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Desmoplastic small round cell tumor (DSRCT) is an aggressive, rare cancer most often presenting as multiple masses in abdomen of young males. The characteristic clinical presentation of abdominal pain and mass, with radiology featuring multifocal abdominal masses, and pathology demonstrating unique chromosomal translocation (t11;22) (p13:q12) and EWSRI positivity, is diagnostic for DSRCT. Typical imaging findings include multiple heterogeneous, lobular abdominal masses, which can grow very large. Often there is a dominant mass with additional peritoneal, omental, retroperitoneal and retrovesical masses.






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A Case of Desmoplastic Small Round Cell Tumor

Free full text article: A Case of Desmoplastic Small Round Cell Tumor

Abstract
Desmoplastic small round cell tumor is a rare, aggressive tumor primarily affecting young males. It is considered a childhood cancer, and is characterized by a unique chromosomal translocation which leads to failure to suppress tumor growth. It is classified as a soft tissue sarcoma, sharing some features with other small round cell tumors such as Ewing`s Sarcoma and primitive neuroectodermal tumor. Typical imaging findings include multiple heterogeneous, lobular abdominal masses, which can grow very large. Often there is a dominant mass with additional peritoneal, omental, retroperitoneal and retrovesical masses. Prognosis is relatively poor with a 3 year survival rate of 50% in those treated aggressively with surgical resection, chemotherapy, and radiation therapy. The clinical presentation, imaging characteristics and pathology are discussed in regards to a recent case.






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