Cancer in Cystic Fibrosis: A Narrative Review of Prevalence, Risk Factors, Screening, and Treatment Challenges: Adult Cystic Fibrosis Series

Abstract

Cystic fibrosis (CF) is a progressive monogenetic disorder that causes persistent pulmonary disease, but also affects other organ systems, including the digestive tract. Recent advances in treatment and care of patients with CF, including the use of new and highly effective CF transmembrane conductance regulator modulators, have led to a dramatic increase in survival. Young patients with CF now can expect to live to or beyond middle age, when cancer is more frequent. Patients with CF now are known to face an increased risk of digestive tract cancer, particularly cancer of the colon. The risk, which could be triggered by associated CF-related conditions or other genetic mechanisms, is even greater in patients who received a transplant. Also some evidence suggests that adenomatous polyps develop more frequently in patients with CF and at an earlier age than in patients without CF. To reduce the excess risk of intestinal cancer in patients with CF, the Cystic Fibrosis Foundation has developed colonoscopy-based guidelines. For nontransplanted patients, colonoscopy should begin at 40 years of age, with rescreening at 5-year intervals; the screening interval should be shortened to 3 years if adenomatous polyps are discovered. For transplanted patients, screening should start at 30 years of age, or within 2 years of the transplant operation. Before colonoscopy, it is essential for patients with CF to undergo a special, more intensive bowel preparation than normally used for those without CF. Whether the new drugs that have dramatically improved morbidity and mortality for patients with CF will alter the risk of cancer is unknown and needs to be assessed in future studies.

Keywords: cancer; cystic fibrosis; screening colorectal cancer.