title: Desmoplastic small round cell tumor 20 years after its discovery.
abstract: Desmoplastic small round cell tumor (DSRCT) was proposed as a distinct disease entity by William L Gerald and Juan Rosai in 1991. Over 850 patients have been reported in the medical literature. A specific translocation, t(11;22)(p13;q12), is seen in almost all cases, juxtaposing the EWS gene to the WT1 tumor suppressor gene. DSRCT is composed of nests of small round cells with polyphenotypic differentiation, typically a mixture of epithelial, mesenchymal and neural features, surrounded by a prominent desmoplastic stroma. DSRCT has a predilection for adolescent and young adult males, and primarily involves the abdominal cavity and pelvis. Survival is low despite their initial response to multimodal treatment. Most patients relapse with disseminated disease that is unresponsive to further therapy.
authors: Department of Pediatric Oncology, Hospital Sant Joan de Déu, Barcelona, Spain.
authors: Jaume
authors: J
authors: Mora
authors: Shakeel
authors: S
authors: Modak
authors: Nai-Kong
authors: NK
authors: Cheung
authors: Paul
authors: P
authors: Meyers
authors: Enrique
authors: E
authors: de Alava
authors: Brian
authors: B
authors: Kushner
authors: Heather
authors: H
authors: Magnan
authors: Oscar M
authors: OM
authors: Tirado
authors: Michael
authors: M
authors: Laquaglia
authors: Marc
authors: M
authors: Ladanyi
authors: Juan
authors: J
authors: Rosai
mesh_terms: D000008:Abdominal Neoplasms / Q000266:history* / Q000473:pathology / Q000628:therapy; D058405:Desmoplastic Small Round Cell Tumor / Q000266:history* / Q000473:pathology / Q000628:therapy; D049673:History, 20th Century; D049674:History, 21st Century; D006801:Humans
keywords: EWS gene; WT1 gene; William L Gerald; adolescent and young adult oncology; desmoplastic tumors; developmental cancer; small round cell tumors