Desmoplastic small round cell tumor (peritoneal)
Updates to Article Attributes
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.
Clinical presentation
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.
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 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.
Differential diagnosis
See also
-<li>malignant peritoneal mesothelioma</li>- +<li><a title="peritoneal mesothelioma" href="/articles/peritoneal-mesothelioma">malignant peritoneal mesothelioma</a></li>
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