Desmoplastic small round cell tumours 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, 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 heterogeneously on contrast studies. Necrosis, haemorrhage and fibrous components are common.
Peritoneal seeding, lymph nodal involvement, liver and bone metastases are a common mode of spread.
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