Cystic Fibrosis and Cancer: Unraveling the Complex Role of CFTR Gene in Cancer Susceptibility

Simple Summary Cystic fibrosis (CF) is a genetic condition that affects the lungs, digestion, and other body systems. People with CF have a higher chance of developing certain types of cancer. The reason for this is related to a gene called CFTR, which is altered in CF patients. This gene normally helps regulate the movement of substances in and out of cells. When it does not work properly, it can lead to changes in cells that make them more likely to become cancerous. The cancers most commonly associated with CF are colorectal, pancreatic, and respiratory cancers. By understanding how CFTR and cancer are connected, doctors can develop better ways to screen for and treat these cancers in people with CF. More research is needed to fully understand this link and improve care for CF patients.Abstract Cystic fibrosis (CF) is a genetic disorder affecting multiple organs, primarily the lungs and digestive system. Over the years, advancements in medical care and treatments have significantly increased the life expectancy of individuals with CF. However, with this improved longevity, concerns about the potential risk of developing certain types of cancers have arisen. This narrative review aims to explore the relationship between CF, increased life expectancy, and the associated risk for cancers. We discuss the potential mechanisms underlying this risk, including chronic inflammation, immune system dysregulation, and genetic factors. Additionally, we review studies that have examined the incidence and types of cancers seen in CF patients, with a focus on gastrointestinal, breast, and respiratory malignancies. We also explore the impact of CFTR modulator therapies on cancer risk. In the gastrointestinal tract, CF patients have an elevated risk of developing colorectal cancer, pancreatic cancer, and possibly esophageal cancer. The underlying mechanisms contributing to these increased risks are not fully understood, but chronic inflammation, altered gut microbiota, and genetic factors are believed to play a role. Regular surveillance and colonoscopies are recommended for early detection and management of colorectal cancer in CF patients. Understanding the factors contributing to cancer development in CF patients is crucial for implementing appropriate surveillance strategies and improving long-term outcomes. Further research is needed to elucidate the molecular mechanisms involved and develop targeted interventions to mitigate cancer risk in individuals with CF.