Hereditary Colorectal Cancer: From Diagnosis to Surgical Options

被引:0
|
作者
Aoun, Rami James N. [1 ]
Kalady, Matthew F. [1 ,2 ]
机构
[1] Ohio State Univ, Dept Surg, Div Colon & Rectal Surg, Wexner Med Ctr, Columbus, OH USA
[2] James Comprehens Canc Ctr, Columbus, OH USA
关键词
colorectal cancer; hereditary colorectal cancer; Lynch syndrome; familial adenomatous polyposis; FAMILIAL ADENOMATOUS POLYPOSIS; PEUTZ-JEGHERS-SYNDROME; CLINICAL-PRACTICE GUIDELINES; SOCIETY TASK-FORCE; LYNCH-SYNDROME; JUVENILE POLYPOSIS; MICROSATELLITE INSTABILITY; COWDEN SYNDROME; DESMOID TUMORS; HIGH-RISK;
D O I
10.1055/s-0044-1787884
中图分类号
R57 [消化系及腹部疾病];
学科分类号
摘要
Hereditary colorectal cancer (CRC) syndromes account for up to 5% of CRC. Patients have an increased risk of CRC and extracolonic cancers, both of which develop at an early age. The main polyposis syndromes include familial adenomatous polyposis, MYH-associated polyposis, Peutz-Jeghers syndrome, juvenile polyposis syndrome, and PTEN hamartoma syndrome. The non-polyposis syndromes include Lynch syndrome and familial colorectal cancer type X. Each of the syndromes have distinct but sometimes overlapping phenotypes. Clinical evaluation and ultimately the underlying germline genetic pathogenic variants define the syndromes. Each syndrome has polyp, CRC, and extracolonic risks and management is based on early and timely surveillance with therapeutic and often extended prophylactic surgery. Surgical intervention strategies are individualized, considering not only the earlier onset of malignancies and heightened risks for metachronous cancers but also the patient's needs and quality of life. This article reviews the different diagnostic approaches to hereditary CRC and highlights subsequent disease-specific management and surgical decision-making strategies.
引用
收藏
页码:179 / 190
页数:12
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