Molecular biology of the Ewing's sarcoma/primitive neuroectodermal tumor family

Ewing's sarcoma (ES) and primitive neuroectodermal tumor (PNET) are members of a tumor family consistently associated with chromosomal translocation a nd functional fusion of the EWS gene to any of several structurally related transcription factor genes. Similar gene fusion events occur in other mese nchymal and hematopoietic tumors and are tumor-specific. The resulting nove l transcription factor-like chimeric proteins are believed to contribute to tumor biology by aberrant regulation of gene expression altering critical controls of cell proliferation and differentiation. These tumor-specific mo lecular rearrangements are useful for primary diagnosis, may provide progno stic information, and present potential therapeutic targets. The recent adv ances in our understanding of the molecular biology of ES and PNET represen t a paradigm for the application of the basic biology of neoplasia to clini cal management of patients. (C) 2000 by American Society of Clinical Oncolo gy.