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Desmoplastic small round cell tumor (DSRCT) is a rare type of soft tissue cancer (sarcoma) that usually begins in the abdomen. It primarily affects children and young adults and is more common in males. It is formed by small, round cancer cells surrounded by scar-like tissue and is often found in the tissue (peritoneum) that lines the inside of the abdomen and pelvis. The tumor cells have a characteristic genetic change involving a translocation between chromosomes 11 and (https://ghr.nlm.nih.gov/chromosome/22), which is important in differentiating from other similar tumors. The genetic change involved in DSRCT is acquired throughout a person's lifetime and is not inherited.
Symptoms may include abdominal pain and a feeling of fullness, abdominal mass, and symptoms of gastrointestinal obstruction, such as constipation. Treatment options may include: chemotherapy, radiation therapy, surgery, and stem cell transplantation.
Source: GARD Last updated on 05-01-20
The Liddy Shriver Sarcoma Initiative offers an information page on Desmoplastic small round cell tumor. Please click on the link to access this resource.
Last updated on 04-27-20
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