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Desmoplastic Small Round Cell Tumor

Also called: 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:

  • Pain or a lump in the abdomen
  • Cramping
  • Nausea
  • Vomiting
  • Diarrhea
  • Constipation
  • Trouble having a bowel movement and/or passing gas
  • Abdominal swelling

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:

  • Surgery — is used to remove as much of the cancer as possible. Often, DSRCTs have spread too far for complete removal, but surgeons try to remove at least 90 percent of them.
  • Hyperthermic intraperitoneal chemotherapy (HIPEC) — may be given during the surgery to kill cancer cells that cannot be removed surgically. (The patient also avoids the side effects of standard chemotherapy.)
    • HIPEC is done by circulating a heated, sterile chemo solution through the part of the abdomen where the tumors are found, for up to two hours.
  • Chemotherapy (“chemo”) — uses powerful medicines to kill cancer cells or stop them from growing (dividing) and making more cancer cells.
    • Chemo may be injected into the bloodstream, so that it can travel throughout the body.
    • Some chemo may be given by mouth.
    • Combination therapy uses more than one type of chemo at a time.
  • Radiation therapy — uses high-energy X-rays or other types of radiation to kill cancer cells or stop them from growing.
    • External radiation uses machines outside the body to deliver the X-ray dose.
    • Internal radiation uses needles, seeds, wires or catheters to deliver the radiation directly into or close to the cancer.

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?

  • St. Jude is the only National Cancer Institute-designated Comprehensive Cancer Center devoted solely to children.
  • St. Jude has created more clinical trials for cancer than any other children’s hospital in the United States.
  • The nurse-to-patient ratio at St. Jude is unmatched— averaging 1:3 in hematology and oncology, and 1:1 in the Intensive Care Unit.

Associated Clinical Trials

ANGIO-A: Study of Cyclophosphamide, Sorafenib, Bevacizumab, and Atezolizumab in Children and Young Adults with Solid Tumors

Safety and Tolerability of Oral Cyclophosphamide and Sorafenib with Intravenous Bevacizumab with the Addition of Atezolizumab in Pediatric Solid Tumor Patients

Diseases Treated:

Solid tumors
Hepatocellular carcinoma
Fibrolamellar hepatocellular carcinoma
Malignant rhabdoid tumors
Desmoplastic small round cell tumors


Part 1

  • 1–30 years old
  • Diagnosis of a solid tumor that has grown or has come back after treatment
  • Tumor accessible through biopsy

Part 2

  • 1–30 years old
  • Diagnosis of:
    • Hepatocellular carcinoma that has grown or has come back after treatment
    • Fibrolamellar hepatocellular carcinoma
    • Desmoplastic small round cell tumor
    • Malignant rhabdoid tumor that is not in the central nervous system
  • Tumor accessible through biopsy
View Trial

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