Desmoplastic small round cell tumor (peritoneal)
Updates to Article Attributes
Desmoplastic small round cell tumours of the peritoneum are a rare and highly aggressive primary peritoneal malignancy.
Epidemiology
They areDesmoplastic small round cell tumour is usually seen in young adolescents and hashave a male predominance with a mean survival of 2-3 years.
Clinical presentation
ItA 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.
Differential diagnosis
See also
-<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>- +<p><strong>Desmoplastic small round cell tumours</strong> of the <strong>peritoneum</strong> are a rare and highly aggressive <a href="/articles/primary-peritoneal-neoplasms">primary peritoneal malignancy</a>.</p><h4>Epidemiology</h4><p>Desmoplastic small round cell tumour is usually seen in young adolescents and have a male predominance with a mean survival of 2-3 years.</p><h4>Clinical presentation</h4><p>A desmoplastic small round cell tumour 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 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>
-<li><a href="/articles/peritoneal-mesothelioma">malignant peritoneal mesothelioma</a></li>- +<li><a href="/articles/malignant-peritoneal-mesothelioma">malignant peritoneal mesothelioma</a></li>
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