Desmoplastic small round cell tumor

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

They are usually seen in young adolescents and has male predominance with a mean survival of 2-3 years.

It 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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Article information

rID: 26748
Tag: refs, cases
Synonyms or Alternate Spellings:
  • Desmoplastic small round cell tumor (DSRCT)

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