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KMID : 0365719930090010101
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Journal of Pusan Surgical Society
1993 Volume.9 No. 1 p.101 ~ p.110
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A Case of Hereditary Non-Polyposis Colon Cancer (Lynch Syndrome)
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강구정
양동휘/김중규/박용기/최창록
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Abstract
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We experiened a case of hereditary nonpolyposis colon of the site-specific type (Lynch Syndrome I) with a metachronous lesion, in a 34-year-old man. But preoperatively, we could not have recognized the definite heredity of the disease in his
family, for
he had intended not to expose his family history of the malignant disease.
HNPCC seems to be not so well known, even though it has rather larger proportion(5~10%) in the entire colon cancer cases than the relatively well known familial polyposis colon cancer which has its proportion of less than 1% in the colon cancers.
Up to now, there is neither a biomarker not other means to recognize the HNPCC, exept the full pedigree of the family. So, the physicians treating a colon cancer should pay attention to the family history, and should be especially alert when the
patient
has members with the colon cancer in the first degree relative. Followings are the cardinal features of HNPCC.
1) Autosomal dominant inheritance pattern.
2) Early age of colon cancer onset : mean age of 46 years.
3) Proximal colon cancer involvement : 69% located proximal to the splenic flexure.
4) Increased incidence of synchronous(18%) and metachronous(40% by 10 years) colon cancer.
5) The presence of specific extracolonic cancers in some families (Lynch Syndrome II).
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KEYWORD
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