Chapter 10: Native and Recurrent Cholangiocarcinoma

INCIDENCE AND EPIDEMIOLOGY

• Cholangiocarcinoma comprises about 3% of gastrointestinal malignancies.

• It is the most common biliary malignancy.

• It is the second most common primary hepatic malignancy.

• It accounts for about 10% to 20% of primary malignant tumors.

• It is more frequent in Asian countries, with increasing incidence in Western countries during the past few decades.

• Mean age at diagnosis is 50 years.

• Five-year survival for resected cases is between 15% and 40%, according to the stage of the disease.

CLASSIFICATION

• Cholangiocarcinoma is classified according to its localization as intrahepatic (5% to 10%), perihilar (60% to 70%), and distal (20% to 30%) (Fig. 10-1).

• The second-order bile ducts serve as the level of separation between intrahepatic and perihilar cholangiocarcinoma.

• The cystic duct serves as the anatomic boundary between perihilar and distal cholangiocarcinoma.

• Based on the gross appearance of the tumor, intrahepatic cholangiocarcinoma is further classified as mass-forming type, mass-forming and periductal infiltrating type, periductal infiltrating type, or intraductal growth type (Liver Cancer Study Group of Japan, Fig. 10-2).

• Perihilar tumors are stratified according to the Bismuth-Corlette classification, on the basis of biliary involvement, to 4 types, I to IV (Fig. 10-3).

DIAGNOSIS

• Requires a multimodality approach, involving clinical, laboratory, radiologic, endoscopic, and pathologic analysis.

• Nonspecific symptoms, such as abdominal pain, cachexia, malaise, fatigue, and night sweats.

• Diagnosis of exclusion: must rule out metastatic adenocarcinoma in patients with intrahepatic cholangiocarcinoma.

• Itching may precede weeks before the establishment of obstructive jaundice in the case of perihilar cholangiocarcinoma.

• Diagnosis is easier in patients with primary sclerosing cholangitis.

• Gamma-glutamyl transferase is increased in most patients.

• Serum levels of tumor biomarker carbohydrate antigen 19-9 (Ca 19-9) can help the diagnosis. Caution in cases with cholestasis (false positive).

• Ca 19-9 levels of ≥100 U/mL may be indicative of intrahepatic cholangiocarcinoma and Ca 19-9 ...