNSTs)...
  40. Scheithauer B, Erdogan S, Rodriguez F, Burger P, Woodruff J, Kros J, et al. Malignant peripheral nerve sheath tumors of cranial nerves and intracranial contents: a clinicopathologic study of 17 cases. Am J Surg Pathol. 2009;33:325-38 pubmed publisher
    Malignant peripheral nerve sheath tumors (MPNSTs) arising from cranial nerves or their branches are very uncommon. The literature consists mainly of isolated case reports and small series...
  41. Ziadi A, Saliba I. Malignant peripheral nerve sheath tumor of intracranial nerve: a case series review. Auris Nasus Larynx. 2010;37:539-45 pubmed publisher
  42. Plaat B, Molenaar W, Mastik M, Hoekstra H, Te Meerman G, van den Berg E. Computer-assisted cytogenetic analysis of 51 malignant peripheral-nerve-sheath tumors: sporadic vs. neurofibromatosis-type-1-associated malignant schwannomas. Int J Cancer. 1999;83:171-8 pubmed
    ..2), is not a common cytogenetic finding in NF-1-associated MPNSTs. The observed differences between NF-1-associated and sporadic MPNSTs might reflect different oncogenetic pathways. ..
  43. Mertens F, Dal Cin P, De Wever I, Fletcher C, Mandahl N, Mitelman F, et al. Cytogenetic characterization of peripheral nerve sheath tumours: a report of the CHAMP study group. J Pathol. 2000;190:31-8 pubmed
    ..The cytogenetic findings in peripheral nerve sheath tumours show that the karyotype is a good discriminator between BPNSTs and MPNSTs, and that the pattern of aberrations among the latter may add prognostic information. ..
  44. Patnaik A, Zachos T, Sams A, Aitken M. Malignant nerve-sheath tumor with divergent and glandular differentiation in a dog: a case report. Vet Pathol. 2002;39:406-10 pubmed
    ..The tumor was located in the peripheral nerve roots at the first and second lumbar vertebrae. The dog survived for 161 days after resection of the tumor. ..
  45. Kobayashi C, Oda Y, Takahira T, Izumi T, Kawaguchi K, Yamamoto H, et al. Chromosomal aberrations and microsatellite instability of malignant peripheral nerve sheath tumors: a study of 10 tumors from nine patients. Cancer Genet Cytogenet. 2006;165:98-105 pubmed
    ..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. ..
  46. Chijiwa K, Uchida K, Tateyama S. Immunohistochemical evaluation of canine peripheral nerve sheath tumors and other soft tissue sarcomas. Vet Pathol. 2004;41:307-18 pubmed
    Seventeen cases of canine peripheral nerve sheath tumors (PNSTs), 11 malignant PNSTs (MPNSTs), and six benign PNSTs (BPNSTs) were examined...
  47. Stark A, Buhl R, Hugo H, Mehdorn H. Malignant peripheral nerve sheath tumours--report of 8 cases and review of the literature. Acta Neurochir (Wien). 2001;143:357-63; discussion 363-4 pubmed
    ..Modern clinical studies and the development of effective chemotherapy is needed in order to gain control of the disease. ..
  48. Koga T, Iwasaki H, Ishiguro M, Matsuzaki A, Kikuchi M. Frequent genomic imbalances in chromosomes 17, 19, and 22q in peripheral nerve sheath tumours detected by comparative genomic hybridization analysis. J Pathol. 2002;197:98-107 pubmed
    ..The significance of the losses of chromosome 19 in these cases is not clear at present, but in NF1-associated neurofibromas, the presence of some as yet unknown tumour suppressor genes on chromosome 19 cannot be ruled out. ..
  49. Storlazzi C, Brekke H, Mandahl N, Brosjo O, Smeland S, Lothe R, et al. Identification of a novel amplicon at distal 17q containing the BIRC5/SURVIVIN gene in malignant peripheral nerve sheath tumours. J Pathol. 2006;209:492-500 pubmed
  50. Barkan B, Starinsky S, Friedman E, Stein R, Kloog Y. The Ras inhibitor farnesylthiosalicylic acid as a potential therapy for neurofibromatosis type 1. Clin Cancer Res. 2006;12:5533-42 pubmed
    ..FTS treatment of NF1 cells normalized Ras-GTP levels, resulting in reversal of the transformed phenotype and inhibition of tumor growth. FTS may therefore be considered as a potential drug for the treatment of NF1. ..
  51. Watanabe T, Oda Y, Tamiya S, Masuda K, Tsuneyoshi M. Malignant peripheral nerve sheath tumour arising within neurofibroma. An immunohistochemical analysis in the comparison between benign and malignant components. J Clin Pathol. 2001;54:631-6 pubmed
    ..On the basis of these findings, TGF-beta 1, HGF-alpha, and p53 might be involved in the malignant transformation of neurofibroma to MPNST. ..
  52. Vauthey J, Woodruff J, Brennan M. Extremity malignant peripheral nerve sheath tumors (neurogenic sarcomas): a 10-year experience. Ann Surg Oncol. 1995;2:126-31 pubmed
    ..studies have shown a high rate of local recurrence and a dismal overall prognosis in malignant peripheral nerve sheath tumors (MPNSTs)...
