Cholangiocarcinoma arising in Von Meyenburg complex associated with hepatocellular carcinoma in genetic haemochromatosis.
A 61-year-old man had a liver resection for a bilobar mass thought to be, by imaging techniques, an hepatocellular carcinoma. He had been treated for the last 12 years by venesections for genetic haemochromatosis complicated by well-compensated cirrhosis. At surgery, prothrombin time and platelet count were normal, as was alpha-fetoprotein. On the resected specimen, the non-tumoral liver was not cirrhotic; septal fibrosis was present as well as mild iron overload and numerous Von Meyenburg complexes. The bilobar tumour was composed of two different parts: one was a cholangiocarcinoma arising from Von Meyenburg complexes, the other was a moderately differentiated hepatocellular carcinoma with a partially invaded capsule. The two tumours, in close proximity, did not communicate. This observation raises three questions: the relative risk of primary liver cancer including both hepatocellular carcinoma and cholangiocarcinoma in haemochromatosis without cirrhosis; the development of cholangiocarcinoma from Von Meyenburg complexes; the reversibility of cirrhosis in treated patients.
Blanc
J F
JF
Fédération d'Hépato-Gastro-Entérologie, Hôpital Saint André, Université Bordeaux II, France.
Bernard
P H
PH
Carles
J
J
Le Bail
B
B
Balabaud
C
C
Bioulac-Sage
P
P
D006528:Carcinoma, Hepatocellular / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery; D018281:Cholangiocarcinoma / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery; D003937:Diagnosis, Differential; D006432:Hemochromatosis / Q000150:complications* / Q000235:genetics; D006801:Humans; D008113:Liver Neoplasms / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery; D008297:Male; D008875:Middle Aged; D016609:Neoplasms, Second Primary / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery