胃にびまん性ポリポーシスがみられたPeutz-Jeghers症候群の1例

Peutz-Jeghers Syndrome with Diffuse Gastric Polyposis, Well-differentiated Adenocarcinoma of the Descending Colon and Meckel's Diverticulum, Report of a Case

要旨　患者は50歳，女性．上腹部痛，下血を主訴として当科入院．本例は下口唇，手指，足底部に色素沈着を認め，十二指腸，小腸および大腸にはそれぞれ山田Ⅱ～Ⅳ型のポリープが多発しており，Peutz-Jeghers（P-J）症候群と診断された．本例の特異な点は胃の穹窿部から幽門前庭部までびまん性に山田Ⅰ～Ⅱ型の小ポリープが密集していたことである．このような報告は本邦で3例目で極めてまれである．本例は胆囊内に2個の結石と回腸にはMeckel憩室を合併していた．組織学的に下行結腸ポリープはP-Jポリープから癌化したと思われる粘膜癌であり，回腸のポリープはfocal cancerが疑われた．その他のポリープはすべてP-Jポリープに一致した組織所見であった．

　A 50-year-old woman was admitted to our institution because of melena and upper abdominal pain. An upper gastrointestinal series showed numerous sessile polyps throughout the stomach. At that time, differential diagnosis included gastric lesions of familial adenomatosis coli (FAC), non-familial fundic gland polyposis, and juvenile polyposis coli. The patient had dark brown macules on her lip, fingers and toes. A small bowel x-ray series showed two pedunculated polyps in the jejunum and a large pedunculated polyp in the ileum. A barium enema also showed three pedunculated polyps and a sessile polyp in the sigmoid colon and a pedunculated polyp in the descending colon and in the hepatic flexure, respectively. The surface of all these pedunculated polyps has the characteristic of somewhat coarse lobulation. Hypotonic duodenography also revealed multiple polyps in the second portion of the duodenum. Thus, the patient was diagnosed as having Peutz-Jeghers syndrome. Pedunculated polyps of the duodenum and colon were endoscopically polypectomized prior to an operation. Those of the small intestine and concomitant cholelithiasis, on the other hand, were surgically treated. A large pedunculated polyp of the jejunum was surgically removed and other polyps were endoscopically polypectomized by intraoperative endoscopy. Histologically, all the polyps except for that of the descending colon showed lobular proliferation of intestinal mucosa with tree-like extensions of smooth muscle bundles, consistent with Peutz-Jeghers polyposis. The polyp of the descending colon had histologically well-differentiated adenocarcinoma confined to the mucosa and that of the ileum, pattern suggestive of focal cancer in situ. The biopsy specimens of gastric polyps showed hyperplastic foveolar epithelium and extension of thin muscular bundles to the mucosal surface, also consis-tent with Peutz-Jeghers polyposis. The patient also had Meckel's diverticulum in the ileum as well.