Intended for UK healthcare professionals only

What is intrahepatic cholangiocarcinoma (iCCA)?

iCCA is a subset of cholangiocarcinoma (CCA)

CCA, the most common primary malignancy of the bile duct, is classified by anatomic origin into iCCA and extrahepatic CCA (eCCA). eCCA is further divided into 2 types:^1-3

Perihilar CCA (pCCA) (also termed “Klatskin’s tumour”) is the most common form, accounting for 50–70% of all CCA cases^1,3
Distal CCA (dCCA) accounts for 30–40% of total CCA cases^1,3

iCCA originates in the bile ducts within the liver and accounts for <10% of CCA cases^1,3

CCA is classified by anatomic origin^3,4

CCA, cholangiocarcinoma; dCCA, distal cholangiocarcinoma, eCCA, extrahepatic cholangiocarcinoma; iCCA, intrahepatic cholangiocarcinoma, pCCA, perihilar cholangiocarcinoma.

Figure adapted from Blechacz B. 2017.⁴

Incidence of iCCA

Throughout Europe, incidence rates of CCA range between 0.5/100,000 and 3.4/100,000⁶

Although rare, the incidence of iCCA is increasing worldwide^7-9

The increasing incidence of iCCA may be due to factors including:^7-9

Increasing burden of chronic liver disease
The potential role of environmental toxins
Improved diagnostic tools and imaging
Epidemiological trends such as increasing prevalence of diabetes, obesity and alcohol use

Worldwide incidence of CCA (cases per 100,000)⁶

Data refer to the period 1971–2009. Where available, the more incident form (iCCA vs eCCA) and the temporal trend of incidence
(↑increasing trend; ↔ stable trend; ↓decreasing trend) have been reported.
CCA, cholangiocarcinoma; eCCA, extrahepatic cholangiocarcinoma; iCCA, intrahepatic cholangiocarcinoma.

Figure adapted from Banales JM, et al. 2016.⁶

Worldwide incidence of CCA (cases per 100,000)⁶

Austria

2.67 ↑eCCA>↑iCCA
(1990—2009)

Denmark

1.27 ↓eCCA>↓iCCA
(1978—2002)

Finland

1.05

France

1.3 ↔eCCA>↔iCCA
(1976—2005)

Germany

Saarland 2 Hamburg 3 ↑eCCA=↑iCCA
(1970—2006)

Italy

3.36 ↑eCCA>↑iCCA
(1988—2005)

Poland

0.7

Spain

0.5

Switzerland

0.45

UK

2.17 ↑iCCA>↓eCCA
(1971—2001)

Canada

0.35

USA

1.6 ↑iCCA>↔eCCA
(2003—2009)

Costa Rica

0.3

Puerto Rico

0.35

China

Shanghai 7.55 Qidong 7.45 iCCA>eCCA

Hong Kong 2.25 Guangzhou 0.97 iCCA>eCCA

Israel

0.3

Japan

Osaka 3.5 Hiroshima 3.05 ↓eCCA>↑iCCA
(1985—2005)

Philippines

1.2 iCCA>eCCA

Singapore

1.45 iCCA>eCCA

South Korea

Gwangju 8.75 Daegu 7.25 Busan 7.1 ↑iCCA>↑eCCA
(1999—2005)

Taiwan

4.7 iCCA>eCCA

Thailand

Northeast 85 North 14.6 Central 14.4 South 5.7 ↑iCCA>↔eCCA
(1998—2002)

Vietnam

0.1 iCCA>eCCA

Australia

0.43

New Zealand

0.4

Unmet needs in iCCA

Patients with iCCA face significant clinical challenges, including a poor prognosis and limited treatment options¹⁰

In spite of improved technology, numerous diagnostic challenges persist in iCCA, including:^10-12

The asymptomatic nature at early stages
Nonspecific clinical presentation
The absence of specific biochemical and serum tumour markers
Difficulty distinguishing from metastatic disease of other cancers

As a result, 60–70% of patients are diagnosed with metastatic or unresectable disease, with a median survival of ~12 to 15 months.¹¹ For the 30–40% eligible for resection—the only treatment with curative intent—the majority (~60–65%) experience disease recurrence^11,13-15

Biopsy is necessary to confirm a specific diagnosis of iCCA¹²

As the scientific understanding of, and clinical management options for, iCCA evolve, it is critical to accurately diagnose iCCA and distinguish it from other cancers of the biliary tract; imaging alone is not sufficient¹²

REFERENCES: 1. Ghouri YA, et al. J Carcinog. 2015;14:1. 2. Ghidini M, et al. Cancer Manag Res. 2019;11:379–88. 3. Razumilava N, Gores GJ. Lancet. 2014;383:2168–79. 4. Blechacz B. Gut Liver. 2017;11:13–26. 5. Lowery MA, et al. Clin Cancer Res. 2018;24:4154–61. 6. Banales JM, et al. Nat Rev Gastroenterol Hepatol. 2016;13:261–80. 7. Khan SA, et al. Liver Int. 2019;39(suppl 1):19–31. 8. Kirstein MM, et al. Visc Med. 2016;32:395–400. 9. Valle JW, et al. Ann Oncol. 2016;27(suppl 5):v28–v37. 10. Blechacz B, et al. Nat Rev Gastroenterol Hepatol. 2011;8:512–22. 11. Bridgewater J, et al. J Hepatol. 2014;60:1268–89. 12. Banales JM, et al. Nat Rev Gastroenterol Hepatol. 2020;17:557–88. 13. Rizvi S, et al. Gastroenterology. 2013;145:1215–29. 14. Choi SB, et al. Ann Surg Oncol. 2009;16:3048–56. 15. Endo I, et al. Ann Surg. 2008;248:84–96.

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What is intrahepatic cholangiocarcinoma (iCCA)?

Incidence of iCCA

Worldwide incidence of CCA (cases per 100,000)6

Worldwide incidence of CCA (cases per 100,000)6

Europe Hide Show

Austria

Denmark

Finland

France

Germany

Italy

Poland

Spain

Switzerland

UK

North America Hide Show

Canada

USA

South America Hide Show

Costa Rica

Puerto Rico

Asia Hide Show

China

Israel

Japan

Philippines

Singapore

South Korea

Taiwan

Thailand

Vietnam

Oceania Hide Show

Australia

New Zealand

Unmet needs in iCCA

Patients with iCCA face significant clinical challenges, including a poor prognosis and limited treatment options10

Biopsy is necessary to confirm a specific diagnosis of iCCA12

Worldwide incidence of CCA (cases per 100,000)⁶

Worldwide incidence of CCA (cases per 100,000)⁶

Patients with iCCA face significant clinical challenges, including a poor prognosis and limited treatment options¹⁰

Biopsy is necessary to confirm a specific diagnosis of iCCA¹²