Patients with cystic fibrosis (CF) are at a higher risk of developing colorectal cancer compared with the general population, according to a study published in the Journal of Cystic Fibrosis. 

Studies have indicated that patients with cystic fibrosis are at a higher risk of developing cancer in the digestive tract, notably colorectal cancer. In addition, studies have shown that individuals with cystic fibrosis are predisposed to having more aggressive forms of colorectal cancer stemming from mutations in the cystic fibrosis transmembrane conductance regulator gene. 

Advances in cystic fibrosis therapeutics mean that individuals with this disease tend to live longer; while this is positive news, it also means that patients are more likely to reach an age at which colorectal cancer becomes an issue. Studies have indicated that colorectal cancer incidence increases with age. 

The authors set out to quantify any excess risk of developing colorectal cancer among the population of patients with cystic fibrosis in England. Analyses were performed using three main data sources: the COloRECTal cancer dataset repository (CORRECT-T), the Linked Cystic Fibrosis Trust UK Cystic Fibrosis registry data and the Secondary Use Statistics, and the version 10 release of the Genomics England 100,000 Genomes Project. 

Read more about cystic fibrosis etiology 

These data sources contain information from all individuals diagnosed with colorectal cancer between January and December 2017. Patients were recorded to have cystic fibrosis if they were diagnosed with the disease at any point during their inpatient stay at a hospital. To minimize the occurrence of false positives, patients with fewer than 2 records of cystic fibrosis were classified as not having the disease. 

The research team identified 28 patients with both cystic fibrosis and colorectal cancer; the mean cystic fibrosis population was 8894 individuals, translating to an incidence rate of 0.29 per 1000 person-years. 

“When compared to the prevalence of cystic fibrosis transmembrane conductance regulator mutations in other population studies, the overall rate of mutations in the colorectal cancer population was significantly higher than expected,” the authors wrote. 

The study revealed that patients with cystic fibrosis are at an increased risk of developing colorectal cancer compared with the general population. In view of this, targeted screening for colorectal cancer in this patient population has the potential to save lives. 

Reference

Birch RJ, Peckham D, Wood HM, et al. The risk of colorectal cancer in individuals with mutations of the cystic fibrosis transmembrane conductance regulator (CFTR) gene: an English population-based studyJ Cyst Fibros. 2022;S1569-1993(22)01383-2. doi:10.1016/j.jcf.2022.10.001