Hamartomatous polyposis syndromes
Hamartomatous polyposis syndromes
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DOI：
10.1016/j.bpg.2009.02.007
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发表时间：
2009-04-01
影响因子：
3.2
通讯作者：
Burt, Randall W.
中科院分区：
文献类型：
--
作者：
Gammon, Amanda;Jasperson, Kory;Burt, Randall W.
错构瘤性息肉病综合征是一组不同的遗传性疾病，因为它们表现出错构瘤性而不是上皮性息肉组织学。每种综合征都表现出特征性的息肉组织学、胃肠道息肉分布、胃肠道癌症风险、肠外良性发现和通常的肠外癌症风险。识别这些综合征的风险个体并准确定义精确的诊断对于计划监测和管理以预防良性和恶性并发症是必要的。特征性综合征的特点，包括胃肠道的表现，病理，遗传学，和三个最常见的错构瘤性息肉病综合征，Peutz-Jeghers综合征，PTEN错构瘤道岔综合征和青少年息肉病的管理选项将在此审查。(C)2009爱思唯尔有限公司版权所有。
Hamartomatous polyposis syndromes are a diverse group of inherited conditions grouped together because they exhibit hamartomatous rather than epithelial polyp histology. Each syndrome exhibits characteristic polyp histology, gastrointestinal polyp distribution, gastrointestinal cancer risks, extra-intestinal benign findings and often extra-intestinal cancer risks. Identifying individuals at risk for these syndromes and accurately defining the precise diagnosis are necessary for planning surveillance and management in order to prevent the benign and malignant complications. Characteristic syndrome features including gastrointestinal findings, pathology, genetics, and management options for the three most common hamartomatous polyposis syndromes, Peutz-Jeghers syndrome, PTEN hamartoma turnout syndrome, and juvenile polyposis will be presented in this review. (C) 2009 Elsevier Ltd. All rights reserved.