von Recklinghausen's Disease Complicated by Gastric Diffuse Ganglioneuromatosis and Early Gastric Cancer: A Case Report

Hitoshi Yamaie, Toshiya Ito, Kaname Ishizu, Fumio Kitaoka, Koichi Aoki and Hiroshi Ohtani^*

Department of Surgery, Youmeikai Obase Hospital, ^*The First Department of Pathology, Nagasaki University School of Medicine

We treated an extremely rare case of von Recklinghausen's disease complicated by gastic ganglioneuromatosis and gastric cancer. The 63-year-old woman sought medical attention for general malaise and anemia. An upper gastrointestinal tract (GI) series showed a submucosal tumor 5 cm in diameter in the cardiac region and a discoid, protruding, early gastric cancer 7cm in diameter in the prepyloric region, necessitating total gastrectomy. Histopathologically, the cardiac tumor was diffuse ganglioneuromatosis and the prepyloric tumor was type IIa well-differentiated tubular adenocarcinoma. The cutaneous tumor was a neurofibroma. To determine the biologic properties of the cutaneous and gastric submucosal tumors, we conducted immunohistochemical examination and found that both were positive for S-100 protein, in focal positive for vimentin, and negative for c-kit, CD34, desmin, and α-SMA. Only the gastric tumor was NSE-positive. Both were thus affiliated neurogenic tumors that had much in common.

Key words
von Recklinghausen's disease, gastric ganglioneuromatosis, early gastric cancer

Reprint requests
Hitoshi Yamaie Department of Surgery, Youmeikai Obase Hospital
1598 Kanda-cho, Miyako-gun, Fukuoka, 800-0344 JAPAN

Accepted
March 27, 2002

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