Evolving data on risk and current screening recommendations for colorectal cancer in cystic fibrosis: Pre- and posttransplant
Zain Raza MD
Department of Internal Medicine, Division of Gastroenterology & Hepatology, Saint Louis University School of Medicine, Saint Louis, Missouri, USA
Contribution: Writing - original draft
Search for more papers by this authorBianca N. Islam MD, PhD
Department of Medicine, Division of Gastroenterology and Liver Disease, University Hospitals Cleveland Medical Center, Cleveland, Ohio, USA
Department of Medicine, Case Western Reserve University, School of Medicine, Cleveland, Ohio, USA
Contribution: Writing - original draft
Search for more papers by this authorChristine Y. Hachem MD
Department of Internal Medicine, Division of Gastroenterology & Hepatology, Saint Louis University School of Medicine, Saint Louis, Missouri, USA
Contribution: Supervision, Writing - review & editing, Conceptualization
Search for more papers by this authorCorresponding Author
Linda C. Cummings MD, MS
Department of Medicine, Division of Gastroenterology and Liver Disease, University Hospitals Cleveland Medical Center, Cleveland, Ohio, USA
Department of Medicine, Case Western Reserve University, School of Medicine, Cleveland, Ohio, USA
Correspondence Linda C. Cummings, MD, MS, Department of Medicine, Division of Gastroenterology and Liver Disease, University Hospitals Cleveland Medical Center, Cleveland, OH, USA.
Email: [email protected]
Contribution: Writing - review & editing, Supervision, Visualization, Conceptualization
Search for more papers by this authorZain Raza MD
Department of Internal Medicine, Division of Gastroenterology & Hepatology, Saint Louis University School of Medicine, Saint Louis, Missouri, USA
Contribution: Writing - original draft
Search for more papers by this authorBianca N. Islam MD, PhD
Department of Medicine, Division of Gastroenterology and Liver Disease, University Hospitals Cleveland Medical Center, Cleveland, Ohio, USA
Department of Medicine, Case Western Reserve University, School of Medicine, Cleveland, Ohio, USA
Contribution: Writing - original draft
Search for more papers by this authorChristine Y. Hachem MD
Department of Internal Medicine, Division of Gastroenterology & Hepatology, Saint Louis University School of Medicine, Saint Louis, Missouri, USA
Contribution: Supervision, Writing - review & editing, Conceptualization
Search for more papers by this authorCorresponding Author
Linda C. Cummings MD, MS
Department of Medicine, Division of Gastroenterology and Liver Disease, University Hospitals Cleveland Medical Center, Cleveland, Ohio, USA
Department of Medicine, Case Western Reserve University, School of Medicine, Cleveland, Ohio, USA
Correspondence Linda C. Cummings, MD, MS, Department of Medicine, Division of Gastroenterology and Liver Disease, University Hospitals Cleveland Medical Center, Cleveland, OH, USA.
Email: [email protected]
Contribution: Writing - review & editing, Supervision, Visualization, Conceptualization
Search for more papers by this authorZain Raza and Bianca N. Islam shared equal authorship.
Abstract
Advances in treatment for cystic fibrosis (CF), including cystic fibrosis transmembrane conductor regulator (CFTR) modulators, have ushered in an era where patients with CF have much longer life expectancies. This shift in life expectancy demands increased attention to diseases of aging in patients with CF. A notable complication of CF is early-onset colorectal cancer (CRC), which is especially prevalent in patients with severe mutations and after transplant. CFTR acts as a tumor suppressor gene based on knockout models. Lack of CFTR expression promotes carcinogenic processes such as intestinal inflammation and deleterious gut microbiome changes. The consensus Cystic Fibrosis Foundation recommendations advocate treating this population as a high-risk group, using a colonoscopy-only screening strategy starting at age 40 in patients without transplant and at age 30 after transplant. Screening should be considered every 5 years if negative and every 3 years or sooner for patients with adenomatous polyps. Future research will determine the role of noninvasive CRC screening tools in this population, as well as the effects of CFTR modulators on the risk of developing CRC.
CONFLICT OF INTEREST STATEMENT
The authors declare no conflict of interest.
Open Research
DATA AVAILABILITY STATEMENT
Data sharing is not applicable to this article as no new data were created or analyzed in this study.
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