title: 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.
authors.affiliation: Department of Radiology, Allegheny General Hospital, Pittsburgh, Pennsylvania, USA.
authors.forename: David
authors.initials: D
authors.lastname: Reisner
authors.affiliation: Department of Radiology, Allegheny General Hospital, Pittsburgh, Pennsylvania, USA.
authors.forename: Deborah
authors.initials: D
authors.lastname: Brahee
authors.affiliation: Department of Pathology, Allegheny General Hospital, Pittsburgh, Pennsylvania, USA.
authors.forename: Shweta
authors.initials: S
authors.lastname: Patel
authors.affiliation: Department of Radiology, Allegheny General Hospital, Pittsburgh, Pennsylvania, USA.
authors.forename: Matthew
authors.initials: M
authors.lastname: Hartman
mesh_terms: D000008:Abdominal Neoplasms / Q000175:diagnosis* / Q000473:pathology / Q000628:therapy; D000328:Adult; D003131:Combined Modality Therapy; D058405:Desmoplastic Small Round Cell Tumor / Q000175:diagnosis* / Q000473:pathology / Q000628:therapy; D003937:Diagnosis, Differential; D003952:Diagnostic Imaging; D006801:Humans; D008297:Male; D016896:Treatment Outcome
keywords: abdominal mass; chromosomal translocation; desmoplastic small round cell tumor; round cell tumor; soft tissue sarcoma