胃巨大絨毛様腫瘤に癌性変化をみたCronkhite-Canada症候群の1例

Cronkhite-Canada Syndrome with a Giant Villous Tumor of the Stomach-Developing Cancerous Change, Report of a Case

要旨　患者は59歳，女性．浮腫，爪萎縮，脱毛があり，精査のため入院．著明な低蛋白血症（T.P3.7g/dl），貧血（Hb3.1g/dl）を認めた．¹²⁵I-PVP test 4.6％と蛋白漏出を示し，胃透視，内視鏡所見より，胃全体に大小不同の隆起性病変が密集し，十二指腸管腔への絨毛状腺腫を思わせる手拳大の腫瘤が嵌頓．生検により癌が疑われ，開腹術を施行し腫瘤（10×8cm，160g）を摘出．組織所見は，腫瘤の大部分は，Cronkhite-Canada症候群の特徴的病理所見である間質の増大，浮腫，腺管の嚢胞状拡張を持ち，一部に高分化型腺癌がみられ癌への移行を思わせた．これまで本症候群と癌の合併は12例報告され，癌化に関しては賛否両論があるが，自験例を経験し本症候群の癌化は十分ありうると考えた．

　A 50 year-old woman was admitted to our hospital because of generalized edema and exertional dyspnea due to severe anemia (Hb 3.1g/dl). Physical examination revealed the atrophy of nails and loss of hair, but pigmentation was not seen. Hypoproteinemia was remarkable (Total Protein 3.7g/dl) and ¹²⁵I-PVP test (4.6%) suggested protein-losing gastroenteropathy. The upper gastrointestinal series showed multiple small polypoid lesions in the stomach and a fist-sized tumor with soap-bubble appearance was recognized in the second part of the duodenum, but polyposis of the large intestine could not be seen. The gastroduodenofiberscopy revealed multiple polypoid lesions resembling salmon roe in the stomach and a huge villous tumor in the duodenum.

　As the specimen of the biopsy suggested malignant change, wedge resection including the villous tumor was performed. In the histological findings the excised tumor (10×8cm, 160g) showed typical findings of Cronkhite-Canada (C-C) syndrome, where wide and edematous interstitium and cystically dilated glands were seen. A small part of the villous tumor revealed a cancerous change, which was diagnosed as welldifferentiated adenocarcinoma. As the typical part of C-C syndrome transferred continuously to the cancer, we inferred that it was the tubulovillous adenoma of C-C syndrome that transferred to the cancer, Only 12 cases have been reported about carcinoma which was associated with C-C syndrome. Relationship between C-C syndrome and its cancerous change has not been established yet, but we believe that there is likelihood of cancerous change in C-C syndrome.