ｖｏｎ　Ｒｅｃｋｌｉｎｇｈａｕｓｅｎ病に合併したｇａｓｔｒｏｉｎｔｅｓｔｉｎａｌ　ｓｔｒｏｍａｌ　ｔｕｍｏｒの２例

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  • Two cases of gastrointestinal stromal tumor with von Recklinghausen's disease.

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We report two cases of gastrointestinal stromal tumor with von Recklinghausen's disease.<br> Patient 1, a 49-year-old man with von Recklinghausen's desease was admitted to the hospital because of hematemesis. Emergency endoscopic examination revealed a submucosal mass in the 3rd portion of the duodenum. Abdominal CT scan demonstrated a solid mass with the size of 4cm contiguous to the Treitz ligament. Laparotomy showed a submucosal tumor originated from the 4th portion of the duodenum, and a partial resection of the duodenum and jejunum was performed.<br> Patient 2, a 68-year-old man with von Recklinghausen's desease was operated on for rectal cancer. During laparotomy, multiple small submucosal tumors were found in the jejunum and a partial resection of the jejunum was performed.<br> Immunohistochemically, the both tumors showed positive for c-kit (CD117), and were diagnosed as gastrointestinal stromal tumor (GIST) in a narrow sense. It is believed that most non-epithelial tumors associated with von Recklinghausen's disease are neurogenic, however, such tumors categorized into GIST in a narrow sense will increase in a progression of immunohistochemical examinations.

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