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Familial Adenomatosis of the Colon Associated with Brain Tumor (Turcot's Syndrome): Report of a Case and Review of the Literature K. Ohsato 1 , H. Hashimoto 1 , H. Itoh 1 , K. Kinoshita 2 , C. Cheng 2 , T. Yao 3 , H. Watanabe 4 1Departments of Surgery Ⅰ, Kyushu University Faculty of Medicine 2Departments of Neurosurgery, Kyushu University Faculty of Medicine 3Departments of Internal Medicine Ⅱ, Kyushu University Faculty of Medicine 4Departments of Pathology Ⅱ, Kyushu University Faculty of Medicine pp.1511-1517
Published Date 1975/11/25
DOI https://doi.org/10.11477/mf.1403112192
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 A report is made of a 21-year-old woman with familial adenomatosis of the colon associated with brain tumor, agenesis of the corpus callosum and porencephaly in addition to two punched out lesions of the skull, café-au-lait spots on the skin and two adenomatous polyps in the duodenum. She underwent a total colectomy with ileorectal anastomosis in May 1972 and was followed up since that time. In September 1974, brain scanning and carotid angiography revealed a large less-vascular mass in the left frontal lobe. Surgery was carried out and the tumor, astrocytoma grade Ⅲ, was removed subtotally. Her sister, aged 19 years, also suffered from colonic adenomatosis with multiple gastric cancers, a right ovarian cyst, café-au-lait spots and subcutaneous lipomas. Seven members of the family died of intestinal cancer.

 In addition to the present case, fifteen cases of colonic polyposis complicated by brain tumor, mainly glioma, have so far been reported in the literature. These cases are distributed from 9 to 26 years in age except for one in eighth decade, and the male to female ratio is 10: 6. Besides brain tumor, such associations as thyroid cancer, Gardner's stigmata, von Recklinghausen's disease, upper gastrointestinal adenomas etc. have been recorded in these cases.

 These associated lesions including brain tumor seem to be a part of the manifestations of specific oncogenetic disposition in familial adenomatosis coli.


Copyright © 1975, Igaku-Shoin Ltd. All rights reserved.

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電子版ISSN 1882-1219 印刷版ISSN 0536-2180 医学書院

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