下行結腸癌を合併したPeutz-Jeghers症候群の1例

Peutz-Jeghers Syndrome Associated with Carcinoma of the Descending Colon, Report of a Case

要旨　Peutz-Jeghers症候群における消化管ポリポーシスの癌化や悪性腫瘍の合併など最近注目されてきている．患者は42歳，男性で，口唇，手指に典型的な色素斑がある．腸管にはポリープが散在し，下行結腸には腫瘍による狭窄が存在していた．内視鏡的ポリペクトミーおよび小腸部分切除，左半結腸切除，術中経腸的ポリペクトミーなどを施行した．ポリープは組織学的にすべて過誤腫でfocal cancerを認めなかった．また，結腸の腫瘍は高分化腺癌，中心に粘液癌を混在していたが，過誤腫または腺腫成分の共存を認めなかった．以上，本症候群と大腸癌の合併例を報告すると共に，本症候群の癌化，癌の合併などについて考察を加えた．

　A 42 year-old man, who had perioral and digital skin pigmentations, complained of low abdominal pain and constipation. Barium enema study disclosed multiple polyps in the small and large intestine, and long stricture with irregularity of the descending colon. Colonoscopic polypectomy of the rectal polyps was done, followed by surgery with some procedures of partial jejunal resection, left hemicolectomy, and transenteric polypectomy. Surgical specimen were histologically examlned in detail which were found that each polyp was hemartoma as one of Peutz-Jeghers syndromes without co-existence of carcinoma in situ, and a colonic tumor was well differentiated adenocarcinoma with subserosal invasion with mucoid formation. Review of the literature shows that there are some evidence genetically easy to develop the tumor in the colon and rectum including other organs, but still colonic carcinogenesis of Peutz-Jeghers syndrome is unknown.