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MeSH Review

Neuroectodermal Tumors, Primitive

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Disease relevance of Neuroectodermal Tumors, Primitive


High impact information on Neuroectodermal Tumors, Primitive


Chemical compound and disease context of Neuroectodermal Tumors, Primitive


Biological context of Neuroectodermal Tumors, Primitive


Anatomical context of Neuroectodermal Tumors, Primitive


Gene context of Neuroectodermal Tumors, Primitive

  • PTCH mutations were detected in eight BCCs and one PNET [15].
  • In summary, our results indicate that both PTCH and SMOH represent important targets for genetic alterations in sporadic BCCs and PNETs [15].
  • Northern blot analyses revealed neurofilament (NF) protein mRNAs in 6 of 7 PNET cell lines but no glial fibrillary acidic protein (GFAP) mRNA [23].
  • This extends evidence that multipotent CNS precursors are the cellular substrate from which certain PNET develop and shows that FGF2 is a potent proliferation and differentiation inducer for PNET cells in vitro, suggesting that FGF2 may also modulate the evolution of PNET in vivo [24].
  • FLI-1 protein expression is also helpful in distinguishing ES/PNET from other tumors that may be CD99-positive, such as PDSS and RMS [25].

Analytical, diagnostic and therapeutic context of Neuroectodermal Tumors, Primitive

  • Twenty patients with bone [osteogenic (OS) and Ewing's (ES) and/or primitive neuroectodermal tumor (PNET)] sarcoma, treated with chemotherapy and surgery or with chemotherapy alone, underwent (31)P MRSI studies pre- and post-therapy [26].
  • Assessment of TrkC mRNA levels may aid in treatment planning for patients with PNETs and should be incorporated prospectively into PNET clinical trials [12].
  • PCR band signals of each exon of the hSNF5/INI1 were weak or nearly undetectable in both MRTK and PNET, whereas those of the corresponding normal kidney were clearly detected [22].
  • The authors' results question the use of p30/32MIC2 immunostaining alone for the identification of ES/PNET and suggest the adoption of RT-PCR as an advantageous alternative [27].
  • Immunohistochemistry for the carboxy-terminus of FLI-1 is sensitive and highly specific for the diagnosis of ES/PNET [28].


