MALIGNANCY
2004 McKeon D. Day
A. Parmar J. Alexander G. Bilton D. Hepatocellular carcinoma in association
with cirrhosis in a patient with cystic fibrosis. J Cyst Fibros 2004; 3:193-195. [PubMed]
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. A 32-year-old lady with cystic
fibrosis (CF) had ultrasound lesions consistent with hepatocellular carcinoma,
confirmed on histology. She was also 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. 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.
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