Pseudotumoral portal cavernoma mimicking hilar cholangiocarcinoma: A diagnostic pitfall

Portal cavernoma cholangiopathy (PCC), also referred to as portal biliopathy, encompasses a heterogeneous spectrum of biliary tract abnormalities that develop as a consequence of extrahepatic portal vein obstruction (EHPVO) with subsequent cavernous transformation of the portal vein. Pseudotumoral portal cavernoma represents a distinct subtype of PCC and is characterized by the presence of numerous fine collateral venous channels that encase and simulate the course of the common bile duct (CBD). We report 2 cases of pseudotumoral portal cavernoma secondary to portal vein thrombosis in patients with underlying myeloproliferative disorders. These cases illustrate the diagnostic challenges associated with this rare entity and underscore the central role of cross-sectional imaging in establishing an accurate diagnosis and preventing misinterpretation as a biliary neoplasm.