The risk of gastrointestinal malignancies in cystic fibrosis

The life expectancy for cystic fibrosis (CF) patients has increased dramatically over the last 30 years. Although the overall cancer risk for CF patients does not appear to be increased there is a marked increased risk of gastrointestinal malignancies especially in the post lung transplant population. CF patients that do develop gastrointestinal malignancies do so at an earlier age and there is often a lag in the diagnosis and management of these individuals. We present a 39 year old male CF patient that underwent a colonoscopy for colon cancer screening and a large, near obstructing, villous adenoma of his ileum was found. The polyp was removed successfully via endoscopy without incident and there was no evidence of malignancy. An upper endoscopy revealed a long segment of Barrett's esophagus with no evidence of dysplasia. We present this case as well as a detailed review of the literature on cancer risk in CF and a discussion of the mechanisms that may be involved. We also present the risk of GI malignancies in non-CF patients as a guide on how to assess and manage the risk of GI malignancies in this ever-changing patient population.