Perihilar cholangiocarcinomas (Klatskin's tumors) and Klatskin mimicking lesions. An unresolved dilemma

Abstract

Introduction: Perihilar cholangiocarcinoma(Klatskin's tumor), is a rare malignancy that arises in the confluence of the hepatic ducts of the porta hepatis and it has a rather poor prognosis. There are number of lesions that mimic these tumors and lead to misdiagnosis, resulting in major complex operations with high morbidity and considerable mortality. These lesions are known as “Klatskin-mimicking lesions”. Methods: We analyzed our prospectively established bile duct tumor database. The last 20 years we treated 73 patients who were referred to our tertiary center with a primary diagnosis of perihilar cholangiocarcinoma. All of the patients underwent thorough evaluation before the decision of treatment was made. Results: Seventy three patients were managed as having a perihilar cholangiocarcinoma but in only 59 of them the final histopathological examination confirmed the preoperative diagnosis. In 14 patients the final diagnosis differed from the primary cause of referral and the lesions were regarded as “Klatskin-mimicking lesions”. Five patients had intrahepatic lithiasis, 4 patients had Mirizzi syndrome, one patient had portal lymphadenopathy secondary to rectal cancer, one patient had non functioning neuroendocrine tumor, 1 intrahepatic gastrinoma , and two patients had IgG4 cholangiopathy. Conclusion: Clinicians should always have a high suspicion of “Klatskin-mimicking lesions” when they evaluate a patient for the possibility of a perihilar cholangiocarcinoma so that they can avoid misdiagnosis and propose a proper treatment. Based in our experience we propose an algorithm for the management of these patients