DSRCT; Desmoplastic small round-cell tumor

Desmoplastic small round cell tumor (DSRCT) is a rare, highly aggressive type of soft tissue cancer (sarcoma) that usually begins in the abdomen. It primarily affects children and young adults and is more common in males. It is formed by small, round cancer cells surrounded by scar-like tissue and is often found in the tissue (peritoneum) that lines the inside of the abdomen and pelvis.

DSRCT often is not diagnosed until the cancer is advanced, tumors grow large and spread through the lymph system or blood stream to other parts of the body. They can spread to many areas of the body, including the lymph nodes, lungs, bone, and liver.

Diagnostic testing: A diagnosis of DSRCT is usually made by a combination of the histologic appearance and immunohistochemical staining results from a biopsy specimen.

Other testing may include:

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  Cytogenic analysis; and

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  Fluorescence in situ hybridization (FISH).

Physical findings:

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  Abdominal pain and a feeling of fullness;

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  Abdominal distention;

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  Abdominal mass;

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  Hepatomegaly (enlarged liver);

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  Nausea and vomiting;

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  Diarrhea;

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  Ileus (obstruction of ileum or other part of the intestine); and

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  Symptoms of gastrointestinal obstruction, such as constipation.

ICD-9: 158

DSRCT typically arises in serous lined surfaces of the abdominal or pelvic peritoneum, and spreads to the omentum, lymph nodes, and hematogenously (carried by blood) disseminates especially to the liver.

Treatment of DSRCT may include chemotherapy, hyperthermic intraperitoneal chemotherapy (HIPEC), radiation therapy, surgery, and stem cell transplantation. DSRCT is often resistant to treatment and frequently recurs. Despite aggressive therapy, 3-year overall survival has been estimated at 44% and the 5-year survival rate remains around 15%.

SUGGESTED PROGRAMMATIC ASSESSMENT*

Suggested MER for Evaluation:

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  Clinical history and examination that describes diagnostic features of the impairment;

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  Results of cytogenic analysis or FISH testing; and

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  Results of immunohistochemical staining.

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