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家族性大腸腺腫症は高率に癌化する無数の大腸腺腫を有する遺伝性疾患として知られ,従来諸家の関心はもっぱら大腸病変に向けられてきた.しかし1953年骨腫と軟部腫瘍(Gardner症候群)1),1959年脳腫瘍(Turcot症候群)2),1962年歯牙病変3),1968年甲状腺癌4)などの合併が報告されたほか,近年わが国において胃5)~7),十二指腸8),空・回腸9)10)病変なども高率に合併することが指摘され,本症を大腸の限局性疾患として扱ってきた従来の考え方は改められつつある.
先にわれわれは合併胃病変の病理学的検討を報告した11)が,今回はそのX線・内視鏡像の特徴や診断上の問題点さらに胃病変の経過などについて検討を加えた.
The present study describes 24 patients with familial adenomatosis of the colon belonging to 16 families. The stomachs of all the patients were examined carefully by means of X-ray and endoscopy followed by endoscopic biopsy.
1) As a result, various gastric lesions were detected in 17 patients (71%). Fundic gland polyposis accounted for eight cases, adenoma for nine and carcinoma for two.
2) In addition, polypoid lesions consisting of erosive gastritis were found in the antrum of six patients. The endoscopic pictures of the lesions were very similar to those of adenoma. Therefore, adequate endoscopic biopsies were most important in the differential diagnosis between erosive gastritis and adenoma.
3) The fundic gland polyps were multiple in occurrence and small (less than 5 mm in diameter) with sessile and hemispheric appearance. They were completely limited to the fundic gland mucosa and histologically were made up of simple hyperplasia of the fundic glands. They were considered to represent the gastric lesions specific to this disorder.
4) The adenomas, distributed in the antral or pyloric mucosa, were demonstrated endoscopically as discolored sessile polyps with central depression and reddened spots. There was no essential difference in histology between gastric adenomas in patients with this disease and those in patients without it. However, familial adenomas had a tendency to multiplicity.
5) Gastric carcinoma found in two patients was multiple in occurrence. One of them had two adenocarcinomas with focal adenomatous area and multiple adenomas in the antrum.
6) Follow-up study was performed on fundic gland polyposis in six patients and adenoma in five. Fundic gland polyposis in two patients clearly decreased in number and size on X-ray examination in the course of two or three years after total colectomy. However, there were no visible changes in the gastric polyps of the remaining nine patients.
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