Hepatocellular carcinoma arising in the absence of cirrhosis in genetic haemochromatosis: three case reports and review of literature

Genetic haemochromatosis constitutes a high risk factor for the development of hepatocellular carcinoma. It is widely accepted that venesection preven ts the evolution of cirrhosis in haemochromatosis and indirectly protects a gainst the development of hepatocellular carcinoma. Clinical, pathological and radiological data are presented on three patients who did not conform t o the 'siderosis-cirrhosis-carcinoma' sequence and in whom prompt and adequ ate iron depletion did not prevent the development of cancer. This is the f irst report of hepatocellular carcinoma intervening in non-cirrhotic liver in two siblings with genetic haemochromatosis. The current literature on th e subject is reviewed. The direct oncogenic role of iron remains to be eluc idated. Hepatocellular carcinoma should be considered as a differential dia gnosis in patients with non-cirrhotic genetic haemochromatosis who present with clinical deterioration during the course of an otherwise uneventful ve nesection programme, Eur J Gastroenterol Hepatol 11:915-919 (C) 1999 Lippin cott Williams & Wilkins.