Intra-Abdominal Desmoplastic Small Round Cell Tumor Report of a Case

Youichi Kumagai, Michio Iida, Yutaka Tohkairin, Takanori Ochiai, Shigeru Yamazaki, Hajime Odajima¹⁾ and Tohru Igari²⁾

Department of Surgery and Department of Pathology¹⁾, Ohta Nishinouchi Hospital
Department of Pathology, Tokyo Medical and Dental University²⁾

A 24-year-old man admitted for left abdominal pain, and previously found elsewhere to have intraabdominal tumors and liver metastasis was found in contrast-enhancement computed tomography (CT) to have a 90 mm tumor in the left abdomen and pelvis with multiple liver metastasis. No tumor causing peritoneal dissemination or liver metastasis was found, however, in endoscopic examination or PET. CA125 at 100 U/ml and Neuron-specific eolase (NSE) at 83.4 ng/ml were elevated. Unable to obtain a histological diagnosis with fine-needle aspiration cytology, we conducted open incisional biopsy and totally resected the omental tumor. The definitive diagnosis was desmoplastic small round cell tumor (DSRCT). The man was treated to 5 courses of high-dose chemotherapy based on the P6 regimen. Two courses of chemotherapy reduced tumors of abdomen and liver metastasis and decreased serum CA125 and NSE levels, but the man died 7 months after diagnosis due to tumor regrowth. In cases of abdominal tumor in young patients, DSRCT should be considered as a differential diagnosis.

Key words
desmoplastic small round cell tumor, P6 regimen, chemotherapy

Reprint requests
Youichi Kumagai Department of Surgery, Ohta Nishinouchi Hospital
2-5-20 Nishinouchi, Kohriyama, 963-8558 JAPAN

Accepted
December 17, 2008

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