Cholangiocarcinoma

Cholangiocarcinoma
Other namesBile duct cancer, cancer of the bile duct[1]
Micrograph of an intrahepatic cholangiocarcinoma (right of image) adjacent to normal liver cells (left of image). H&E stain.
Pronunciation
SpecialtyOncology
SymptomsAbdominal pain, yellowish skin, weight loss, generalized itching, fever[1]
Usual onset70 years old[3]
TypesIntrahepatic, perihilar, distal[3]
Risk factorsPrimary sclerosing cholangitis, ulcerative colitis, infection with certain liver flukes, some congenital liver malformations[1]
Diagnostic methodConfirmed by examination of the tumor under a microscope[4]
TreatmentSurgical resection, chemotherapy, radiation therapy, stenting procedures, liver transplantation[1]
PrognosisGenerally poor[5]
Frequency1–2 people per 100,000 per year (Western world)[6]

Cholangiocarcinoma, also known as bile duct cancer, is a type of cancer that forms in the bile ducts.[2] Symptoms of cholangiocarcinoma may include abdominal pain, yellowish skin, weight loss, generalized itching, and fever.[1] Light colored stool or dark urine may also occur.[4] Other biliary tract cancers include gallbladder cancer and cancer of the ampulla of Vater.[7]

Risk factors for cholangiocarcinoma include primary sclerosing cholangitis (an inflammatory disease of the bile ducts), ulcerative colitis, cirrhosis, hepatitis C, hepatitis B, infection with certain liver flukes, and some congenital liver malformations.[1][3][8] However, most people have no identifiable risk factors.[3] The diagnosis is suspected based on a combination of blood tests, medical imaging, endoscopy, and sometimes surgical exploration.[4] The disease is confirmed by examination of cells from the tumor under a microscope.[4] It is typically an adenocarcinoma (a cancer that forms glands or secretes mucin).[3]

Cholangiocarcinoma is typically incurable at diagnosis which is why early detection is ideal.[9][1] In these cases palliative treatments may include surgical resection, chemotherapy, radiation therapy, and stenting procedures.[1] In about a third of cases involving the common bile duct and less commonly with other locations the tumor can be completely removed by surgery offering a chance of a cure.[1] Even when surgical removal is successful chemotherapy and radiation therapy are generally recommended.[1] In certain cases surgery may include a liver transplantation.[3] Even when surgery is successful the 5-year survival is typically less than 50%.[6]

Cholangiocarcinoma is rare in the Western world, with estimates of it occurring in 0.5–2 people per 100,000 per year.[1][6] Rates are higher in Southeast Asia where liver flukes are common.[5] Rates in parts of Thailand are 60 per 100,000 per year.[5] It typically occurs in people in their 70s; however, in those with primary sclerosing cholangitis it often occurs in the 40s.[3] Rates of cholangiocarcinoma within the liver in the Western world have increased.[6]

  1. ^ a b c d e f g h i j k "Bile Duct Cancer (Cholangiocarcinoma) Treatment". National Cancer Institute. 23 September 2020. Retrieved 29 May 2021.
  2. ^ a b "cholangiocarcinoma". National Cancer Institute. 2 February 2011. Retrieved 21 January 2019.
  3. ^ a b c d e f g Razumilava N, Gores GJ (June 2014). "Cholangiocarcinoma". Lancet. 383 (9935): 2168–79. doi:10.1016/S0140-6736(13)61903-0. PMC 4069226. PMID 24581682.
  4. ^ a b c d "Bile Duct Cancer (Cholangiocarcinoma)". National Cancer Institute. 5 July 2018. Retrieved 21 January 2019.
  5. ^ a b c Bosman, Frank T. (2014). "Chapter 5.6: Liver cancer". In Stewart, Bernard W.; Wild, Christopher P (eds.). World Cancer Report (PDF). the International Agency for Research on Cancer, World Health Organization. pp. 403–12. ISBN 978-92-832-0443-5. Open access icon
  6. ^ a b c d Bridgewater JA, Goodman KA, Kalyan A, Mulcahy MF (2016). "Biliary Tract Cancer: Epidemiology, Radiotherapy, and Molecular Profiling". American Society of Clinical Oncology Educational Book. American Society of Clinical Oncology. Annual Meeting. 35 (36): e194-203. doi:10.1200/EDBK_160831. PMID 27249723.
  7. ^ Benavides M, Antón A, Gallego J, Gómez MA, Jiménez-Gordo A, La Casta A, et al. (December 2015). "Biliary tract cancers: SEOM clinical guidelines". Clinical & Translational Oncology. 17 (12): 982–7. doi:10.1007/s12094-015-1436-2. PMC 4689747. PMID 26607930.
  8. ^ Steele JA, Richter CH, Echaubard P, Saenna P, Stout V, Sithithaworn P, et al. (May 2018). "Thinking beyond Opisthorchis viverrini for risk of cholangiocarcinoma in the lower Mekong region: a systematic review and meta-analysis". Infectious Diseases of Poverty. 7 (1): 44. doi:10.1186/s40249-018-0434-3. PMC 5956617. PMID 29769113.
  9. ^ Zhang, Tan; Zhang, Sina; Jin, Chen; et al. (2021). "A Predictive Model Based on the Gut Microbiota Improves the Diagnostic Effect in Patients with Cholangiocarcinoma". Frontiers in Cellular and Infection Microbiology. 11: 751795. doi:10.3389/fcimb.2021.751795. PMC 8650695. PMID 34888258.