Intrahepatic Cholangiocarcinoma

doi:10.1016/j.soc.2013.10.004

Surgical Oncology Clinics of North America

Volume 23, Issue 2, April 2014, Pages 231-246

https://doi.org/10.1016/j.soc.2013.10.004 Get rights and content

Section snippets

Key points

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Intrahepatic cholangiocarcinomas (ICCs) are aggressive, locally invasive tumors with limited 5-year survival. Multifocality, vascular invasion, lymphatic spread, and tumor histology are all determinants of staging and prognosis.
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Both the incidence and the mortality of ICCs have risen over the past several decades.
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Surgical resection is the only viable treatment option for patients who present with ICCs. Minimally invasive hepatectomy is increasingly becoming a valid option in select cases.

Epidemiology

Intrahepatic cholangiocarcinoma (ICC) is a subtype of a family of aggressive cholangiocarcinomas, tumors that arise from cholangiocytes of the biliary tree. There are several key epidemiologic considerations of ICC:

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ICCs are rare, accounting for 20% to 25% of all cholangiocarcinomas; perihilar (50%–60%) or distal common bile duct (20%–25%) tumors are more common (Fig. 1).¹ They are still the second most common primary liver malignancy, following hepatocellular carcinoma.²
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The incidence rate of

Clinical Presentation

The clinical presentation of ICCs is usually nonspecific, and symptoms can include generalized abdominal pain, or less commonly, weight loss and jaundice.

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In a retrospective review of a 31-year experience at Johns Hopkins University, patients with ICC most commonly presented with abdominal pain and were less likely to experience jaundice or weight loss than patients with extrahepatic cholangiocarcinoma.²¹ This finding was confirmed in other studies.22, 23
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Because these tumors are discrete from

7th Edition AJCC Staging

Previous iterations of the American Joint Committee on Cancer (AJCC) staging for ICCs had been based on data from patients with hepatocellular carcinoma. Findings from population-based studies⁵ and basic science literature39, 40 have demonstrated that ICCs are pathologic entities with a more aggressive tumor biology and distinct phenotype than hepatocellular carcinoma. Recognizing this, Nathan and colleagues¹⁰ used Surveillance, Epidemiology, and End Results-Medicare data from 1988 to 2004 to

Management and treatment

Although there have been some developments in adjuvant therapies, surgical resection remains the only potentially curative treatment modality for patients with ICCs.21, 45, 46 This section deals with the considerations of operative resection, including the extent of resection, minimally invasive surgical techniques, and orthotopic liver transplantation (OLT). Finally, the role of other nonsurgical therapies is discussed.

Summary

ICCs are aggressive malignancies that have been increasing in incidence and mortality over time. Few patients present with resectable disease at the time of presentation, and diagnosis is often difficult because of the occult nature and anatomic position of ICCs. Prognostic features, such as multifocality, vascular invasion, lymphatic spread, and histopathology, should be considered in the management and treatment of these patients. However, because of the relative rarity of ICCs, little is

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