Hepatocellular carcinoma risk in patients with porphyria cutanea tarda

It has been suggested that patients with porphyria cutanea tarda (PCT) are at high risk of developing hepatocellular carcinoma (HCC); however, this has not been confirmed by other workers. The aim of our study was to evaluate the incidence of HCC in patients with PCT, and to assess the possible co-factors associated with cancer development.

Thirty-nine consecutive patients with a diagnosis of PCT were included. Hepatitis B virus and hepatitis C virus (HCV) infection was investigated, and a percutaneous liver biopsy was performed. Patients were treated with phlebotomies, which resulted in a clinical remission in all. These patients were included in a surveillance programme for the detection of HCC, with ultrasonography and serum alpha-fetoprotein every 6 months.

Thirty-nine patients (92% male; mean age, 55 +/- 16 years) with PCT were included. Alcohol abuse was reported in 87% of the cases. The mean follow-up time since the initial diagnosis of PCT was 9.7 years (378 patient-years of follow-up). Serological markers of past infection with hepatitis B virus were found in 20% of the patients, while HCV infection was diagnosed in 56%. The stage of fibrosis in patients having liver biopsy was: 0 (32%), 1 (32%), 2 (9%), 3 (18%), and 4 (9%). HCC was diagnosed in 1/39 patients with PCT (cumulative incidence, 2.6%), giving a yearly incidence of 0.26% per patient-year. This patient was a 69-year-old male, alcohol abuser, with HCV infection, with a 12-year period between diagnosis of PCT and HCC, and with liver biopsy (3 years before) showing fibrosis stage 3.

The risk of developing HCC in patients with PCT in our area is relatively low (a yearly incidence of less than 1% per patient-year of follow-up), and perhaps attributable, at least in part, to concomitant HCV infection. Patients presenting with PCT should undergo both HCV infection determination and liver biopsy, and those with concomitant HCV infection or advanced fibrosis/cirrhosis should probably be included in a standard surveillance programme in order to achieve early diagnosis of HCC.