Desmoplastic Small Round Cell Tumor

desmoplastic small round cell tumor


What is a Desmoplastic Small Round Cell Tumor?

Desmoplastic small round cell tumor (DSRCT) is a type of cancer. It is usually found in the abdomen (belly), but it can also occur in other parts of the body. Tumors are formed by small, round cancer cells and are surrounded by scarlike tissue. The tumor has a specific genetic abnormality that helps confirm the diagnosis. DSRCT in the abdomen may not be found until the tumors have grown large. As a result, often the disease has spread to the liver, lymph nodes, lungs or bones by the time it is diagnosed.

Diagnosis of Desmoplastic Small Round Cell Tumor

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 for Desmoplastic Small Round Cell Tumor

DSRCT is a very aggressive neoplasm with a 5-year survival of less than 15%. Treatment options include surgery, radiotherapy, chemotherapy with or without stem cell transplantation, and recently introduced molecularly targeted therapies. Unfortunately there is no standard therapeutic regimen described since no modality is clearly superior to any other. Surgery is usually extensive and often includes excision of the omentum, splenectomy and lymph node resections. Due to the invasive nature of this tumor, complete resection with negative margins is usually not possible. Debulking surgery has been described as an attempt to eliminate 90% of the tumor bulk