There are no known risk factors that have been identified specific to the disease. The tumor appears to arise from the primitive cells of childhood, and is considered a childhood cancer. Research has indicated that there is a chimeric relationship between<unk>  and Wilms' tumor and Ewing sarcoma. Together with neuroblastoma and non-Hodgkin's lymphoma, they form the small-cell tumors.<unk>  is associated with a unique chromosomal translocation (notated as t(11;22)(p13:q12)) that merges the EWSR1 FET family gene normally located on band 12 of the long (or "q") arm of chromosome 22 with part of the WT1 transcription factor gene normally located on band 13 of the short arm of chromosome 11. The resulting EWSR1-WT1 fusion gene is converted to a fusion transcript that directs the formation of an EWSR1-WT1 chimeric protein. The EWSR1-WT1 chimeric protein contains the N-terminal transactivation domain of EWSR1 and the DNA-binding domain of WT1. This translocation is seen in virtually all cases of<unk> . The EWS/WT1 translocation product targets ENT4. ENT4 is also known as PMAT.
Desmoplastic small-round-cell tumor
Plexiform angiomyxoid myofibroblastic tumor
Perivascular epithelioid cell tumour
Adenomatoid tumor
Solid pseudopapillary tumour
Sclerosing epithelioid fibrosarcoma
Small-blue-round-cell tumor
Multinucleate cell angiohistiocytoma