Hoskins B, Wasuwanich P, Scheimann AO, Karnsakul W. Screening strategy for gastrointestinal and hepatopancreatobiliary cancers in cystic fibrosis. World J Gastrointest Oncol 2021; 13(9): 1121-1131 [PMID: 34616517 DOI: 10.4251/wjgo.v13.i9.1121]
Corresponding Author of This Article
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
Research Domain of This Article
Gastroenterology & Hepatology
Article-Type of This Article
Minireviews
Open-Access Policy of This Article
This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (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/
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
Abstract
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 pancreas. An 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 immunosuppressed. The risk continues to rise as life expectancies steadily climb due to advancements in medical care and treatment for CF. The colorectal cancer risk is at such a high level that CF has now been declared a hereditary colon cancer syndrome by the Cystic Fibrosis Foundation. The CFTR gene has been strongly-associated with the development of gastrointestinal (GI) cancers and mortality in the CF population. Even CF carriers have shown an increased rate of GI cancers compared to the general population. Several limitations exist with the reported guidelines for screening of GI and hepatopancreatobiliary cancers in the CF population, which are largely universal and are still emerging. There 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 factors. In this review, we propose changes to the guidelines for GI screening of patients with CF. With the development of CFTR modulators, additional studies are necessary to elucidate if there is an effect on cancer risk.
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.
