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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 types1-3:

  • Perihilar CCA (pCCA) (also termed "Klatskin’s tumor")
  • Distal CCA (dCCA)3

iCCA originates in the bile ducts within the liver.1

CCA can be classified into 3 anatomic subtypes

Important molecular differences are emerging among anatomic subtypes.5

Although rare, the incidence of iCCA has been increasing

The increasing incidence of iCCA may be due to factors including6-9:

  • 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
Image of a chart with text that says "Reported annual incidence of iCCA in the US in the last decade has more than DOUBLED"

Adapted from: Wu L, et al. World J Surg. 2019;43(7):1777-1787.

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

In spite of improved technology, numerous diagnostic challenges persist in iCCA, including:

  • Its asymptomatic nature at early stages4
  • Nonspecific clinical presentation12
  • The absence of specific biochemical and serum tumor markers7,12
  • Difficulty distinguishing from metastatic disease of other cancers—such as breast, lung, and pancreas—that have spread to the liver from other primary sites7

As a result, 60% to 70% of patients were first diagnosed with metastatic or unresectable disease, with a median survival of ~12 to 15 months.13 For the 30% to 40% eligible for resection—the only potentially curative treatment—the majority (~60%-65%) experienced disease recurrence.14-16

Patients with advanced iCCA and other biliary cancers have a poor prognosis. While gemcitabine/cisplatin is an accepted standard for first-line treatment, therapeutic options are limited in the second line (2L): and with no approved therapies, patients may only receive best supportive care.13,17

Image of chart - Short PFS and OS with second-line (2L) treatment in iCCA and other biliary tract cancers

REFERENCES: 1. Ghouri YA, Mian I, Blechacz B. J Carcinog. 2015;14:1. 2. Ghidini M, Pizzo C, Botticelli A, et al. Cancer Manag Res. 2019;11:379-388. 3. Razumilava N, Gores GJ. Lancet. 2014;383(9935):2168-2179. 4. Blechacz B, Komuta M, Roskams T, Gores GJ. Nat Rev Gastroenterol Hepatol. 2011;8(9):512-522. 5. Lowery MA, Ptashkin R, Jordan E, et al. Clin Cancer Res. 2018;24(17):4154-4161. 6. Khan SA, Tavolari S, Brandi G. Liver Int. 2019;39(suppl 1):19-31. 7. Saha SK, Zhu AX, Fuchs CS, Brooks GA. Oncologist. 2016;21(5):594-599. 8. Mukkamalla SKR, Naseri HM, Kim BM, Katz SC, Armenio VA. J Natl Compr Canc Netw. 2018;16(4):370-376. 9. Petrick JL, Yang B, Altekruse S, et al. PLoS ONE. 2017;12(10):e0186643. 10. Wu L, Tsilimigras DI, Paredes AZ, et al. World J Surg. 2019;43(7):1777-1787. 11. Patel N, Benipal B. Cureus. 2019;11(1):1-10. 12. Banales JM, Cardinale V, Carpino G, et al. Nat Rev Gastroenterol Hepatol. 2016;13(5):261-280. 13. Bridgewater J, Galle PR, Khan SA, et al. J Hepatol. 2014;60(6):1268-1289. 14. Rizvi S, Gores GJ. Gastroenterology. 2013;145(6):1215-1229. 15. Choi SB, Kim KS, Choi JY, et al. Ann Surg Oncol. 2009;16(11):3048-3056. 16. Endo I, Gonen M, Yopp AC, et al. Ann Surg. 2008;248(1):84-96. 17. Valle JW, Lamarca A, Goyal L, Barriuso J, Zhu AX. Cancer Discov. 2017;7(9):943-962. 18. Lamarca A, Hubner RA, David Ryder W, Valle JW. Ann Oncol. 2014;25(12):2328-2338. 19. Lamarca A, Palmer DH, Wasan HS, et al. Presented at: American Society of Clinical Oncology (ASCO) Annual Meeting; May 31-June 4, 2019; Chicago, IL: Abstract 4003.