A Case of Desmoplastic Small Round Cell Tumor

Authors

  • David Reisner
  • Deborah Brahee
  • Schweta Patel
  • Matthew Hartman

DOI:

https://doi.org/10.3941/jrcr.v9i8.2526

Keywords:

soft tissue sarcoma, abdominal mass, chromosomal translocation, desmoplastic small round cell tumor, 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.

Published

2015-08-27

Issue

Section

General Radiology