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Case Reports in Gastrointestinal Medicine
Volume 2013, Article ID 907136, 6 pages
http://dx.doi.org/10.1155/2013/907136
Case Report

Desmoplastic Small Round Cell Tumor of Stomach

1College of Medicine, Alfaisal University, P.O. Box 50927, Riyadh 11533, Saudi Arabia
2Department of General Surgery, Faculty of Medicine, Alexandria University, Alexandria 21526, Egypt
3Department of Pathology and Laboratory Medicine, King Faisal Specialist Hospital and Research Center (KFSH&RC), P.O. Box 3354, Riyadh 11211, Saudi Arabia
4Department of Surgical Oncology, King Faisal Specialist Hospital and Research Center (KFSH&RC), P.O. Box 3354, Riyadh 11211, Saudi Arabia

Received 27 April 2013; Accepted 27 May 2013

Academic Editors: S. Kikuchi and H. Sugimura

Copyright © 2013 Ahmed Abu-Zaid et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Desmoplastic small round cell tumor (DSRCT) is an extremely uncommon, highly aggressive, and malignant mesenchymal neoplasm of undetermined histogenesis. Less than 200 case reports have been documented in literature so far. Herein, we report a 26-year-old otherwise healthy female patient who presented with a 1-month history of epigastric pain. On physical examination, a palpable, slightly mobile, and tender epigastric mass was detected. All laboratory tests were normal. A chest, abdominal, and pelvic contrast-enhanced computed tomography (CT) scans showed a 3.8 × 7.2 × 8.7 cm ill-defined mass, involving gastric fundus and extending into gastric cardia and lower gastroesophageal junction. It was associated with multiple enlarged gastrohepatic lymph nodes; the largest measured 1.2 cm. There was no evidence of ascites or retroperitoneal or mesenteric lymphatic metastases. Patient underwent total gastrectomy with D2 lymphadenectomy, splenectomy, and antecolic Roux-en-Y esophagojejunal anastomosis. Histopathological examination revealed coexpression of mesenchymal, epithelial, and neural markers. The characteristic chromosomal translocation (t(11; 22)(p13; q12)) was demonstrated on fluorescence in situ hybridization (FISH) technique. Diagnosis of DSRCT of stomach was confirmed. Patient received no postoperative radiotherapy or chemotherapy. A postoperative 3-month followup failed to show any recurrence. In addition, a literature review on DSRCT is included.