  1. EWS-Fli1 antisense oligodeoxynucleotide inhibits proliferation of human Ewing's sarcoma and primitive neuroectodermal tumor cells. Tanaka, K., Iwakuma, T., Harimaya, K., Sato, H., Iwamoto, Y. J. Clin. Invest. (1997) [Pubmed]
  2. Quantitative study of monocyte chemoattractant protein-1 (MCP-1) in cerebrospinal fluid and cyst fluid from patients with malignant glioma. Kuratsu, J., Yoshizato, K., Yoshimura, T., Leonard, E.J., Takeshima, H., Ushio, Y. J. Natl. Cancer Inst. (1993) [Pubmed]
  3. Ewing sarcoma 11;22 translocation produces a chimeric transcription factor that requires the DNA-binding domain encoded by FLI1 for transformation. May, W.A., Gishizky, M.L., Lessnick, S.L., Lunsford, L.B., Lewis, B.C., Delattre, O., Zucman, J., Thomas, G., Denny, C.T. Proc. Natl. Acad. Sci. U.S.A. (1993) [Pubmed]
  4. Comparison of CSF cytology and spinal magnetic resonance imaging in the detection of leptomeningeal disease in pediatric medulloblastoma or primitive neuroectodermal tumor. Fouladi, M., Gajjar, A., Boyett, J.M., Walter, A.W., Thompson, S.J., Merchant, T.E., Jenkins, J.J., Langston, J.W., Liu, A., Kun, L.E., Heideman, R.L. J. Clin. Oncol. (1999) [Pubmed]
  5. Induction of brain tumors in mice using a recombinant platelet-derived growth factor B-chain retrovirus. Uhrbom, L., Hesselager, G., Nistér, M., Westermark, B. Cancer Res. (1998) [Pubmed]
  6. Loss of cell polarity causes severe brain dysplasia in Lgl1 knockout mice. Klezovitch, O., Fernandez, T.E., Tapscott, S.J., Vasioukhin, V. Genes Dev. (2004) [Pubmed]
  7. Dianhydrogalactitol and radiation therapy. Treatment of supratentorial glioma. Eagan, R.T., Childs, D.S., Layton, D.D., Laws, E.R., Bisel, H.F., Holbrook, M.A., Fleming, T.R. JAMA (1979) [Pubmed]
  8. Insulin-like growth factor I expression by tumors of neuroectodermal origin with the t(11;22) chromosomal translocation. A potential autocrine growth factor. Yee, D., Favoni, R.E., Lebovic, G.S., Lombana, F., Powell, D.R., Reynolds, C.P., Rosen, N. J. Clin. Invest. (1990) [Pubmed]
  9. Carcinogenicity of the antineoplastic agent, 5-(3,3-dimethyl-1-triazeno)-imidazole-4-carboxamide, and its metabolites in rats. Beal, D.D., Skibba, J.L., Croft, W.A., Cohen, S.M., Bryan, G.T. J. Natl. Cancer Inst. (1975) [Pubmed]
  10. Synergistic effect of combined hyperthermia and a nitrosourea in treatment of a murine ependymoblastoma. Thuning, C.A., Bakir, N.A., Warren, J. Cancer Res. (1980) [Pubmed]
  11. Treatment of metastatic Ewing's sarcoma or primitive neuroectodermal tumor of bone: evaluation of combination ifosfamide and etoposide--a Children's Cancer Group and Pediatric Oncology Group study. Miser, J.S., Krailo, M.D., Tarbell, N.J., Link, M.P., Fryer, C.J., Pritchard, D.J., Gebhardt, M.C., Dickman, P.S., Perlman, E.J., Meyers, P.A., Donaldson, S.S., Moore, S., Rausen, A.R., Vietti, T.J., Grier, H.E. J. Clin. Oncol. (2004) [Pubmed]
  12. TrkC expression predicts good clinical outcome in primitive neuroectodermal brain tumors. Grotzer, M.A., Janss, A.J., Fung, K., Biegel, J.A., Sutton, L.N., Rorke, L.B., Zhao, H., Cnaan, A., Phillips, P.C., Lee, V.M., Trojanowski, J.Q. J. Clin. Oncol. (2000) [Pubmed]
  13. Potentiation of 1-(2-chloroethyl)-3-cyclohexyl-1-nitrosourea by amphotericin B in murine ependymoblastoma. Laurent, G., Atassi, G., Hildebrand, J. Cancer Res. (1976) [Pubmed]
  14. EWS/Fli-1 chimeric protein is a transcriptional activator. Ohno, T., Rao, V.N., Reddy, E.S. Cancer Res. (1993) [Pubmed]
  15. Missense mutations in SMOH in sporadic basal cell carcinomas of the skin and primitive neuroectodermal tumors of the central nervous system. Reifenberger, J., Wolter, M., Weber, R.G., Megahed, M., Ruzicka, T., Lichter, P., Reifenberger, G. Cancer Res. (1998) [Pubmed]
  16. hTERT gene amplification and increased mRNA expression in central nervous system embryonal tumors. Fan, X., Wang, Y., Kratz, J., Brat, D.J., Robitaille, Y., Moghrabi, A., Perlman, E.J., Dang, C.V., Burger, P.C., Eberhart, C.G. Am. J. Pathol. (2003) [Pubmed]
  17. Lovastatin induces apoptosis in a primitive neuroectodermal tumor cell line in association with RB down-regulation and loss of the G1 checkpoint. Kim, J.S., Pirnia, F., Choi, Y.H., Nguyen, P.M., Knepper, B., Tsokos, M., Schulte, T.W., Birrer, M.J., Blagosklonny, M.V., Schaefer, O., Mushinski, J.F., Trepel, J.B. Oncogene (2000) [Pubmed]
  18. The Ews/Fli-1 fusion gene switches the differentiation program of neuroblastomas to Ewing sarcoma/peripheral primitive neuroectodermal tumors. Rorie, C.J., Thomas, V.D., Chen, P., Pierce, H.H., O'Bryan, J.P., Weissman, B.E. Cancer Res. (2004) [Pubmed]
  19. Loss of p16 pathways stabilizes EWS/FLI1 expression and complements EWS/FLI1 mediated transformation. Deneen, B., Denny, C.T. Oncogene (2001) [Pubmed]
  20. Failure of HPV E6 to rapidly degrade p53 in human HeLa x PNET cell hybrids. Isaacs, J.S., Chen, P., Garza, A., Hansen, M.F., Barrett, J.C., Weissman, B.E. Oncogene (1997) [Pubmed]
  21. Cerebral medulloepithelioma with bone, cartilage, and striated muscle. Light microscopic and immunohistochemical study. Auer, R.N., Becker, L.E. J. Neuropathol. Exp. Neurol. (1983) [Pubmed]
  22. Novel germ-line deletion of SNF5/INI1/SMARCB1 gene in neonate presenting with congenital malignant rhabdoid tumor of kidney and brain primitive neuroectodermal tumor. Kusafuka, T., Miao, J., Yoneda, A., Kuroda, S., Fukuzawa, M. Genes Chromosomes Cancer (2004) [Pubmed]
  23. Co-expression of low molecular weight neurofilament protein and glial fibrillary acidic protein in established human glioma cell lines. Tlhyama, T., Lee, V.M., Trojanowski, J.Q. Am. J. Pathol. (1993) [Pubmed]
  24. Human primitive neuroectodermal tumour cells behave as multipotent neural precursors in response to FGF2. Derrington, E.A., Dufay, N., Rudkin, B.B., Belin, M.F. Oncogene (1998) [Pubmed]
  25. Immunohistochemical detection of FLI-1 protein expression: a study of 132 round cell tumors with emphasis on CD99-positive mimics of Ewing's sarcoma/primitive neuroectodermal tumor. Folpe, A.L., Hill, C.E., Parham, D.M., O'Shea, P.A., Weiss, S.W. Am. J. Surg. Pathol. (2000) [Pubmed]
  26. Identification of prognostic markers in bone sarcomas using proton-decoupled phosphorus magnetic resonance spectroscopy. Zakian, K.L., Shukla-Dave, A., Meyers, P., Gorlick, R., Healey, J., Thaler, H.T., Huvos, A.G., Panicek, D.M., Koutcher, J.A. Cancer Res. (2003) [Pubmed]
  27. Immunostaining of the p30/32MIC2 antigen and molecular detection of EWS rearrangements for the diagnosis of Ewing's sarcoma and peripheral neuroectodermal tumor. Scotlandi, K., Serra, M., Manara, M.C., Benini, S., Sarti, M., Maurici, D., Lollini, P.L., Picci, P., Bertoni, F., Baldini, N. Hum. Pathol. (1996) [Pubmed]
  28. Primary Ewing's sarcoma/primitive neuroectodermal tumor of the kidney: a clinicopathologic and immunohistochemical analysis of 11 cases. Jimenez, R.E., Folpe, A.L., Lapham, R.L., Ro, J.Y., O'Shea, P.A., Weiss, S.W., Amin, M.B. Am. J. Surg. Pathol. (2002) [Pubmed]
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