Unusual aspects of desmoplastic small round cell tumor

Cesare Bosman, Renata Boldrini

Research output: Contribution to journalArticlepeer-review

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.

Original languageEnglish
Pages (from-to)83-96
Number of pages14
JournalUltrastructural Pathology
Volume28
Issue number2
DOIs
Publication statusPublished - Mar 2004

Keywords

  • Desmoplasia
  • Desmoplastic small round cell tumor (DSRCT)
  • Intracellular fibrillogenesis

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Instrumentation
  • Structural Biology

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