Is there a predisposition gene for ewing's sarcoma?

R Randall, S. L. Lessnick, K. B. Jones, L. G. Gouw, J. E. Cummings, L. Cannon-Albright, J. D. Schiffman

Research output: Contribution to journalReview article

24 Scopus citations

Abstract

Ewing's sarcoma is a highly malignant tumor of children and young adults. The molecular mechanisms that underlie Ewing's Sarcoma development are beginning to be understood. For example, most cases of this disease harbor somatic chromosomal translocations that fuse the EWSR1 gene on chromosome 22 with members of the ETS family. While some cooperative genetic events have been identified, such as mutations in TP53 or deletions of the CDKN2A locus, these appear to be absent in the vast majority of cases. It is therefore uncertain whether EWS/ETS translocations are the only consistently present alteration in this tumor, or whether there are other recurrent abnormalities yet to be discovered. One method to discover such mutations is to identify familial cases of Ewing's sarcoma and to then map the susceptibility locus using traditional genetic mapping techniques. Although cases of sibling pairs with Ewing's sarcoma exist, familial cases of Ewing's sarcoma have not been reported. While Ewing's sarcoma has been reported as a 2nd malignancy after retinoblastoma, significant associations of Ewing's sarcoma with classic tumor susceptibility syndromes have not been identified. We will review the current evidence, or lack thereof, regarding the potential of a heritable condition predisposing to Ewing's sarcoma.

Original languageEnglish (US)
Article number397632
JournalJournal of Oncology
DOIs
StatePublished - May 6 2010
Externally publishedYes

ASJC Scopus subject areas

  • Oncology

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    Randall, R., Lessnick, S. L., Jones, K. B., Gouw, L. G., Cummings, J. E., Cannon-Albright, L., & Schiffman, J. D. (2010). Is there a predisposition gene for ewing's sarcoma? Journal of Oncology, [397632]. https://doi.org/10.1155/2010/397632