A case of desmoplastic small round cell tumor

David Reisner, Deborah Brahee, Shweta Patel, Matthew Hartman

Research output: Contribution to journalArticlepeer-review

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.

Original languageEnglish (US)
Pages (from-to)1-7
Number of pages7
JournalJournal of Radiology Case Reports
Volume9
Issue number8
DOIs
StatePublished - Aug 1 2015

ASJC Scopus subject areas

  • Radiology Nuclear Medicine and imaging

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