Background: Cystic fibrosis liver disease (CFLD) occurs in 37% of patients with CF. To date and to the best of our knowledge, there has not been a documented case of hepatocellular carcinoma in association with cirrhosis and CF.
Case report: A 32-year-old lady with cystic fibrosis (CF) presented for her annual review. She had been diagnosed with CFLD since early adolescence. A routine ultrasound of her liver revealed lesions consistent with hepatocellular carcinoma. This was confirmed on histology. She had no risk factors for hepatitis, and thorough investigation revealed no other cause for her chronic liver disease. She was also found to be pregnant at the time of diagnosis. Her tumour was considered too large for resection and liver transplantation and she was referred to a national centre for laser ablative therapy.
Conclusion: It is our concern that with the increased life expectancy of patients with CF and the chronic nature of CFLD that this may be an increasingly recognised complication amongst the CF adult population. Therefore, we have changed our practice to more intense surveillance of patients with established CFLD to incorporate biannual ultrasound imaging of the hepatic system and yearly serum concentration measurements of alpha-fetoprotein.