Hilar cholangiocarcinoma: diagnosis and staging

Abstract

Cancer arising from the proximal biliary tree, or hilar cholangiocarcinoma, remains a difficult clinical problem. Significant experience with these uncommon tumors has been limited to a small number of centers, which has greatly hindered progress. Complete resection of hilar cholangiocarcinoma is the most effective and only potentially curative therapy, and it now clear that concomitant hepatic resection is required in most cases. Simply stated, long-term survival is generally possible only with an en bloc resection of the liver with the extrahepatic biliary apparatus, leaving behind a well perfused liver remnant with adequate biliary-enteric drainage. Preoperative imaging studies should aim to assess this possibility and must evaluate a number of tumor-related factors that influence resectability. Advances in imaging technology have improved patient selection, but a large proportion of patients are found to have unresectable disease only at the time of exploration. Staging laparoscopy and (13)fluoro-deoxyglucose positron emission tomography (FDG-PET) may help to identify some patients with advanced disease; however, local tumor extent, an equally critical determinant of resectability, may be underestimated on preoperative studies. This paper reviews issues pertaining to diagnosis and preoperative evaluation of patients with hilar biliary obstruction. Knowledge of the imaging features of hilar tumors, particularly as they pertain to resectability, is of obvious importance for clinicians managing these patients.

Figure 1.

(a) PTC image showing hilar biliary ductal obstruction with a large filling defect. Note that the duct is expanded rather than contracted, characteristic of a papillary tumor. (b) Axial CT image showing a well-circumscribed tumor at the biliary confluence (arrows), without apparent invasion of the underlying portal vein. (c) Low power, hematoxylin/eosin-stained section of the resected tumor showing little invasion of the bile duct wall. Reprinted from Annals of Surgery 2005; 241: 703–14, Jarmagin WR et al. Copyright 2005, with permission from Elsevier.

Figure 2.

(a) Axial MRCP image of a patient with hilar cholangiocarcinoma and left lobe atrophy. The bile ducts appear white in this image (black arrow). The left liver is shrunken (white arrow), with crowding and dilatation of the bile ducts. (b) Ultrasound image showing the tumor at the biliary confluence (arrow) with severe narrowing of the left portal vein.

Figure 3.

Axial MRCP image of a patient with hilar cholangiocarcinoma. Note that the bile ducts appear white in this image. The tumor in the bile duct is shown, with evidence of portal vein involvement and atrophy of the anterior sector of the right liver (delineated by the two unlabelled lines). An intrahepatic metastasis is also shown.

Figure 4.

ERCP image of a patient with Mirizzi's syndrome. A gallstone is impacted in the neck of the gallbladder (arrow), causing biliary obstruction.