Unusual Aspects of Desmoplastic Small Round Cell Tumor

• Published in: Ultrastructural pathology Vol. 28; no. 2; pp. 83 - 96
• Main Authors: Bosman, Cesare, Boldrini, Renata
• Format: Journal Article
• Language: English
• Published: England Informa UK Ltd 01-03-2004; Taylor & Francis
• Subjects: Actins - ultrastructure; Adolescent; Carcinoma, Small Cell - metabolism; Carcinoma, Small Cell - pathology; Carcinoma, Small Cell - ultrastructure; Collagen - ultrastructure; Desmin - ultrastructure; desmoplasia; desmoplastic small round cell tumor (DSRCT); Humans; Immunohistochemistry; Immunophenotyping; intracellular fibrillogenesis; Male; Microscopy, Electron, Transmission; Retroperitoneal Neoplasms - metabolism; Retroperitoneal Neoplasms - pathology; Retroperitoneal Neoplasms - ultrastructure; Soft Tissue Neoplasms - metabolism; Soft Tissue Neoplasms - pathology; Soft Tissue Neoplasms - ultrastructure; WT1 Proteins - metabolism
• ISSN: 0191-3123; 1521-0758
• DOI: 10.1080/01913120490430634

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.