Primary liver cancer in genetic hemochromatosis: a clinical, pathological, and pathogenetic study of 54 cases.
BACKGROUND
Although liver cancer arises frequently in the course of genetic hemochromatosis (GH), it has not been previously studied in a large series of patients with well-defined GH.


METHODS
The bioclinical and pathological data from 1 cholangiocarcinoma and 53 hepatocellular carcinomas (HCCs) complicating GH in 32 untreated and 22 de-ironed patients are reported.


RESULTS
This study (1) adds three new well-documented cases of HCC in noncirrhotic but only fibrotic hemochromatotic liver, (2) shows the high prevalence (83%) of proliferative and often dysplastic (70%) iron-free foci in the nontumorous liver of untreated patients, and (3) emphasizes the significant increase of cirrhosis (81% vs. 28%) and of associated noniron-related risk factors, mainly chronic alcoholism (48% vs. 25%) and tobacco smoking (50% vs. 18%) in patients with HCC compared with matched hemochromatotic patients without HCC.


CONCLUSIONS
These data (1) suggest that iron-free foci may be markers of an early stage of HCC in GH and (2) supply the basis for defining a cost-effective policy for the screening of HCC in GH patients.
Deugnier
Y M
YM
Clinique des Maladies du Foie and INSERM U 49, CHRU Pontchaillou, Rennes, France.
Guyader
D
D
Crantock
L
L
Lopez
J M
JM
Turlin
B
B
Yaouanq
J
J
Jouanolle
H
H
Campion
J P
JP
Launois
B
B
Halliday
J W
JW
D005260:Female; D006432:Hemochromatosis / Q000097:blood / Q000150:complications / Q000235:genetics*; D006801:Humans; D007501:Iron / Q000097:blood; D008113:Liver Neoplasms / Q000150:complications* / Q000209:etiology / Q000473:pathology; D008297:Male; D008875:Middle Aged; D012307:Risk Factors
D007501:Iron