Background: A correlation between acute intermittent porphyria or porphyria cutanea tarda and hepatocellular carcinoma (HCC) has been noted in several studies, but only one case of association between HCC and porphyria variegata has been reported. We therefore report another case of association between HCC and porphyria variegata.
Methods: A 54-year-old nurse with familial porphyria variegata who developed an HCC was studied. The diagnosis of porphyria variegata was made in the course of a familial survey by means of measuring lymphocyte protoporphyrinogen oxidase activity, at a time when the patient had no symptoms. Eighteen years later the patient presented with a firm enlargement of the liver.
Results: Histologic examination showed a well-differentiated HCC. The diagnosis was confirmed by positive immunostaining for alpha-foetoprotein antibodies.
Discussion: Sixteen months after surgical resection of the HCC the patient was still alive.