147 Klatskin Tumors: A Comprehensive Review of Rare and Aggressive Biliary Malignancies

Abstract Introduction/Objective Klatskin tumors (perihilar cholangiocarcinoma) are rare and aggressive malignancies originating in the bile ducts at the hepatic hilum. Symptoms such as jaundice, abdominal pain, and weight loss are nonspecific, often delaying diagnosis. Surgical resection, including extended hemihepatectomy, extrahepatic biliary tract resection, and lymphadenectomy, is the only curative option. With an annual incidence of 1–2 cases per 100,000 individuals, the median survival with complete resection is approximately 40 months. Methods/Case Report A retrospective analysis was conducted using data from January 2015 to December 2024, identifying six cases. Five cases had liver resection, and one case required a Whipple procedure due to tumor extent. Results The study included six patients (two females, four males), aged 44 to 81. All presented with abdominal pain; one patient had HIV, and another had X linked agammaglobulinemia(XLA). Tumor sizes ranged from 2.3 cm to 11.5 cm. Histologically, tumors were mostly moderately to poorly differentiated, with one well-differentiated case. Out of six patients, 1 died, 2 are still under follow-up, and 3 lost to follow-up. Conclusion In conclusion, perihilar cholangiocarcinoma is a rare malignancy, but recent data suggests a rising incidence. Our case series included three cases from 2024, reflecting this trend. One case involved a patient with XLA, a condition more commonly associated with cancers such as stomach, colon, and melanoma. This case may have an association with XLA, although the literature has not reported such an association so far. Its potential links XLA emphasize the need for further research.