摘要
The term Hereditary Non-Polyposis Colorectal Cancer （HNPCC） is a poor descriptor of the syndrome described by Lynch. Over the last decade, the term has been applied to heterogeneous groups of families meeting limited clinical criteria, for example the Amsterdam criteria. It is now apparent that not all Amsterdam criteria-positive families have the Lynch syndrome. The term HNPCC has also been applied to clinical scenarios in which CRCs with DNA microsateUite instability are diagnosed but in which there is no vertical transmission of an altered DNA mismatch repair （MMR） gene. A term that has multiple, mutually incompatible meanings is highly problematic, particularly when it may influence the management of an individual family. The Lynch syndrome is best understood as a hereditary predisposition to malignancy that is explained by a germline mutation in a DNA MMR gene. The diagnosis does not depend in an absolute sense on any particular family pedigree structure or age of onset of malignancy. Families with a strong family history of colorectal cancer that do not have Lynch syndrome have been grouped as ‘Familial Colorectal Cancer Type-X＇. The first step in characterizing these cancer families is to distinguish them from Lynch syndrome. The term HNPCC no longer serves any useful purpose and should be phased out.
条款世袭 Non-Polyposis Colorectal 癌症(HNPCC ) 是林奇描述的症候群的一个差的描述符。在最后十年，条款被用于满足有限临床的标准的家庭的异构的组，例如阿姆斯特丹标准。并非所有阿姆斯特丹标准积极的家庭有 Lynch 症候群，现在是明显的。条款 HNPCC 也被用于有 DNA 微卫星不稳定性的 CRC 在，但是在在哪个没有一个改变的 DNA 失配修理(MMR ) 的垂直传播被诊断，但是在哪个的临床的情形基因。有多重、互相不兼容的意思的一项条款是高度有问题的，特别地当它可以影响一个单个家庭的管理时。Lynch 症候群最好作为遗传素因被理解到恶意被一个细菌线变化在 DNA MMR 基因解释。诊断不在绝对意义取决于任何特别家庭家谱结构或恶意的发作的年龄。有颜色的强壮的家庭历史的家庭没有林奇症候群的表面的癌症作为“家庭 Colorectal 癌症 Type-X ”被组织了。在描绘这些癌症家庭的第一步是把他们与林奇症候群区分开来。术语 HNPCC 不再服务任何有用目的并且应该被逐步停止。
