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MeSH Review

Nerve Sheath Neoplasms

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Disease relevance of Nerve Sheath Neoplasms


Psychiatry related information on Nerve Sheath Neoplasms


High impact information on Nerve Sheath Neoplasms


Chemical compound and disease context of Nerve Sheath Neoplasms


Biological context of Nerve Sheath Neoplasms

  • In addition to providing a model for studying the molecular pathogenic pathways of malignant peripheral nerve sheath tumors, these mutant zebrafish lines provide a unique platform for modifier screens to identify genetic mutations or small molecules that affect tp53-related pathways, including apoptosis, cell-cycle delay, and tumor suppression [8].
  • Collaborative research should be promoted with the aim of harnessing advances in molecular genetics to develop targeted therapies for MPNST in people with NF1 [2].
  • Methylation analysis and mutation analysis of CDKN2A/p16 in MPNSTs did not reveal any abnormalities [17].
  • These phenotypes were correlated with proliferative index, as assessed by Ki-67, as well as clinicopathological parameters of poor outcome. p53 nuclear overexpression was found in 10 of 35 (29%) MPNSTs, and it was lacking in NFs (P = 0.02) [18].
  • A deviation from the disomic status of chromosome 17 was observed in two of the MPNST from NF1 patients [19].

Anatomical context of Nerve Sheath Neoplasms


Gene context of Nerve Sheath Neoplasms


Analytical, diagnostic and therapeutic context of Nerve Sheath Neoplasms


  1. Epidermal growth factor receptor expression in neurofibromatosis type 1-related tumors and NF1 animal models. DeClue, J.E., Heffelfinger, S., Benvenuto, G., Ling, B., Li, S., Rui, W., Vass, W.C., Viskochil, D., Ratner, N. J. Clin. Invest. (2000) [Pubmed]
  2. International consensus statement on malignant peripheral nerve sheath tumors in neurofibromatosis. Ferner, R.E., Gutmann, D.H. Cancer Res. (2002) [Pubmed]
  3. Notch and Schwann cell transformation. Li, Y., Rao, P.K., Wen, R., Song, Y., Muir, D., Wallace, P., van Horne, S.J., Tennekoon, G.I., Kadesch, T. Oncogene (2004) [Pubmed]
  4. Expression and function of somatostatin receptors in peripheral nerve sheath tumors. Mawrin, C., Schulz, S., Hellwig-Patyk, A., Kirches, E., Roessner, A., Lendeckel, U., Firsching, R., Vorwerk, C.K., Keilhoff, G., Dietzmann, K., Grimm, K., Lindberg, G., Gutmann, D.H., Scheithauer, B.W., Perry, A. J. Neuropathol. Exp. Neurol. (2005) [Pubmed]
  5. Tumor suppressor mutations and growth factor signaling in the pathogenesis of NF1-associated peripheral nerve sheath tumors: II. The role of dysregulated growth factor signaling. Carroll, S.L., Stonecypher, M.S. J. Neuropathol. Exp. Neurol. (2005) [Pubmed]
  6. Neurofibromatosis 1. Lynch, T.M., Gutmann, D.H. Neurologic clinics. (2002) [Pubmed]
  7. Mouse models of tumor development in neurofibromatosis type 1. Cichowski, K., Shih, T.S., Schmitt, E., Santiago, S., Reilly, K., McLaughlin, M.E., Bronson, R.T., Jacks, T. Science (1999) [Pubmed]
