Cholangiocellular carcinoma

Cholangiocellular carcinoma (CCC), also called cholangiocarcinoma, is a malignancy of the cholangiocytes that line the bile ducts. It can occur in the extrahepatic or intrahepatic bile ducts, with the former being more common. It can also occur in the gallbladder. If a CCC occurs at the confluence of the right and left hepatic bile ducts, it’s known as a Klatskin tumor.

It's an aggressive malignancy with a poor prognosis.

Clinical features

Extrahepatic cholangiocarcinoma presents with signs of cholestasis, including jaundice, pale stool, dark urine, and pruritus. Courvoisier sign (palpable nontender gallbladder) is typical. Intrahepatic CCC and gallbladder cancer is more likely to be asymptomatic and present later.

Diagnosis and evaluation

Tumour markers CA 19-9 and CEA are typically elevated. Ultrasound is good for initial imaging and shows bile duct dilation, but MRCP or ERCP are required for the diagnosis.

Treatment

For cases with resectable disease, radical resection with lymphadenectomy followed by adjuvant chemotherapy is the standard.