胃過形成性ポリープの大量吐血で発症したPeutz-Jeghers症候群の1例

Peutz-Jeghers Syndrome with Hematemesis as the Patient's Chief Complaint, Report of a Case

要旨　患者は口唇，口腔粘膜，手指に色素沈着を伴う18歳の女性で，吐血および血圧低下のため，当院第2外科に緊急入院となった．出血は胃体部大彎の巨大ポリープからであり，待期的内視鏡的ポリペクトミーが施行された．消化管検索の結果，S状結腸ポリープが指摘され，同様に内視鏡的ポリペクトミーの適応となった．病理組織学的には胃は過形成性ポリープ，S状結腸は過誤腫性ポリープであった．本邦報告144例のPeutz-Jeghers症候群では腸重積，イレウス，腹痛などでの発症が主であり，吐血での発症は検索しえなかった．また，過去10年間の当院第2外科における胃内視鏡検査5,405例中，吐血で発症した胃腫瘍は11例あり，ポリープからの出血は本例のみであった．

　We encountered a case of Peutz-Jeghers syndrome with hematemesis as the chief complaint. The case was that of an 18-year-old female with pigmentation of lips, oral mucosa and fingers. At the emergency endoscopy, a giant gastric polyp was found. As the bleeding had already stopped, we scattered ten thousand units of thrombin over the polyp. Next day, endoscopic polypectomy was performed. The histological diagnosis was hyperplastic polyp. A small duodenal polyp in the descending portion, and a sigmoid colon polyp were detected. For the sigmoid colon polyp, endoscopic polypectomy was performed. The histological diagnosis of the polyp was hamartomatous polyp with branching bands of smooth muscles. Including this case of Peutz-Jeghers syndrome as the patient's chief complaint, 145 cases have been recorded in Japan. The main complaints were intussusception, mechanical obstruction and abdominal pain, but hematemesis was reported only in this case. Gastroendoscopic examination was carried out on 5,405 patients in the last 10 years in the 2nd Department of Surgery, Chiba University School of Medicine, and eleven gastric tumors had hematemesis. Histologically, six were carcinoma, two were malignant lymphoma, one was leiomyosarcoma, one was leiomyoma and one was hyperplastic polyp. Benign gastric tumors seldom reveal hematemesis. Among patients with gastric polyp only this patient exhibited hematemesis.