胃ポリープに腺扁平上皮癌の発生をみたCronkhite-Canada症候群の1剖検例

Cronkhite-Canada Syndrome Associated with Adenosquamous Carcinoma in Gastric Polyps, Report of an Autopsy Case

　Cronkhite-Canada症候群は主として中年以後に発症し，外胚葉性変化（皮膚色素沈着，爪萎縮，および脱毛）を伴う非遺伝性の消化管ポリポーシスで，極めてまれな疾患である．最近われわれはCronkhite-Canada症候群の患者の胃ポリープに腺扁平上皮癌と腺管腺腫の発生をみた1例を経験し，剖検の機会を得たので若干の文献的考察を加えて報告する．

　症　例

　患　者：70歳，男性．

　主　訴：食欲不振，体重減少，脱毛，爪萎縮．

　家族歴，既往歴：特記すべきことなし．（特にポリポーシスを有するものはいない．）

　現病歴：1979年11月ごろより，心窩部の不快感を覚えるようになり，1980年4月には食欲不振，体重減少，悪心，嘔吐が出現し，このころから頭髪，眉毛，腋毛の脱落と爪の萎縮がみられるようになり大分県立病院を受診，1980年5月12日，入院となる．

　A 70 year-old man was admitted to the hospital with chief complaints of anorexia, weight and hair loss, and atrophy of finger and toenails. Pigmentation of skin and oral mucosa was not observed. Laboratory studies revealed hypoproteinemia of 5g/dl and a hard and irregular mass was palpable in the epigastrium. X-ray and endoscopic examination showed innumerable polyps of varying size and a large cauliflower-like lesion in the antrum. Biopsy specimens obtained from the cauliflower-like lesion revealed adenosquamous carcinoma, and thus the patient had a partial gastrectomy on June 23, 1980. Histological examinations of the resected stomach showed numerous polyps consisting of cylindrical epithelium, cystically dilated glands containing mucus, and edematous stroma with infiltration of inflammatory mononuclear cells. Some polyps showed hyperplasia of surface and foveolar epithelium, which resembled adenomatous polyps. Hyperplasia of surface and foveolar epithelium showing cellular atypia of moderate degree in the area surrounding adenosquamous carcinoma were marked. These features suggest that same polyps in Cronkhite-Canada syndrome have a possibility to show malignant transformation. After the operation, his hair and nails grew again, however, the patient died of massive metastasis of adenosquamous carcinoma to the liver on December 11, 1980. At autopsy, polyps were observed only in the remnant stomach.