Although the overall risk of malignancy in patients with cystic fibrosis (CF) is similar to that of the general population, there is a six-fold increased risk of digestive tract cancers (Neglia et al, 1995). Of the 13 digestive tract cancers reported in the above North American and European cohort, five were of liver or biliary tract origin.
There have been two reports of hepatocellular carcinoma in patients with CF. The first, a 32 year old lady with a longstanding history of Cystic fibrosis related liver disease (CFLD) with cirrhosis. At annual review a routine ultrasound revealed lesions consistent with hepatocellular carcinoma which was confirmed on histology (McKeon et al, 2004). The annual ultrasound in the previous year had not revealed malignancy, but by the time of diagnosis the tumour was too large to consider treatment with liver transplantation.
The second case report was an 18 year old female who presented with advanced hepatocellular carcinoma and no prior clinical evidence of CFLD (Kelleher et al, 2005).
With the increased life expectancy of patients with CF and the chronic nature of CFLD, liver cancer may become an increasingly recognised complication among the adult CF population. Increased surveillance is required in patients with CFLD. Gene polymorphisms may help to identify patients at higher risk of hepatic complications (Henrion-Caude et al, 2002).
• Overall risk of malignancy in patients with CF is similar to that of the general population
• There appears to be a six-fold increased risk of digestive tract cancers
• Increased surveillance is required in patients with CFLD
Henrion-Caude A, Flamant C, Roussen M, et al. Liver disease in CF patients is associated with glutathione S-transferase P1 polymorphism. Hepatology 2002; 36: 913-917. [PubMed]
Kelleher T, Staunton M, O’Mahony S, et al. Advanced hepatocellular carcinoma associated with cystic fibrosis. Eur J Gastroenterol Hepatol 2005; 17: 1123-1124. [PubMed]
McKeon D, Day A, Parmar J, et al. Hepatocellular carcinoma in association with cirrhosis in a patient with cystic fibrosis. J Cyst Fibros 2004; 3: 193-195. [PubMed]
Neglia JP, Fitzsimmons SC, Maisonneuve P, et al. The risk of cancer among patients with cystic fibrosis. N Engl J Med 1995; 332: 494-499. [PubMed]