Hereditary nonpolyposis colorectal cancer (lynch syndromes I and II). I. Clinical description of resource
Hereditary nonpolyposis colorectal cancer (lynch syndromes I and II). I. Clinical description of resource
复制标题
遗传性非息肉病性结直肠癌（林奇综合征 I 和 II）。
作者：
H. Lynch;W. Kimberling;W. Albano;J. Lynch;Karen A. Biscone;G. Schuelke;A. Sandberg;M. Lipkin;E. Deschner;Y. Mikol;R. Elston;J. Bailey;B. Danes
遗传性非息肉病性结直肠癌（HNPCC）由以下组成：（1）癌症家族综合征（CFS），或林奇综合征II，表现出早发性近端结肠癌和其他相关的结肠外腺癌，特别是子宫内膜癌;和（2）遗传性位点特异性结肠癌（HSSCC），或林奇综合征I，除结肠外癌症外，表现出所有相同的特征。九个CFS家庭和两个HSSCC家庭提供了生物标志物测试的资源（见配套文章）。所有的家庭都经过了仔细的家谱和癌症验证评估。生物标本是在实地访问期间获得的地理上最接近的家庭的地区。向患者及其医生提供癌症教育和监测/管理建议。此外，40个家庭（约3000人）与CFS或HSSCC已被确定。综合征癌症仅限于直系亲属，而不是非血缘亲属，反对参与精神因素。一个记录的临床特征是在CFS和HSSCC动力学中近端结肠癌与远端结肠癌的偏好。这具有重要的临床意义，因为它阐明了早期建立有效监测以检测主要近端结肠癌的必要性。
Hereditary nonpolyposis colorectal cancer (HNPCC) is comprised of the following: (1) the cancer family syndrome (CFS), or Lynch syndrome II, which shows early‐onset proximal colonic cancer predominance and other associated extracolonic adenocarcinomas, particularly endometrial carcinoma; and (2) hereditary site‐specific colon cancer (HSSCC), or Lynch syndrome I, which shows all of the same characteristics, except for extracolonic cancer. Nine families with CFS and two with HSSCC provided the resource that was tested for biomarkers (see companion article). All families were meticulously evaluated for genealogy and cancer verification. Biologic specimens were obtained during field visits to areas of closest geographic proximity to the families. Cancer education and recommendations for surveillance/management were provided to patients and their physicians. Additionally, 40 families (about 3000 individuals) with either CFS or HSSCC have been ascertained. Syndrome cancers were restricted to direct‐line relatives as opposed to nonbloodline relatives, arguing against involvement of environmntal factors. One documented clinical feature was a predilection for proximal versus distal colonic cancer in both CFS and HSSCC kindreds. This has important clinical significance in that it clarifies the need for instituting effective surveillance earlier to detect the predominantly proximal colonic cancers.