Introduction: Extrahepatic Cholangiocarcinoma is a rare tumor arising from the biliary epithelium. About two thirds are located at the hepatic duct bifurcation (Klatskin tumor). The overall incidence is about 1 in 100 000 with 3000 new cases annually. Male to female ratio is 1.3:1 and the average age of the patients is between 50-70 years old. Most patients with unresectable disease die within one year of the diagnosis. The average five-year survival rate of resectable ones is between 10-30 %, while in unresectable ones it is less than five.
Materials and methods: A case of a 48-year-old patient with cholangiocarcinoma of the extrahepatic bile ducts is presented. He was first admitted to the Infectious Disease Department at St. Marina University
Hospital Varna with jaundice, general weakness and vomiting. The patient underwent different diagnostic modalities such as ultrasonography, computed tomography and magnetic resonance imaging. A neoplastic process was suspected. An exploratory surgery was therefore performed which deemed the patient unresectable and after that an open biopsy confirmed a T4NxMx cholangiocarcinoma of the extrahepatic bile ducts.
Results: Currently surgical excision is the only potentially curative treatment for cholangiocarcinoma.
Patients with unresectable disease are often offered treatment with GEMCIS - a combination of gemcitabine and cisplatin; GEMOX - a combination of gemcitabine and oxaliplatin; monotherapy with gemcitabine or therapy with leucovorin and fluoropyrimidines. The Oncological Committee decided to subject the patient to first line of chemotherapy consisting of four cycles of GEMOX. Following that, computed tomography showed signs of stable disease and it was decided to continue the treatment with three more cycles. After the last course of chemotherapy, positron emission tomography showed that the tumor had disappeared.
Conclusion: Extrahepatic cholangiocarcinoma is a devastating disease that rarely responds to chemotherapy.
That being said, the presented case is a miraculous exception of a complete remission.