  8. tp53 mutant zebrafish develop malignant peripheral nerve sheath tumors. Berghmans, S., Murphey, R.D., Wienholds, E., Neuberg, D., Kutok, J.L., Fletcher, C.D., Morris, J.P., Liu, T.X., Schulte-Merker, S., Kanki, J.P., Plasterk, R., Zon, L.I., Look, A.T. Proc. Natl. Acad. Sci. U.S.A. (2005) [Pubmed]
  9. Elevated risk for MPNST in NF1 microdeletion patients. De Raedt, T., Brems, H., Wolkenstein, P., Vidaud, D., Pilotti, S., Perrone, F., Mautner, V., Frahm, S., Sciot, R., Legius, E. Am. J. Hum. Genet. (2003) [Pubmed]
  10. Large-scale molecular comparison of human schwann cells to malignant peripheral nerve sheath tumor cell lines and tissues. Miller, S.J., Rangwala, F., Williams, J., Ackerman, P., Kong, S., Jegga, A.G., Kaiser, S., Aronow, B.J., Frahm, S., Kluwe, L., Mautner, V., Upadhyaya, M., Muir, D., Wallace, M., Hagen, J., Quelle, D.E., Watson, M.A., Perry, A., Gutmann, D.H., Ratner, N. Cancer Res. (2006) [Pubmed]
  11. Topoisomerase-II alpha is upregulated in malignant peripheral nerve sheath tumors and associated with clinical outcome. Skotheim, R.I., Kallioniemi, A., Bjerkhagen, B., Mertens, F., Brekke, H.R., Monni, O., Mousses, S., Mandahl, N., Soeter, G., Nesland, J.M., Smeland, S., Kallioniemi, O.P., Lothe, R.A. J. Clin. Oncol. (2003) [Pubmed]
  12. Differential modulation of malignant peripheral nerve sheath tumor growth by omega-3 and omega-6 fatty acids. Mashour, G.A., Drissel, S.N., Frahm, S., Farassati, F., Martuza, R.L., Mautner, V.F., Kindler-Röhrborn, A., Kurtz, A. Oncogene (2005) [Pubmed]
  13. Mutation and expression of PDGFRA and KIT in malignant peripheral nerve sheath tumors, and its implications for imatinib sensitivity. Holtkamp, N., Okuducu, A.F., Mucha, J., Afanasieva, A., Hartmann, C., Atallah, I., Estevez-Schwarz, L., Mawrin, C., Friedrich, R.E., Mautner, V.F., von Deimling, A. Carcinogenesis (2006) [Pubmed]
  14. Neuromuscular choristoma. Mitchell, A., Scheithauer, B.W., Ostertag, H., Sepehrnia, A., Sav, A. Am. J. Clin. Pathol. (1995) [Pubmed]
  15. Hepatocyte growth factor and c-MET in benign and malignant peripheral nerve sheath tumors. Rao, U.N., Sonmez-Alpan, E., Michalopoulos, G.K. Hum. Pathol. (1997) [Pubmed]
  16. Activated MET Is a Molecular Prognosticator and Potential Therapeutic Target for Malignant Peripheral Nerve Sheath Tumors. Torres, K.E., Zhu, Q.S., Bill, K., Lopez, G., Ghadimi, M.P., Xie, X., Young, E.D., Liu, J., Nguyen, T., Bolshakov, S., Belousov, R., Wang, S., Lahat, G., Liu, J., Hernandez, B., Lazar, A.J., Lev, D. Clin. Cancer Res. (2011) [Pubmed]
  17. Malignant transformation of neurofibromas in neurofibromatosis 1 is associated with CDKN2A/p16 inactivation. Nielsen, G.P., Stemmer-Rachamimov, A.O., Ino, Y., Moller, M.B., Rosenberg, A.E., Louis, D.N. Am. J. Pathol. (1999) [Pubmed]
  18. Expression of p27(kip) and other cell cycle regulators in malignant peripheral nerve sheath tumors and neurofibromas: the emerging role of p27(kip) in malignant transformation of neurofibromas. Kourea, H.P., Cordon-Cardo, C., Dudas, M., Leung, D., Woodruff, J.M. Am. J. Pathol. (1999) [Pubmed]
  19. Alterations at chromosome 17 loci in peripheral nerve sheath tumors. Lothe, R.A., Slettan, A., Saeter, G., Brøgger, A., Børresen, A.L., Nesland, J.M. J. Neuropathol. Exp. Neurol. (1995) [Pubmed]
