Benign tumours and pseudotumours within the porta hepatis masquerading as perihilar cholangiocarcinoma

Abstract

Background: Hilar cholangiocarcinoma (HC), also referred to as Altemeier-Klatskin’s tumour, is a lethal primary extrahepatic carcinoma of biliary epithelial origin, arising within 2 cm of the hilar confluence. Radical surgical excision provides the best chance for a cure; however, the management of patients with HC is challenging not only because of the need for a high level of skill inbiliary and hepatic resections, but also because of the difficulty in reaching an accurate diagnosis preoperatively. In fact, the differential diagnosis of HC is a dia gnostic dilemma which is currently persisting, as modern, sophisticated dia gnostic 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 precise preoperative characterisation of a hilar stricture extremely important by preventing unnecessary, high-risk, major surgical procedures. Therefore, alternative benign entities masquerading as Altemeier-Klatskin’s tumour deserve an important place in the differentialdia gnosis of hilar obstruction. Purpose: Consider ing the important clinical implications that a precise diagnosis of the aetiological cause of a biliary obstruction at the liver hilum would have, this paper will focus on the diff erentiation between HC and benign hilar obstructions and will review benign tumours and pseudotumours masquerad ing as HC along with their specific diagnostic features.