Intra-abdominal desmoplastic small round cell tumor with elevated serum CA 125: a case report.
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.
Yang
Sheau-Fang
SF
Department of Pathology, Kaohsiung Medical University Chung-Ho Memorial Hospital, Kaohsiung, Taiwan.
Wang
Sheng-Lan
SL
Chai
Chee-Yin
CY
Su
Yu-Chieh
YC
Fu
Ou-Yang
OY
Chen
Chau-Yun
CY
D000008:Abdominal Neoplasms / Q000097:blood / Q000473:pathology*; D000328:Adult; D018394:CA-125 Antigen / Q000097:blood*; D018288:Carcinoma, Small Cell / Q000097:blood / Q000473:pathology*; D006801:Humans; D008297:Male; D015514:Oncogene Proteins, Fusion / Q000235:genetics
D018394:CA-125 Antigen; C106032:EWS1-WT1 fusion protein, human; D015514:Oncogene Proteins, Fusion