Disease Information

Solid Tumor: Desmoplastic Small Round Cell Tumor

Alternate Names: DSRCT, polyphenotypic 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.

How common is desmoplastic small round cell tumor?

DSRCT is extremely rare. Fewer than 200 cases have been reported, about 85 percent of them in Caucasian people.

Boys and young men are about four times as likely to have DSRCT as girls and young women. It is usually diagnosed in males between ages 10 and 30.

What are the symptoms of desmoplastic small round cell tumor?

Symptoms of desmoplastic small round cell tumor include:

How is desmoplastic small round cell tumor treated?

Because DSRCT is so rare, no standard way to treat it has been developed. The following treatment methods have been used:

DSRCT located outside the abdomen without any spread seems to respond better to treatment than DSRCT in the abdomen or than DSRCT which has spread into other parts of the body.

What are the survival rates for desmoplastic small round cell tumor?

Because so few people have DSRCT, only a small amount of information on treatment results is available. Currently DSRCT has a 15 percent five-year survival rate. However, researchers are looking for treatments that will improve those odds.

Why choose St. Jude for your child’s desmoplastic small round cell tumor treatment?


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