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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).

FIGURE 10-1

Classification of cholangiocarcinoma according to its localization in the biliary tree.

FIGURE 10-2

Classification of intrahepatic cholangiocarcinoma based on the gross appearance of the tumor: (A) mass-forming type, (B) mass-forming and periductal infiltrating type, (c) periductal infiltrating type, and (d) intraductal growth type.

FIGURE 10-3

Bismuth-Corlette classification for perihilar cholangiocarcinoma: (a) type I, tumor involves only the common hepatic duct, distal to the confluence of the left and right hepatic ducts (biliary confluence); (b) type II, tumor involves the biliary confluence; (c) type IIIa, tumor affects the right hepatic duct in addition to the biliary confluence; (d) type IIIb, tumor involves the left hepatic duct in addition to the biliary confluence; (E) type IV, tumors either involve both the right and left hepatic ducts in addition to the biliary confluence or (f) are multifocal.

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 ...

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