Desmoplastic small round cell tumors are rare, affecting only about 20 young people in the United States each year, so most pediatric cancer teams have never treated them. Miami Cancer Institute works with Memorial Sloan Kettering Alliance, and our Miami partner, Nicklaus Children’s Hospital, to provide access to advanced expertise, treatment and support for children and families affected by these tumors. Our pediatric cancer care team is experienced in working with children and families affected by rare and serious diseases, and we offer child-friendly care and support in a nurturing, nonthreatening environment.
What are desmoplastic small round cell tumors?
Desmoplastic small round cell tumors (DSRCT) are extremely rare but often aggressive tumors that usually start in the abdomen or pelvis. They are a type of sarcoma. DSRCT is formed by small, round cancer cells surrounded by scar-like tissue, usually in the peritoneum, the tissue that lines the inside of the abdomen and pelvis. DSRCT is linked to a genetic change in certain chromosomes.
What are the risk factors for desmoplastic small round cell tumors?
DSRCT is most common in males from 10 to 30 years old. It is linked to a genetic mutation in which genetic material is exchanged between two specific chromosomes. Wilms’ tumor and Ewing sarcoma may be associated with DSRCT, but the relationship is not clear.