Desmoplastic small round cell tumor (peritoneal)
Updates to Article Attributes
Desmoplastic small round cell tumours are a rare and highly aggressive primary peritoneal malignancy. It is
Epidemiology
They are usually seen usually in young adoloscentadolescents 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 heterogenouslyheterogeneously 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.
Differential diagnosis
- peritoneal carcinomatosis
- non
Hodgkin-Hodgkin lymphoma - malignant peritoneal mesothelioma
- rhabdomyosarcoma
See also
-<p><strong>Desmoplastic small round cell tumour (DSRCT)</strong> 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.</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 heterogenously on contrast studies. Necrosis, haemorrhage and fibrous components are common.</p><p>Peritoneal seeding, lymph nodal involvement, liver and bone metastases are 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 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>
-<li><a href="/articles/non-hodgkin-lymphoma">non Hodgkin lymphoma</a></li>-<li><a title="peritoneal mesothelioma" href="/articles/peritoneal-mesothelioma">malignant peritoneal mesothelioma</a></li>- +<li><a href="/articles/non-hodgkin-lymphoma">non-Hodgkin lymphoma</a></li>
- +<li><a href="/articles/peritoneal-mesothelioma">malignant peritoneal mesothelioma</a></li>
-</ul><h4>See also</h4><ul><li><a href="/articles/small-round-blue-cell-tumours">small round blue cell tumors</a></li></ul>- +</ul><h4>See also</h4><ul><li><a href="/articles/small-round-blue-cell-tumours">small round blue cell tumours</a></li></ul>
Sections changed:
Systems changed:
- Oncology
Unable to process the form. Check for errors and try again.