  20. Brain lipid binding protein in axon-Schwann cell interactions and peripheral nerve tumorigenesis. Miller, S.J., Li, H., Rizvi, T.A., Huang, Y., Johansson, G., Bowersock, J., Sidani, A., Vitullo, J., Vogel, K., Parysek, L.M., DeClue, J.E., Ratner, N. Mol. Cell. Biol. (2003) [Pubmed]
  21. The angiogenic factor midkine is aberrantly expressed in NF1-deficient Schwann cells and is a mitogen for neurofibroma-derived cells. Mashour, G.A., Ratner, N., Khan, G.A., Wang, H.L., Martuza, R.L., Kurtz, A. Oncogene (2001) [Pubmed]
  22. Differential NF1, p16, and EGFR patterns by interphase cytogenetics (FISH) in malignant peripheral nerve sheath tumor (MPNST) and morphologically similar spindle cell neoplasms. Perry, A., Kunz, S.N., Fuller, C.E., Banerjee, R., Marley, E.F., Liapis, H., Watson, M.A., Gutmann, D.H. J. Neuropathol. Exp. Neurol. (2002) [Pubmed]
  23. Metastatic malignant melanoma resembling malignant peripheral nerve sheath tumor: report of 16 cases. King, R., Busam, K., Rosai, J. Am. J. Surg. Pathol. (1999) [Pubmed]
  24. An imprinted locus epistatically influences Nstr1 and Nstr2 to control resistance to nerve sheath tumors in a neurofibromatosis type 1 mouse model. Reilly, K.M., Broman, K.W., Bronson, R.T., Tsang, S., Loisel, D.A., Christy, E.S., Sun, Z., Diehl, J., Munroe, D.J., Tuskan, R.G. Cancer Res. (2006) [Pubmed]
  25. Expression patterns of cell cycle components in sporadic and neurofibromatosis type 1-related malignant peripheral nerve sheath tumors. Agesen, T.H., Flørenes, V.A., Molenaar, W.M., Lind, G.E., Berner, J.M., Plaat, B.E., Komdeur, R., Myklebost, O., van den Berg, E., Lothe, R.A. J. Neuropathol. Exp. Neurol. (2005) [Pubmed]
  26. Chromosome band 9p21 is frequently altered in malignant peripheral nerve sheath tumors: studies of CDKN2A and other genes of the pRB pathway. Berner, J.M., Sørlie, T., Mertens, F., Henriksen, J., Saeter, G., Mandahl, N., Brøgger, A., Myklebost, O., Lothe, R.A. Genes Chromosomes Cancer (1999) [Pubmed]
  27. ATP-binding cassette superfamily transporter gene expression in human soft tissue sarcomas. Oda, Y., Saito, T., Tateishi, N., Ohishi, Y., Tamiya, S., Yamamoto, H., Yokoyama, R., Uchiumi, T., Iwamoto, Y., Kuwano, M., Tsuneyoshi, M. Int. J. Cancer (2005) [Pubmed]
  28. Neurofibromin-deficient fibroblasts fail to form perineurium in vitro. Rosenbaum, T., Boissy, Y.L., Kombrinck, K., Brannan, C.I., Jenkins, N.A., Copeland, N.G., Ratner, N. Development (1995) [Pubmed]
  29. The intermediate filament complement of the spectrum of nerve sheath neoplasms. Gould, V.E., Moll, R., Moll, I., Lee, I., Schwechheimer, K., Franke, W.W. Lab. Invest. (1986) [Pubmed]
  30. Studies on experimental malignant nerve sheath tumors maintained in tissue and organ culture systems. II. Electron microscopy observations. Conley, F.K., Rubinstein, L.J., Spence, A.M. Acta Neuropathol. (1976) [Pubmed]
  31. Subungual perineurioma: a peculiar location. Baran, R., Perrin, C. Br. J. Dermatol. (2002) [Pubmed]
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