Desmoplastic small round cell tumours of the peritoneum are a rare and highly aggressive primary peritoneal malignancy.
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Epidemiology
Desmoplastic small round cell tumour is usually seen in young adolescents and have a male predominance with a mean survival of 2-3 years.
Clinical presentation
A desmoplastic small round cell tumour 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.
Radiographic features
CT
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 common modes of spread.
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