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

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  • 胃びまん性神経節腫症と早期胃癌を合併したvon Recklinghausen病の1例

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Abstract

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.

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