This information is provided by the National Institutes of Health (NIH) Genetic and Rare Diseases Information Center (GARD).
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 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.
For more information, visit GARD.