[Cholangiocarcinomas developing on multiple bile duct hamartomas syndrome].
Two cases of cholangiocarcinoma arising in a background of multiple bile duct hamartomas are reported. In each case, the clinical and radiological investigations showed a liver tumor with no other concomitant disease. Tumors were resected, and microscopic examination revealed in one case a hilar cholangiocarcinoma, in the other case a peripheric cholangiocarcinoma, both being well-differentiated. It revealed also numerous bile duct hamartomas scattered throughout the liver. In one case, some bile duct hamartomas showed a gradual morphologic transition from benign to dysplastic and neoplastic epithelium. Patients with multiple bile duct hamartomas may have an increased risk of developing cholangiocarcinomas.
Droy
Laure
L
Service d'anatomie pathologique, de chirurgie digestive et de gastroentérologie et hépatologie, hôpital Nord-Laënnec, CHU de Nantes, boulevard Jacques-Monod, 44800 Saint-Herblain, France. lauredroy@hotmail.com
Sagan
Christine
C
Paineau
Jacques
J
Gournay
Jérôme
J
Mosnier
Jean-François
JF
D001650:Bile Duct Neoplasms / Q000473:pathology* / Q000601:surgery; D001653:Bile Ducts, Intrahepatic / Q000473:pathology / Q000601:surgery; D002455:Cell Division; D018281:Cholangiocarcinoma / Q000209:etiology / Q000473:pathology* / Q000601:surgery*; D018450:Disease Progression; D005260:Female; D006222:Hamartoma / Q000473:pathology*; D006498:Hepatectomy; D006801:Humans; D008297:Male; D008875:Middle Aged; D009367:Neoplasm Staging; D016896:Treatment Outcome