小腸神経線維腫より大量出血を来したvon Recklinghausen病の1例

Von Recklinghausen's Disease with Severe Hemorrhage from a Jejunal Neurofibroma, Report of a Case

要旨　患者は59歳，女性で，意識消失発作を主訴に来院した．家族歴では，姉2人，母，叔母がvom Recklinghausen病であった．既往歴では，生下時より全身に特異的な大小の腫瘤を認め，52歳時，十二指腸潰瘍で手術を受けた．現病歴は，1986年3月，突然数分間の意識消失発作があり，緊急入院した．初診時現症では全身に特異的な皮膚病変を認め，高度の貧血を呈した．腹部血管撮影，腹部CT，小腸二重造影にて，Treitz靱帯より約7cm肛門側にクルミ大の腫瘤を認めた．同年5月開腹術を施行し，同部とその近傍の計3か所の腫瘤を含めて空腸部分切除を施行した．免疫組織化学を含めた病理学的検索では，神経線維腫の像を堅し，悪性所見は認めなかった．

　A 59-year-old woman was admitted to our hospital in March 1986 because of fainting due to severe gastrointestinal bleeding. Since her childhood she had had skin lesions of multiple pedunculated and sessile tumors and multiple café-au-lait spots. She had a duodenal ulcer operation at 52 years of age. Her mother, a maternal aunt and her two sisters had had similar skin lesions.

　Upon admission she appeared pale. Her pulse rate was 96 a minute and her blood pressure was 130/80 mmHg. Her hemoglobin was 3.6 g/dl but not in a state of shock. She was treated with conventional therapy such as blood transfusion, nothing by mouth, intravenous hyperalimentation. She gradually recovered. Angiographic examination, computerized tomography and upper GI barium study were undertaken. They showed a walnut-sized tumor at the jejunum.

　At the operation, a well-circumscribed mass, 3 cm in diameter was found growing submucosally and intraluminally along the antimesenteric border of the jejunum 7 cm from the ligament of Treitz. Another two small intestinal nodules were found in the proximal jejunum. Partial resection of the jejunum was performed. Pathological examination, including the immunohistochemical study with neuron-specific enolase, revealed neurofibroma of the jejunum.