How are cholangiocarcinoma classified?

Updated: Apr 13, 2018
  • Author: Mahesh Kumar Neelala Anand, MBBS, DNB, FRCR; Chief Editor: John Karani, MBBS, FRCR  more...
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The tumors are classified as extrahepatic tumors (87-92%) or intrahepatic tumors (8-13%). [7]

Extrahepatic tumors are divided into proximal, middle, and distal ductal tumors. Tumors located at the confluence of the right and left hepatic ducts with the proximal common hepatic duct are called Klatskin tumors.

Intrahepatic tumors arise from the small ducts and are often diffuse and multicentric; satellite nodules occur in about 65% of patients.

Solitary well-demarcated tumors are difficult to differentiate from primary hepatocellular carcinomas (HCCs). [8] The diffuse sclerosing or scirrhous types are densely fibrotic and have annular long strictures. Compared with other tumors, they are less cellular and have relatively few well-differentiated carcinoma cells in a dense connective tissue stroma. They are generally confined to the proximal ducts.

The nodular variety is also called the papillary type. The tumors are nodular on the intraluminal and extraluminal surfaces, and they form irregular strictures. They are most common in the distal duct and in the periampullary region.

The papillary tumors are friable and vascular and tend to bleed easily, causing hemobilia.

Intrahepatic tumors have a special predilection for perineural spread. Hematogenous spread to the liver, peritoneum, or lung is extremely rare.

Lymphatic spread is common and occurs in the cystic and common bile duct (CBD) nodes in about 32% of extrahepatic tumors and 15% of intrahepatic tumors.

Extrahepatic tumors also spread to the celiac nodes in about 16% of cases and to the peripancreatic and superior mesenteric nodes. Infiltration of adjacent liver occurs in 23% of cases, and peritoneal seeding occurs in 9%.

See the image below.

Bismuth classification for perihilar cholangiocarc Bismuth classification for perihilar cholangiocarcinoma. Shaded areas represent tumor location.

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