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World J Gastrointest Oncol. Sep 15, 2021; 13(9): 1121-1131
Published online Sep 15, 2021. doi: 10.4251/wjgo.v13.i9.1121
Screening strategy for gastrointestinal and hepatopancreatobiliary cancers in cystic fibrosis
Brett Hoskins, Paul Wasuwanich, Ann O Scheimann, Wikrom Karnsakul
Brett Hoskins, Ann O Scheimann, Wikrom Karnsakul, Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Department of Pediatrics, The Johns Hopkins University School of Medicine, Baltimore, MD 21287, United States
Paul Wasuwanich, Department of Medicine, University of Florida College of Medicine, Gainesville, FL 32610, United States
Author contributions: Hoskins B, Wasuwanich P, Scheimann AO and Karnsakul W contributed equally to writing the article.
Conflict-of-interest statement: The co-authors declare no financial or personal conflicts of interests.
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Wikrom Karnsakul, MD, Associate Professor, Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Department of Pediatrics, The Johns Hopkins University School of Medicine, 600 North Wolfe Street, Baltimore, MD 21287, United States. wkarnsa1@jhmi.edu
Received: February 28, 2021
Peer-review started: February 28, 2021
First decision: July 16, 2021
Revised: July 21, 2021
Accepted: August 5, 2021
Article in press: August 5, 2021
Published online: September 15, 2021
Processing time: 193 Days and 13.5 Hours
Core Tip

Core Tip: Patients with cystic fibrosis are at a significantly elevated risk for gastrointestinal tract and hepatopancreatobiliary cancers when compared to the general population, especially in those with severe cystic fibrosis transmembrane conductance regulator (CFTR) mutations or who have undergone organ transplantation. As life expectancies continue to increase with CFTR modulators and improved care, cancer screening will become increasingly important. Screening recommendations are largely universal and still emerging. Given the financial burden of universal screening, there is a need for more precise screening based on specific risk factors. In this review, we propose changes to the guidelines for gastrointestinal screening of patients with cystic fibrosis.