Open AccessIntra‐Abdominal Desmoplastic Small Round Cell Tumor with Elevated Serum CA 125: A Case Report
Author(s) -
Yang SheauFang,
Wang ShengLan,
Chai CheeYin,
Su YuChieh,
Fu OuYang,
Chen ChauYun
Publication year - 2003
Publication title -
the kaohsiung journal of medical sciences
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.439
H-Index - 36
eISSN - 2410-8650
pISSN - 1607-551X
DOI - 10.1016/s1607-551x(09)70503-0
Subject(s) - desmoplastic small round cell tumor , histogenesis , pathology , medicine , sarcoma , cancer research , immunohistochemistry
Desmoplastic small round cell tumor (DSRCT) is a rare and highly aggressive tumor usually involving the peritoneum. It occurs more commonly in young males and is characterized by distinctive clinical, histologic, and immunophenotypic features. The histogenesis of DSRCT remains unknown. Coexpression of epithelial, mesenchymal, and neural antigens in the same cell provides evidence that DSRCT may arise from a primitive pluripotent stem cell with divergent differentiation. Recently, according to cytogenetic studies, some authors have proposed that the divergent differentiation of DSRCT may be the result of the fusion of Ewing's sarcoma gene and Wilms' tumor suppressor gene. Clinically, an elevated serum CA 125 concentration is found in some patients with DSRCT. We present the case of a 29‐year‐old man with diffuse intra‐abdominal DSRCT and elevated serum CA 125 concentration and briefly review the relevant literature.