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![]() Turcot syndrome However multiple adenomas develop in the colon, its number tends to be less than one of Familial Adenoma Polyposis or Gardner's syndrome. Also relatively large adenomas are found, and they are thought to become malignant more frequently. Adenomas are also recognized in the stomach,and small intestine. As concomitant lesions other than lesions in the gastrointestinal tract, various central nerve system tumors can be associated with this. It is evident that the disease is hereditary. However, because the number of cases is small, it is inconclusive whether this is recessive inheritance or dominant inheritance, a theory of recessive inheritance is widely-accepted. Recently, Turcot syndrome associated with brain tumors in FAP was reported, and in that case it was a genetically-heterogenous disease and the diseases which were arisen from APC genes produced medulloblastoma. On the other hand, that Hereditary Non-Polyposis Colorectal Cancer (HNPCC) sometimes can produce lesions like Turcot syndrome, and it was reported that cancers result from hMSH-2 which is one of the genes causing HNPCC can develop glioblastoma. ![]() |
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