Lynch Patrick M
Department of GI Medicine, Hepatology and Nutrition, University of Texas MD Anderson Cancer Center, Houston, TX 77030, USA.
Digestion. 2007;76(1):68-76. doi: 10.1159/000108395. Epub 2007 Oct 19.
Familial colorectal cancer (CRC) is heterogeneous. Screening recommendations do exist for individuals with a mild to moderate family history of CRC, with or without early age at onset. However, most attention has properly focused on the identification of individuals in whom a defined susceptibility gene mutation can be found. The past 20 years has seen the discovery genes for familial adenomatous polyposis (FAP), its variant forms (attenuated FAP and the recessive MYH-associated polyposis or MAP), nonadenomatous polyposes, and hereditary nonpolyposis CRC (HNPCC). A few retrospective and prospective studies support current recommendations for endoscopic surveillance in FAP and HNPCC, the main subjects of this paper. Where firm data have been lacking, various professional organizations have been very willing to provide consensus clinical practice guidelines. General guidelines need to be tailored to the peculiarities of a patient's circumstances, a task best accomplished in the hands of practitioners familiar with the natural history of the condition. Improvements in the technology of endoscopic treatment of adenomas now enable increasingly aggressive nonsurgical intervention. To this may be added opportunities for chemoprevention, currently consisting mainly of nonsteroidal anti-inflammatory drugs. Surveillance, chemoprevention, and imaginative hybrid surveillance/endoscopic therapy/chemoprevention trials may gradually decrease the present dependence on prophylactic colectomy/proctocolectomy.
家族性结直肠癌(CRC)具有异质性。对于有轻度至中度CRC家族史、无论发病年龄是否早的个体,确实存在筛查建议。然而,大多数注意力都恰当地集中在识别那些能够找到明确的易感基因突变的个体上。在过去20年里,已经发现了家族性腺瘤性息肉病(FAP)、其变异形式(attenuated FAP和隐性MYH相关息肉病或MAP)、非腺瘤性息肉病以及遗传性非息肉病性CRC(HNPCC)的相关基因。一些回顾性和前瞻性研究支持了目前对FAP和HNPCC进行内镜监测的建议,本文主要讨论这两个主题。在缺乏确凿数据的情况下,各种专业组织非常愿意提供共识性临床实践指南。一般指南需要根据患者情况的特殊性进行调整,这项任务最好由熟悉该病自然史的从业者来完成。腺瘤内镜治疗技术的改进现在使得越来越积极的非手术干预成为可能。此外,现在有了化学预防的机会,目前主要包括非甾体类抗炎药。监测、化学预防以及富有想象力的联合监测/内镜治疗/化学预防试验可能会逐渐减少目前对预防性结肠切除术/直肠结肠切除术的依赖。