Desmoplastic Small Round Cell Tumors (DSRCTs)

Desmoplastic Small Round Cell Tumors (DSRCTs)

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

United in Care: (From left) Pediatric surgeon Michael La Quaglia talks with sarcoma experts Paul Meyers and Leonard Wexler.

What are desmoplastic small round cell tumors?

Desmoplastic small round cell tumors (DSRCTs, sometimes also referred to as small blue round cell tumor) are a type of sarcoma that usually develops in the belly or pelvis. They are most often found in white teenagers and young men between age 10 and 30. DSRCTs may cause pain or a mass in the belly. They are very rare, affecting only about 20 young people in the United States each year.

We Understood This Rare Tumor First

MSK doctors have long been leaders in the care of people with DSRCTs. We first described this type of tumor in 1989. We then identified a unique genetic defect that is now used to diagnosis the cancer: EWS-WT1, which is the abnormal fusion of two genes. By identifying this genetic marker, doctors around the world can diagnose DSRCTs accurately.

This information is about sarcoma in children.

Learn about soft tissue sarcoma in adults here.

The World’s Largest DSRCT Program

The MSK Kids team has cared for more than 100 patients with DSRCTs — more than at any other hospital in the world. Our teams have published the largest reports on people with DSRCTs who have been treated with chemotherapy, surgery, radiation therapy, and immunotherapy. When you bring your child to MSK Kids for DSRCT care, they will have access to all of these treatments, delivered by leaders who have more experience than anyone caring for these young patients.