Desmoplastic small round cell tumor (peritoneal)

Last revised by Dalia Ibrahim on 10 May 2022

Desmoplastic small round cell tumors of the peritoneum are a rare and highly aggressive primary peritoneal malignancy.

Desmoplastic small round cell tumor is usually seen in young adolescents and have a male predominance with a mean survival of 2-3 years.

A desmoplastic small round cell tumor usually presents with a palpable abdominal mass and abdominal distension with discomfort.

It is most commonly seen to arise from the pelvic peritoneal cavity, the retrovesical or rectouterine space being the most frequent locations. The tunica vaginalis of the testis is the next most common location.

Solitary or multiple soft tissue masses are seen with no definite organ of origin, usually in the retrovesical or rectouterine space, which enhance heterogeneously on contrast studies. Necrosis, hemorrhage and fibrous components are common.

Peritoneal seeding, lymph nodal involvement, liver and bone metastases are common modes of spread.

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Cases and figures

  • Case 1
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  • Case 2: solitary mass-PET-CT
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