ancer risk among lung transplant recipients with cystic fibrosis

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Original Article

⁎ Corresponding au Bethesda, MD 20814

E-mail address: a

http://dx.doi.org/10.1 1569-1993/© 2016 E

www.elsevier.com/locate/jcf Journal of Cystic Fibrosis 16 (2017) 91–97

Cancer risk among lung transplant recipients with cystic fibrosis

Aliza K. Fink a,⁎, Elizabeth L. Yanik b, Bruce C. Marshall a, Michael Wilschanski c, Charles F. Lynch d, April A. Austin e, Glenn Copeland f, Mahboobeh Safaeian b, Eric A. Engels b

a Cystic Fibrosis Foundation, Bethesda, MD, USA b Division of Cancer Epidemiology and Genetics, National Cancer Institute, Bethesda, MD, USA

c Department of Pediatric Gastroenterology, Hadassah Hebrew University Medical Center, Jerusalem, Israel d Department of Epidemiology, The University of Iowa, Iowa City, IA, USA

e New York State Cancer Registry, New York State Department of Health, Albany, NY, USA f Michigan Cancer Surveillance Program, Michigan Department of Health and Human Services, Lansing, MI, USA

Received 26 April 2016; revised 17 July 2016; accepted 24 July 2016 Available online 15 August 2016

Abstract

Background: Previous studies demonstrated increased digestive tract cancers among individuals with cystic fibrosis (CF), particularly among lung transplant recipients. We describe cancer incidence among CF and non-CF lung recipients. Methods:We used data from the US transplant registry and 16 cancer registries. Standardized incidence ratios (SIRs) compared cancer incidence to the general population, and competing risk methods were used for the cumulative incidence of colorectal cancer. Results:We evaluated 10,179 lung recipients (1681 with CF). Risk was more strongly increased in CF recipients than non-CF recipients for overall cancer (SIR 9.9 vs. 2.7) and multiple cancers including colorectal cancer (24.2 vs. 1.7), esophageal cancer (56.3 vs. 1.3), and non-Hodgkin lymphoma (61.8 vs. 9.4). At five years post-transplant, colorectal cancer was diagnosed in 0.3% of CF recipients aged b50 at transplant and 6.4% aged ≥50. Conclusions: CF recipients have increased risk for colorectal cancer, suggesting a need for enhanced screening. © 2016 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.

Keywords: Cancer; Transplant

1. Introduction

Cystic fibrosis (CF) is one of the most common life- shortening, genetic disorders and occurs in approximately one in 3500 Caucasian births in the US [1]. Mutations in the cystic fibrosis transmembrane regulator (CFTR) gene lead to malfunctioning or absent CFTR protein, which impairs mucosal clearance mechanisms causing recurring lung infec- tions, inflammation, and airflow obstruction. Over time there have been tremendous improvements in CF treatment. In 2014,

thor at: Cystic Fibrosis Foundation, 6931 Arlington Road , USA. fink@cff.org (A.K. Fink).

016/j.jcf.2016.07.011 uropean Cystic Fibrosis Society. Published by Elsevier B

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