Abstract :
Hilar cholangiocarcinoma (HC) is a rare disease with a poor prognosis which typically presentsin the 6th decade of life. Of the 3,000 cases seen annually in the United States, less than one half of thesetumors are resectable. A variety of risk factors have been associated with HC, most notably primarysclerosing cholangitis (PSC), biliary stone disease and parasitic liver disease. Patients typically presentwith abdominal pain, pruritis, weight loss, and jaundice. Computed topography (CT), magnetic resonanceimaging (MRI), and ultrasound (US) are used to characterize biliary lesions. Endoscopic retrogradecholangiopancreatography (ERCP) and percutaneous transhepatic cholangiography (PTC) assess localductal extent of the tumor while allowing for therapeutic biliary drainage. MRCP has demonstratedsimilar efficacies to PTC and ERCP in identifying anatomic extension of tumors with less complications.Treatment consists of surgery, radiation, chemotherapy and photodynamic therapy. Biliary drainage ofthe future liver remnant should be performed to decrease bilirubin levels thereby facilitating future liverhypertrophy. Standard therapy consists of surgical margin-negative (R0) resection with extrahepatic bile ductresection, hepatectomy and en bloc lymphadenectomy. Local resection should not be undertaken. Lymphnode invasion, tumor grade and negative margins are important prognostic indicators. In instances wherecurative resection is not possible, liver transplantation has demonstrated acceptable outcomes in highly selected patients. Despite the limited data, chemotherapy is indicated for patients with unresectable tumorsand adequate functional status. Five-year survival after surgical resection of HC ranges from 10% to 40%however, recurrence can be as high as 50-70% even after R0 resection. Due to the complexity of this disease,a multi-disciplinary approach with multimodal treatment is recommended for this complex disease.
