Hepatocellular carcinoma in porphyria cutanea tarda: frequency and factors related to its occurrence

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Abstract
Thirty‐eight patients with porphyria cutanea tarda (PCT) have been seen in the last 18 years. Five of these patients (13%) developed hepatocellular carcinoma (HCC) during follow‐up. We analyzed the differences in clinical, laboratory and liver histology findings at presentation, between patients who developed HCC during follow‐up (HCC‐group, n = 5) and those who did not (PCT‐group, n = 33). Of the clinical features the duration of skin‐symptoms was longer in the HCC‐group (mean: 10.4±1.1 years) than in the PCT‐group (mean: 1.4±1 years) (p > 0.001). No differences in routine laboratory findings were found. Although 11/38 (29%) patients had serologic evidence of a past hepatitis B virus infection and 7/38 (18%) patients had antibodies against hepatitis C virus, no differences in these parameters were found between the PCT‐group and the HCC‐group. In all 34 liver biopsies a variable degree of siderosis was found (PCT‐group vs. HCC‐group: NS). Only piecemeal necrosis (p>0.01) and advanced fibrosis or cirrhosis (p>0.001) were more common in liver biopsies in the HCC‐group. In conclusion, factors related to an increased risk of HCC in PCT are: a) a long symptomatic period before start of treatment and b) the presence of chronic active hepatitis and/or advanced fibrosis or cirrhosis in liver biopsies.