Hilar cholangiocarcinoma: diagnosis, treatment options, and management

Abstract

Hilar cholangiocarcinoma (HC) is a rare disease with a poor prognosis which typically presents in the 6(th) decade of life. Of the 3,000 cases seen annually in the United States, less than one half of these tumors are resectable. A variety of risk factors have been associated with HC, most notably primary sclerosing cholangitis (PSC), biliary stone disease and parasitic liver disease. Patients typically present with abdominal pain, pruritis, weight loss, and jaundice. Computed topography (CT), magnetic resonance imaging (MRI), and ultrasound (US) are used to characterize biliary lesions. Endoscopic retrograde cholangiopancreatography (ERCP) and percutaneous transhepatic cholangiography (PTC) assess local ductal extent of the tumor while allowing for therapeutic biliary drainage. MRCP has demonstrated similar 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 of the future liver remnant should be performed to decrease bilirubin levels thereby facilitating future liver hypertrophy. Standard therapy consists of surgical margin-negative (R0) resection with extrahepatic bile duct resection, hepatectomy and en bloc lymphadenectomy. Local resection should not be undertaken. Lymph node invasion, tumor grade and negative margins are important prognostic indicators. In instances where curative 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 tumors and 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.

Keywords: Cholangiocarcinoma (CC); biliary neoplasm; hilar.

Figure 1

PRISMA diagram demonstrating inclusion and exclusion methodology for articles in this review.

Figure 2

Right upper quadrant ultrasound on a 79-year-old male with right upper quadrant pain. Note the presence of a large hilar hypoechoic mass.

Figure 3

Axial (A) and coronal (B) CT of the liver in the portal venous phase showing a large heterogeneous mass in both lobes. Axial T2 (C) and contrast enhanced portal venous phase (D) MR shows ductal dilatation resulting from the large central mass.

Figure 4

T2 (A) and contrast enhanced hepatic arterial phase (B) and portal venous phase (C) MR images of the liver demonstrate ill-defined slightly hyperintense mass on T2 with peripheral rim enhancement on the hepatic arterial phase, and central necrosis on the portal venous phase. (D) FDG PET shows increased uptake and central photopenic zone indicating necrosis.

Figure 5

Axial contrast enhanced MRI in the hepatic arterial phase (A) and portal venous phase (B) showing a small intraductal hypervascular mass with washout on the portal venous phase. Coronal MRCP image (C) showing intraductal mass at the confluence of the right and left hepatic ducts causing moderate intrahepatic ductal dilatation.

Figure 6

The Bismuth-Corlette classification of hilar cholangiocarcinoma. Type I tumors are distal to the hepatic duct confluence (HDC) while type II neoplasms extend to and involve the HDC. Type III tumors involve the HDC and either the proximal right hepatic duct (type IIIA) or proximal left hepatic duct (type IIIB). Type IV tumors extend into the bilateral proximal hepatic ducts up to the segmental bile ducts (74). Abbreviations: RHD, right hepatic duct, LHD, left hepatic duct, HDC hepatic duct confluence.