Desmoplastic small round cell tumour

Desmoplastic small round cell tumour (DSRCT) is a rare and highly aggressive primary peritoneal malignancy. It is seen usually in young adoloscent 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, retrovesical or rectouterine space being the most frequent location. Tunica vaginalis of the testis is 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 heterogenously on contrast studies. Necrosis, haemorrhage and fibrous components are common.

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

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Article information

rID: 26748
Section: Pathology
Tags: stub, refs, cases
Synonyms or Alternate Spellings:

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