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Genetic and Rare Diseases Information Center (GARD)

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Desmoplastic small round cell tumor

Other Names for this Disease
  • Desmoplastic small round-cell tumor
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Your Question

I am trying to find studies discussing the potential of genetic inheritance of desmoplastic small round cell tumor.  My friend's brother is now suffering from desmoplastic small round cell tumor and she is concerned about her risks to develop this condition.

Our Answer

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What is desmoplastic small round cell tumors (DSRCT)?

Desmoplastic small round cell tumors (DSRCT), a rare malignant cancer, is a soft tissue sarcoma that usually affects young boys and men and is found most often in the abdomen. Its name means that it is formed by small, round cancer cells surrounded by scarlike tissue. The most common symptoms include abdominal pain, abdominal mass and symptoms of gastrointestinal obstruction.[1] DSRCTs are treated first with chemotherapy, then with surgery to remove the tumor, if possible. Radiation therapy is sometimes given, depending on the tumor. In addition, some people with DSRCT are candidates for a bone marrow transplant.[2]
Last updated: 6/19/2009

Is desmoplastic small round cell tumor (DSRCT) genetic? Does it run in families?

The tumor cells in DSRCT have a specific reciprocal translocation between chromosomes number 11 and 22, the breakpoints involve the EWS gene on 22q12 and the WT1 gene on 11p13 and is identified as t(11;22)(p13;q12).  The chromosome finding is only in the tumor cells.  Familial clustering of DSRCT has not been reported. There is no increased risk in siblings or offspring.[3]
Last updated: 6/22/2009