Cholangiocarcinoma natural history, complications and prognosis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Farima Kahe M.D. [2], Suveenkrishna Pothuru, M.B,B.S. [3]

Overview

The symptoms of cholangiocarcinoma usually develop in the fourth decade of life, and start with symptoms such as abdominal pain, jaundice and fever. Common complications of cholangiocarcinoma infection, liver failure, tumor metastasis.

Natural History, Complications, and Prognosis

Natural history

The symptoms of cholangiocarcinoma usually develop in the fourth decade of life, and start with symptoms such as abdominal pain, jaundice and fever.

Complications

Common complications of cholangiocarcinoma include:[1]

Prognosis

  • Depending on the extent of the cholangiocarcinoma at the time of diagnosis, the prognosis may vary. However, the prognosis is generally regarded as poor with 5-year survival of only 10-44%.[2]
  • The prognosis may be worse for patients with primary sclerosing cholangitis who develop cholangiocarcinoma.
  • The most important factor in prognosis of cholangiocarcinoma is whether or not the tumor is able to be resected.

Extent of the tumor

  • Patients with multiple tumors, larger tumors and tumors that have spread to nearby blood vessels or lymph nodes have a poor outcome.

Resectability

Tumors that can be completely removed by surgery (resectable) have a better prognosis than tumors that cannot be removed by surgery (unresectable).

  • Distal cholangiocarcinoma: Long-term survival rates range from 15%-25%.[3]
  • Intrahepatic cholangiocarcinoma: Survival estimates after surgery ranging from 22%-66%.[4]
  • Perihilar cholangiocarcinoma: 5 years survival rates range from 20-50%.[5]

Surgical margins

  • The best prognostic factors are resection of tumor-free surgical margin without lymph node invasion.[6]
  • Tumor diameter, histology, and differentiation are poor predictors of good outcome with 5-year survival rates varying from 20 to 60%.[7]
  • For extrahepatic cholangiocarcinoma, 5 year survival rate is approximately 30% after resection of tumor-free surgical margins. Majority of patients have recurrence due to following reasons:
    • Disseminated tumors
    • Formation of new tumors in previously oncogenic liver tissue

References

  1. Patel T (2011). "Cholangiocarcinoma--controversies and challenges". Nat Rev Gastroenterol Hepatol. 8 (4): 189–200. doi:10.1038/nrgastro.2011.20. PMC 3888819. PMID 21460876.
  2. DeOliveira ML, Cunningham SC, Cameron JL, Kamangar F, Winter JM, Lillemoe KD, Choti MA, Yeo CJ, Schulick RD (2007). "Cholangiocarcinoma: thirty-one-year experience with 564 patients at a single institution". Ann. Surg. 245 (5): 755–62. doi:10.1097/01.sla.0000251366.62632.d3. PMC 1877058. PMID 17457168.
  3. Kim BH, Kim K, Chie EK, Kwon J, Jang JY, Kim SW, Oh DY, Bang YJ (2017). "Long-Term Outcome of Distal Cholangiocarcinoma after Pancreaticoduodenectomy Followed by Adjuvant Chemoradiotherapy: A 15-Year Experience in a Single Institution". Cancer Res Treat. 49 (2): 473–483. doi:10.4143/crt.2016.166. PMC 5398409. PMID 27554480.
  4. Guglielmi A, Ruzzenente A, Campagnaro T, Pachera S, Valdegamberi A, Nicoli P, Cappellani A, Malfermoni G, Iacono C (2009). "Intrahepatic cholangiocarcinoma: prognostic factors after surgical resection". World J Surg. 33 (6): 1247–54. doi:10.1007/s00268-009-9970-0. PMID 19294467.
  5. Sano T, Shimada K, Sakamoto Y, Ojima H, Esaki M, Kosuge T (2008). "Prognosis of perihilar cholangiocarcinoma: hilar bile duct cancer versus intrahepatic cholangiocarcinoma involving the hepatic hilus". Ann. Surg. Oncol. 15 (2): 590–9. doi:10.1245/s10434-007-9687-y. PMID 18057991.
  6. Farges O, Fuks D, Boleslawski E, Le Treut YP, Castaing D, Laurent A, Ducerf C, Rivoire M, Bachellier P, Chiche L, Nuzzo G, Regimbeau JM (2011). "Influence of surgical margins on outcome in patients with intrahepatic cholangiocarcinoma: a multicenter study by the AFC-IHCC-2009 study group". Ann. Surg. 254 (5): 824–29, discussion 830. doi:10.1097/SLA.0b013e318236c21d. PMID 22042474.
  7. Macias, Rocio I. R. (2014). "Cholangiocarcinoma: Biology, Clinical Management, and Pharmacological Perspectives". ISRN Hepatology. 2014: 1–13. doi:10.1155/2014/828074. ISSN 2314-4041.

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