Desmoplastic small round cell tumor (peritoneal)
Updates to Article Attributes
Desmoplastic small round cell tumours are a rare and highly aggressive primary peritoneal malignancy.
Epidemiology
They are usually seen in young adolescents 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 the retrovesical or rectouterine space being the most frequent locationlocations. TunicaThe 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 a common modemodes of spread.
Differential diagnosis
See also
-<p><strong>Desmoplastic small round cell tumours</strong> are a rare and highly aggressive <a title="Primary peritoneal malignancy" href="/articles/primary-peritoneal-neoplasms">primary peritoneal malignancy</a>.</p><h4>Epidemiology</h4><p>They are usually seen in young adolescents and has male predominance with a mean survival of 2-3 years.</p><h4>Clinical presentation</h4><p>It usually presents with a palpable abdominal mass and abdominal distension with discomfort.</p><p>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.</p><h4>Radiographic features</h4><h5>CT</h5><p>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.</p><p>Peritoneal seeding, lymph nodal involvement, liver and bone metastases are a common mode of spread.</p><h4>Differential diagnosis</h4><ul>- +<p><strong>Desmoplastic small round cell tumours</strong> are a rare and highly aggressive <a href="/articles/primary-peritoneal-neoplasms">primary peritoneal malignancy</a>.</p><h4>Epidemiology</h4><p>They are usually seen in young adolescents and has male predominance with a mean survival of 2-3 years.</p><h4>Clinical presentation</h4><p>It usually presents with a palpable abdominal mass and abdominal distension with discomfort.</p><p>It is most commonly seen to arise from the pelvic peritoneal cavity, the retrovesical or rectouterine space being the most frequent locations. The <a title="Tunica vaginalis (testis)" href="/articles/tunica-vaginalis-testis">tunica vaginalis</a> of the testis is the next most common location.</p><h4>Radiographic features</h4><h5>CT</h5><p>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.</p><p>Peritoneal seeding, lymph nodal involvement, liver and bone metastases are common modes of spread.</p><h4>Differential diagnosis</h4><ul>
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