Intrahepatic Cholangiocarcinoma

Marc J Lee, MD - Case Coordinator
Amy Ho Huang, MD - Radiology Discussion
Jason H Smouse, MD - Pathology Discussion
Pablo R Ros, MD, MPH - Attending Radiologist
Koenraad Mortele, MD - Attending Radiologist

March 10, 2003

Presentation

Imaging Findings

CT images demonstrate a dilated right intrahepatic biliary system and pneumobilia in the left intrahepatic ducts (figure 1). There is a 3.8 x 4.7cm irregular mass at the bifurcation of the right and left hepatic ducts that is hypovascular relative to the liver, enhances heterogeneously, and has a cystic/necrotic subcapsular component (figure 2). This mass invades the stomach (figure 3).

Differential Diagnosis

Diagnosis

Discussion

The gross sample was collected at autopsy. The probe in the image is inserted into the ampulla of Vater, which connects to a common hepatic bile duct stent, which follows into the left hepatic biliary system. This has been dissected out, revealing a necrotic cavity. One cholelith was identified within the cavity. Approximately 2 cm proximal along the biliary tree, there is a 3-cm, well-circumscribed mass. Proximal to the mass, the right hepatobiliary system is quite dilated. Histopathology reveals atypical, pleiomorphic cells and bacterial forms in the necrotic cavity. The bacterial forms were positive for enterococcus. The main findings are:

• Tumor (3x2.5x2cm) in the right hepatic lobe 2cm proximal to the porta hepatis and involving the biliary tract
• Necrotic cavity at the porta hepatis with complete destruction of the gallbladder with extensive necrotic debris and multiple bacterial forms

Radiology Discussion

Cholangiocarcinoma is the most common primary malignancy of the bile ducts and the second most prevalent liver cancer. The average age at onset is 60-65 years, and it strikes men slightly more often than women (1.5:1).

The most common presenting symptoms are unexplained weight loss and jaundice. Predisposing factors include UC, PSC, choledochal cysts, Caroli disease, thoratrast, and parasitic infestation (Clonorchis sinensis ).

The macroscopic appearance can be mass-like, infiltrative, or polypoid. Mass-like cholangiocarcinoma (intrahepatic cholangiocarcinoma) is characterized by a low attenuation mass showing peripheral enhancement and focal ductal dilatation around the tumor. These findings are also common in hepatic metastases.

Infiltrative cholangiocarcinoma is characterized by a focally thickened ductal wall and proximal biliary dilatation. Similar findings are associated with benign stricture.

Polypoid cholangiocarcinoma is characterized by a dilated bile duct filled with polypoid soft tissue and proximal biliary dilatation. These findings are also present with benign biliary tumors, such as papilloma and adenoma.

Cholangiocarcinoma is treated by resection. Complete resection is the only possibility of a cure. The overall 5-year survival rate is 1-5%; this increases to 10-50% following an attempt at resection. The goal of radiologic assessment is to:

1. Identify patients with clearly unresectable tumor
2. For patients with resectable tumor, to evaluate the extent of resection necessary for complete tumor removal

Cholangiocarcinoma cannot be resected if any of the following are found:

• Invasion or occlusion of the main hepatic artery or portal vein or both right and left branches of the hepatic artery or portal vein
• Unilateral hepatic vessel occlusion with extensive contralateral intrahepatic bile duct involvement
• Involvement of second order bile ducts in both the left and right hepatic lobes
• Regional metastases

References

Lee WJ et al. Radiologic Spectrum of Cholangiocarcinoma; Emphasis on Unusual Manifestations and Differential Diagnoses. Radiographics 2001; 21:S97-S116.

Lee JK et al. Computed Body Tomography with MRI Correlation 3rd ed. Lipppincott Williams and Wilkins, Philadelphia, 1998.

Weissleder R et al. Primer of Diagnostic Imaging, 2nd ed. Mosby, St. Louis, 1997.

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