  53. Van Herendael B, Heyman S, Vanhoenacker F, De Temmerman G, Bloem J, Parizel P, et al. The value of magnetic resonance imaging in the differentiation between malignant peripheral nerve-sheath tumors and non-neurogenic malignant soft-tissue tumors. Skeletal Radiol. 2006;35:745-53 pubmed
    ..To assess the sensitivity and specificity of MRI criteria in the differentiation between malignant peripheral nerve sheath tumors (MPNST) and non-neurogenic malignant soft-tissue tumors (MSTT)...
  54. Kluwe L, Friedrich R, Peiper M, Friedman J, Mautner V. Constitutional NF1 mutations in neurofibromatosis 1 patients with malignant peripheral nerve sheath tumors. Hum Mutat. 2003;22:420 pubmed
    Neurofibromatosis type 1 (NF1) patients have 10% of lifetime risk for developing malignant peripheral nerve sheath tumors (MPNST), one of the most aggressive cancers...
  55. Haddadin M, Hawkins A, Long P, Morsberger L, Depew D, Epstein J, et al. Cytogenetic study of malignant triton tumor: a case report. Cancer Genet Cytogenet. 2003;144:100-5 pubmed
    ..2, 17q11.2, and 19q13.1. FISH showed high increase of copy number for MYC and loss of a single copy for TP53...
  56. Fieber L, González D, Wallace M, Muir D. Delayed rectifier K currents in NF1 Schwann cells. Pharmacological block inhibits proliferation. Neurobiol Dis. 2003;13:136-46 pubmed
  57. Matyja E, Naganska E, Górski R, Zabek M. Multiple brain metastases from malignant peripheral nerve sheath tumour (MPNST). Folia Neuropathol. 2004;42:43-8 pubmed
    ..The schwannian nature of brain metastases has been confirmed by immunohistochemical findings showing S-100 protein and GFAP expression and ultrastructural evidences of the pericellular basal lamina...
  58. Park J, Choi K, Lee H, Rhee Y, Lee Y, Chung M. Intrathoracic malignant peripheral nerve sheath tumor in von Recklinghausen's disease. Korean J Intern Med. 2001;16:201-4 pubmed
    ..But intrathoracic manifestations are very rare. We report a case of a 40 year-old man with multiple neurofibromatosis who was presented with an intrathoracic malignant peripheral nerve sheath tumor...
  59. Stemmer Rachamimov A, Louis D, Nielsen G, Antonescu C, Borowsky A, Bronson R, et al. Comparative pathology of nerve sheath tumors in mouse models and humans. Cancer Res. 2004;64:3718-24 pubmed
    ..A novel classification scheme for peripheral nerve sheath tumors in murine models was therefore devised.
  60. Skotheim R, Kallioniemi A, Bjerkhagen B, Mertens F, Brekke H, Monni O, et al. Topoisomerase-II alpha is upregulated in malignant peripheral nerve sheath tumors and associated with clinical outcome. J Clin Oncol. 2003;21:4586-91 pubmed
    ..To identify target genes of clinical significance for patients with malignant peripheral-nerve sheath tumor (MPNST), an aggressive cancer for which no consensus therapy exists...
  61. Arun D, Gutmann D. Recent advances in neurofibromatosis type 1. Curr Opin Neurol. 2004;17:101-5 pubmed
    ..The purpose of this review is to highlight recent advances in defining the molecular etiology of nervous system tumors and learning disabilities...
  62. Aoki M, Nabeshima K, Nishio J, Ishiguro M, Fujita C, Koga K, et al. Establishment of three malignant peripheral nerve sheath tumor cell lines, FU-SFT8611, 8710 and 9817: conventional and molecular cytogenetic characterization. Int J Oncol. 2006;29:1421-8 pubmed
    ..These newly established cell lines provide a valuable resource for biological and pathological investigations into new treatment regimes for MPNST...
  63. Schmidt H, Taubert H, Meye A, Wurl P, Bache M, Bartel F, et al. Gains in chromosomes 7, 8q, 15q and 17q are characteristic changes in malignant but not in benign peripheral nerve sheath tumors from patients with Recklinghausen's disease. Cancer Lett. 2000;155:181-90 pubmed
    ..genomic hybridization (CGH) gained results from six benign neurofibromas and 14 malignant peripheral nerve sheath tumors (MPNSTs) were compared with four benign peripheral nerve sheath tumors (BPNSTs) from patients without NF1...
  64. Gottfried O, Viskochil D, Fults D, Couldwell W. Molecular, genetic, and cellular pathogenesis of neurofibromas and surgical implications. Neurosurgery. 2006;58:1-16; discussion 1-16 pubmed
    ..summarizes the most recent NF1 and neurofibroma literature describing the pathogenesis and treatment of nerve sheath tumors. Understanding the biological underpinnings of tumorigenesis in NF1 has implications for future surgical ..
  65. Grobmyer S, Reith J, Shahlaee A, Bush C, Hochwald S. Malignant Peripheral Nerve Sheath Tumor: molecular pathogenesis and current management considerations. J Surg Oncol. 2008;97:340-9 pubmed publisher
    Malignant Peripheral Nerve Sheath Tumors (MPNSTs) are rare tumors that often occur in patients with neurofibromatosis 1. Surgical resection represents the mainstay of treatment...