Multiple black hepatocellular adenomas in a male patient

A 65-year-old man presented with multiple liver tumours. Imaging techniques could not differentiate between adenomas and hepatocellular carcinomas. He had no relevant past medical history. Liver function tests were normal exc ept for a 1.5-fold rise in GGT, AFP was normal. Viral markers were negative . During laparoscopy, numerous black tumours of different sizes were seen, These tumours were adenomas without malignant transformation. Tumoral hepat ocytes contained a brown pigment in the canalicular area without evidence o f cholestasis. This pigment was Fontana positive and looked like Dubin-John son pigment by electron microscopy, The expression of the canalicular multi specific organic anion transporter (cMOAT) was decreased in the tumours but normal in the non-tumoral liver ruling out the diagnosis of Dubin-Johnson syndrome. There was mild iron deposition possibly related to an homozygous H63D mutation in the HFE gene. Three years after their discovery, the size of the tumours remained stable. It is concluded that this male patient with multiple adenomas and mild iron overload is at risk of developing an hepat ocellular carcinoma and that the black colour of adenomas is probably due t o a partial defect in excretion of organic anions. (C) 2000 Lippincott Will iams & Wilkins.