title: Unusual aspects of desmoplastic small round cell tumor.
abstract: Desmoplastic small round cell tumor (DSRCT) is a neoplasia that occurs mainly in childhood and involves abdominal or peritoneal sites, coexpressing ectodermal and mesenchimal immunophenotypic markers, and is endowed with an impressive stromal desmoplasia that tends to decrease on tumor relapse. To date, over 150 cases have been collected in the literature. Its presumed neuroectodermal histogenesis has been challenged by cytogenetic findings different from those usually associated with neoplasms of neuroectodermal origin. The authors report a case bearing clinical and histologic aspects of typical desmoplastic retroperitoneal small cell tumor, with intense and diffuse nuclear immunopositivity for WT1, but lacking divergent immunophenotype. Ultrastructural investigation revealed that desmoplasia could result from fibrillary synthesis by neoplastic cells.
authors.affiliation: Department of Experimental Medicine and Pathology, La Sapienza University, Rome, Italy.
authors.forename: Cesare
authors.initials: C
authors.lastname: Bosman
authors.forename: Renata
authors.initials: R
authors.lastname: Boldrini
mesh_terms: D000199:Actins / Q000648:ultrastructure; D000293:Adolescent; D018288:Carcinoma, Small Cell / Q000378:metabolism / Q000473:pathology* / Q000648:ultrastructure; D003094:Collagen / Q000648:ultrastructure; D003893:Desmin / Q000648:ultrastructure; D006801:Humans; D007150:Immunohistochemistry; D016130:Immunophenotyping; D008297:Male; D046529:Microscopy, Electron, Transmission; D012186:Retroperitoneal Neoplasms / Q000378:metabolism / Q000473:pathology* / Q000648:ultrastructure; D012983:Soft Tissue Neoplasms / Q000378:metabolism / Q000473:pathology* / Q000648:ultrastructure; D025721:WT1 Proteins / Q000378:metabolism
chemical_list: D000199:Actins; D003893:Desmin; D025721:WT1 Proteins; D003094:Collagen