Desmoplastic small round cell tumors (DSRCT) are a type of soft-tissue sarcoma. It causes multiple tumors in the abdomen and pelvis area, but exactly where it begins usually is not known.
The first case of a DSRCT was recorded in 1989, and about 200 cases have been diagnosed since then. The disease is most common in Caucasian boys. Although researchers think a problem with the genes may cause DSRCT, this has not been proven.
Since DSRCT often is not diagnosed until the cancer is advanced, tumors grow large and spread through the lymph system or blood stream to other parts of the body. They can spread to many areas of the body, including the lymph nodes, lungs, bone and liver. Other types of cancer, including rhabdomyosarcoma, may be in the abdomen as well.
DSRCT is a dangerous type of cancer that often is resistant to treatment and frequently comes back after treatment.
In rare cases, DSRCT may be passed down from one generation to the next. Genetic counseling may be right for you. Visit our genetic testing page to learn more.