DSRCT

Desmoplastic small round cell tumor is a highly aggressive tumor that frequently occurs as multiple masses in the abdomen. DSRCT is classified as a soft tissue sarcoma that predominately affects boys and young adults. DSRCT in females is extremely rare but has been identified in uterine and ovarian cancers.

The cell of origin for this tumor is unclear. The tumor shows characteristics of epithelial, mesenchymal and neural differentiation. A characteristic translocation between chromosome 11 and 22 has been associated with desmoplastic small round cell tumors involving the EWS and WT1 genes. How this translocation contributes to the pathogenesis of this tumor is under active study. However, the identification of this gene abnormality has been very useful in the confirming the diagnosis of this tumor.

Because this is a rare cancer with few known treatment options, this Wiki is intended to gather together information from a variety of patients.