Screening strategy for gastrointestinal and hepatopancreatobiliary cancers in cystic fibrosis

作     者：Brett Hoskins Paul Wasuwanich Ann O Scheimann Wikrom Karnsakul 

作者机构：Division of Pediatric GastroenterologyHepatologyand NutritionDepartment of PediatricsThe Johns Hopkins University School of MedicineBaltimoreMD 21287United States Department of MedicineUniversity of Florida College of MedicineGainesvilleFL 32610United States 

出 版 物：《World Journal of Gastrointestinal Oncology》 (世界胃肠肿瘤学杂志（英文版）（电子版）)

年 卷 期：2021年第13卷第9期

页      面：1121-1131页

核心收录：

学科分类：1002[医学-临床医学] 100214[医学-肿瘤学] 10[医学] 

主　　题：Colorectal cancer CFTR gene Cystic fibrosis Gastrointestinal cancer Hepatopancreatobiliary cancer Screening 

摘      要：Based on systematic review and meta-analysis,the risk for developing cancers in patients with cystic fibrosis(CF)is known to be significantly greater than in the general population,including site-specific cancers of the esophagus,small bowel,colon,liver,biliary tract,and *** even higher risk has been found in patients who have severe CF transmembrane conductance regulator(CFTR)genotypes or who have undergone organ transplantation and are *** risk continues to rise as life expectancies steadily climb due to advancements in medical care and treatment for *** colorectal cancer risk is at such a high level that CF has now been declared a hereditary colon cancer syndrome by the Cystic Fibrosis *** CFTR gene has been stronglyassociated with the development of gastrointestinal(GI)cancers and mortality in the CF *** CF carriers have shown an increased rate of GI cancers compared to the general *** limitations exist with the reported guidelines for screening of GI and hepatopancreatobiliary cancers in the CF population,which are largely universal and are still *** is a need for more precise screening based on specific risk factors,including CFTR mutation,medical co-morbidities(such as gastroesophageal reflux disease,distal intestinal obstruction syndrome,and diabetes mellitus),familial risks for each cancer,gender,age,and other *** this review,we propose changes to the guidelines for GI screening of patients with *** the development of CFTR modulators,additional studies are necessary to elucidate if there is an effect on cancer risk.