Desmoplastic Small Round Cell Tumors

MSK pediatric surgeon Michael La Quaglia talks with sarcoma experts Paul Meyers and Leonard Wexler.

Desmoplastic small round cell tumors (DSRCT) are aggressive tumors that typically begin in the abdomen or pelvis. The most common symptoms include pain or a mass in the abdomen. DSRCT usually occurs in white adolescent and young men between the ages of ten and 30.

DSRCT is a kind of tumor called a sarcoma. In general, sarcomas are cancers in bones, muscles, and connective tissue throughout the body. They can affect children, adolescents, and young adults, even up to age 40. Our experts regularly treat young patients with types of sarcomas that many other healthcare professionals rarely see, including DSRCT.

Memorial Sloan Kettering pathologists William Gerald and Juan Rosai first described DSRCT in 1989. They then defined the unique genetic defect associated with the cancer, called the EWS-WT1 fusion gene, along with MSK pathologist Marc Ladanyi.

Since then, MSK’s Department of Pediatrics has cared for around 100 patients with DSRCT — more than any other hospital in the world. Our team has led the field in developing treatments for this disease, which affects about 20 young people in the United States each year. We have also published the largest reports of patients treated with different methods, including chemotherapy, surgery, radiation therapy, and immunotherapy.