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. 2014 Apr;23(2):231-46.
doi: 10.1016/j.soc.2013.10.004.

Intrahepatic cholangiocarcinoma

Kimberly M Brown  1 , Abhishek D Parmar  2 , David A Geller  3
Affiliations

Intrahepatic cholangiocarcinoma

Kimberly M Brown et al. Surg Oncol Clin N Am. 2014 Apr.

Abstract

Intrahepatic cholangiocarcinoma (ICC) is a rare tumor, with an increasing incidence worldwide and an overall poor prognosis. Symptoms are usually nonspecific, contributing to an advanced tumor stage at diagnosis. The staging system for ICC has recently been updated and is based on number of lesions, vascular invasion, and lymph node involvement. Complete surgical resection to negative margins remains the only potentially curable treatment for ICC. Gemcitabine-based adjuvant therapy can be offered based on limited data from patients with unresectable ICC. Overall 5-year survivals after resection range from 17% to 44%, with median survivals of 19 to 43 months.

Keywords: Bile duct neoplasms; Cholangiocarcinoma; Intrahepatic bile duct cancer; Intrahepatic cholangiocarcinoma; Peripheral cholangiocarcinoma.

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Figures

Fig. 1
Fig. 1
Anatomic distribution of cholangiocarcinomas. Intrahepatic lesions such as the lesion in the right lobe of the liver shown in this image are many times asymptomatic and as a result present at a later stage. dCCA, distal cholangiocarcinoma; iCCA, intrahepatic cholangiocarcinoma; pCCA, perihilar cholangiocarcinoma.
Fig. 2
Fig. 2
Age-adjusted mortality for ICC, 1973–1997. (From Patel T. Increasing incidence and mortality of primary intrahepatic cholangiocarcinoma in the United States. Hepatology 2001;33(6):1354; with permission.)
Fig. 3
Fig. 3
Histologic subtypes of ICC. Mass-forming types (A) are the most common type, typically presenting as a mass lesion. Periductal infiltrating (B) are the next most common and infiltrate the hepatic parenchyma along portal structures. Intraductal growth types (C) carry the best prognosis but are the least common. Mass-forming periductal infiltrating mixed type (D) have been suggested to be the most aggressive of these subtypes.
Fig. 4
Fig. 4
Kaplan-Meier survival curve of patients with cholangiocarcinoma. (From Nathan H, Pawlik TM. Staging of intrahepatic cholangiocarcinoma. Curr Opin Gastroenterol 2010;26(3):271; with permission.)
See this image and copyright information in PMC

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