Benign tumors and pseudotumors within the porta hepatis masquerading perihilar cholangiocarcinoma

Konstantinos Vasiliadis, Orestis Ioannidis, Konstantinos Tsalis


Hilar cholangiocarcinoma (HC), also referred to as Altemeier-Klatskin’s tumor, is a lethal primary extrahepatic carcinoma of biliary epithelial origin, arising within 2 cm of the hilar confluence. Its radical surgical excision provides the best chance for cure however, the management of patients with HC is challenging not only because of the need for a high level of skill in biliary and hepatic resections, but also because of the difficulty to reach preoperatively an accurate diagnosis. In fact, the differential diagnosis of HC is a diagnostic dilemma which is currently holding on, as modern, sophisticated diagnostic modalities are not always able to provide a definitive preoperative diagnosis. This difficulty is compounded by the fact that alternative entities that mimic HC may be present in up to 25% of patients with hilar obstruction. This makes preoperative precise characterization of a hilar stricture extremely important by preventing unnecessary, high risk, major surgical procedures. Therefore, alternative benign entities masquerading Altemeier-Klatskin tumor deserve an important place in the differential diagnosis of hilar obstruction. Considering the important clinical implications that a precise diagnosis of the etiological cause of a biliary obstruction at the liver hilum would have, this paper will focus on the differentiation between HC and benign hilar obstructions and will review benign tumors and pseudotoumors masquerading HC along with their specific diagnostic features. 

Klíčová slova

Altemeier-Klatskin’s tumor, Hilar biliary stricture, Misdiagnosis, Benign Altemeier-Klatskin-like lesions, Klatskin-mimicking lesions

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