Highlighting the Diversity of Desmoplastic Small Round Cell Tumor: A Case Series. (April 2023)

• Record Type: Journal Article
• Authors: Asadbeigi, Sepideh Nikki; Diaz-Perez, Julio A.; Rosenberg, Andrew E.; Pettus, Jason R.; Kerr, Darcy A.; Linos, Konstantinos
• Is Part Of: International journal of surgical pathology. Volume 31:Number 2(2023)
• Page Start: 142
• Page End: 151
• Publication Date: 2023-04
• Subjects: desmoplastic small round cell tumor -- DSRCT -- sarcoma -- kidney -- unusual morphology -- immunohistochemistry; Pathology, Surgical -- Periodicals; 617.07
• DOI: 10.1177/10668969221095178
• Languages: English
• ISSNs: 1066-8969

Abstract

Desmoplastic small round cell tumor (DSRCT) is a rare malignant tumor that occurs mainly in the retroperitoneum of children and young adults. In its prototypical form, DSCRT displays characteristic morphology with nested primitive small round cells in a desmoplastic stroma and a distinctive immunophenotype with polyphenotypic differentiation. However, DSCRT can also exhibit a broader clinical, histologic and immunohistochemical spectrum and, therefore, cause diagnostic difficulties. Given that DSCRT is an aggressive and nearly universally fatal disease, making the correct diagnosis is critically important. Herein, we report three patients with DSRCT and unusual clinical, morphologic or immunohistochemical characteristics, in order to highlight its remarkable diversity and increase awareness of this unusual, distinctive neoplasm.