Intrahepatic Cholangiocarcinoma in Wilson's Disease: 2 Case Reports.

ABSTRACT:

Introduction

Wilson's disease is a rare autosomal recessive inherited disorder of human copper metabolism. The spectrum of clinical manifestations of liver disease ranges from asymptomatic to cirrhosis and acute liver failure. Unlike the increased frequency of hepatobiliary tumours in other chronic liver diseases, the incidence of these tumours in Wilson's disease is an uncommon event, even in cirrhotic patients.

Case presentation

We describe two clinical cases of cirrhotic patients with Wilson disease diagnosed with cholangiocarcinoma.

Discussion

The occurrence of hepatobiliary malignancies in Wilson's disease is rare, even in cirrhotic patients. The exact reasons for this low prevalence are unclear, with some conflicting data. Copper's role in chromatin stabilizatio

SUBMITTER: Aguiar JR 

PROVIDER: S-EPMC12503872 | biostudies-literature | 2025 Sep

REPOSITORIES: biostudies-literature