Pediatric Sarcomas: Desmoplastic Small Round Cell Tumors

Desmoplastic small round cell tumor (DSRCT), a rare type of soft tissue sarcoma, is an aggressive tumor that typically occurs in the abdomen and pelvis of an adolescent or young adult. The most common symptoms include pain or a mass in the abdomen. The origin of DSRCT in the body may not be known.

DSRCT was first described in 1989 by Memorial Sloan Kettering pathologists William Gerald and Juan Rosai. The unique genetic abnormality associated with DSRCT, the EWS-WT1 transcription factor, was then characterized by Drs. Gerald and Rosai, as well as pathologist Marc Ladanyi.(1)

Memorial Sloan Kettering physicians have led the field in developing treatment strategies for this disease, and have published the largest reports of patients treated with different methods, including chemotherapy, surgery, and radiation therapy.(2)

Diagnosis

When our doctors suspect that a patient has DSRCT, the diagnosis is confirmed with a biopsy of the affected tissue. Pathologists examine the tissue for the specific genetic abnormality only found in DSRCT.

Because the disease can spread widely throughout the body, our experts often examine the bone marrow and perform imaging studies such as CT, MRI, and PET scans to determine how far the tumor has spread.

Treatment

Our experts treat patients with DSRCT using many approaches. First, we use chemotherapy to shrink the tumor and to prevent new tumors from forming. We have a clinical trial for newly diagnosed patients, which combines the standard chemotherapy used to treat this tumor with the drugs irinotecan, temozolomide, and bevacizumab.

After several months of chemotherapy, we perform surgery to remove all sites of tumor. Patients then continue with additional chemotherapy. For many of our patients, radiation therapy is also used to treat DSRCT after chemotherapy is completed.

We are also investigating an innovative therapy that uses the 8H9 antibody to deliver radiation directly to tumor cells without damaging normal cells. A patient may qualify for this trial as part of initial therapy or if his or her cancer recurs.

We are hopeful that our novel approaches to treating DSRCT will improve the outcomes for patients with this aggressive disease.