時差性 多發病變을 보인 유전성 비용종성 대장암 1예
Case of Hereditary Non-Polyposis Colon Cancer (Lynch Syndrome)
소속 상세정보
양동휘/Dong-Wheuy Yang
강구정/민중기/김병구/박용기/최창록/지정희/Koo-Jeung Kang/Jung-Ki Min/Byung-Koo Kim/Yong-Ki Park/Chang Rock Choi/Chang-Rok Choi
KMID : 0356719920080030277
Abstract
환자가 암의 가족력이 노출되는 것을 기피하여 수술 후에야 알 수 있었던 HNPCC 1예를
경험하였기에 문헌 고찰과 함께 보고하는 바이다. 현재로서는 HNPCC의 확인 방법이 가계
도 조사밖에는 없으므로, 대장암을 취급할 때는 가족력에 유의하고, 특히 직계가족에 대장암
의 병력이 있는 경우 각별한 주의가 필요하다고 여겨진다. HNPCC의 중요 특징을 요약하면
다음과 같다.
1) 체염색체 우성 유전의 경향
2) 대장암의 조기 발생: 평균 46세
3) 대장의 근위부 침범경향; 비장 만곡부 보다 근위부에 69%
4) 다발성 대장암의 경향; 수술후 1년 이내(synchronous)에 18%, 1년 이후(metachronous)
10년까지의 발생추산 40%
5) 대장이외에 특정 장기암의 병발(Lynch syndrome Ⅱ의 경우)
#초록#
We experienced a case of hereditary nonpolyposis colon cancer 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 nor 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 relatives. 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 yearn)
colon cancer.
5) The presence of specific extracolonic cancers in some families(Lynch Syndrome Ⅱ).
키워드
Hereditary Nonpolyposis Colon Cancer(HNPCC);
원문 및 링크아웃 정보
등재저널 정보