巨大デスモイドを合併した家族性大腸腺腫症の1例

Familial Adenomatosis Coli Syndrome with a Huge Intraabdominal Desmoid Tumor, Report of a Case

要旨　症例は35歳男性，25歳時大腸ポリポーシスにてS状結腸部分切除術を受けた．1983年1月に腸閉塞症状にて来院．腹部に成人頭大の腫瘤を触知，急性腹症にて手術．腹腔内に巨大な腸間膜腫瘤を認めたが切除不能と判断，生検施行し閉腹．その後巨大な腸間膜腫瘍を伴った家族性大腸腺腫症と診断し再手術を施行．腫瘍の大部分と腫瘍により圧迫壊死を起こしていた十二指腸第2部より第3部を合併切除し救命しえた．切除標本の大きさは23×22×6cm，重さ3kgで組織所見はデスモイドであった．デスモイドを合併した家族性大腸腺腫症は本邦では14例が報告され本例が15例目でありデスモイドは報告例中最大であった．

　A 35 year-old man visited our hospital in January 1983 with nausea and vomiting. Partial sigmoidectomy was performed at another hospital 10 years ago because of colonic polyposis. On physical examination a tumor as large as human head was palpable on the abdomen. Emergency operation was made with a diagnosis of ileus and intestinal necrosis. At surgery a huge mesenteric tumor was found and only biopsy was done. Histological diagnosis was desmoid tumor. Barium enema study revealed multiple adenomas of the colon. The diagnosis was familial adenomatosis coli with a desmoid tumor. The second operation consisted with resection of necrotizing duodenum compressed by the tumor and resection of the tumor. The patient did well postoperatively. Resected tumor was 23×22×6 cm, weighed 3 kg. There were 14 cases of familial adenomatosis with desmoid tumor in Japan except this one. The tumor size of this patient was the biggest in these cases.