Cholangiocarcinoma

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Cholangiocarcinoma is a malignant tumour that arise from the bile ducts and may been seen in the liver.

Cholangiocarcinoma
Diagnosis in short

Cholangiocarcinoma. H&E stain.
Synonyms bile duct carcinoma
LM atypical cuboidal or columnar mucin producing cohesive cells (carcinoma) - usually gland forming (adenocarcinoma), classically with a dense fibrous (desmoplastic) stroma
LM DDx metastatic carcinoma - esp. luminal GI tract, hepatocellular carcinoma
IHC CK7 +ve, CK20 +ve/-ve, CK19 +ve, HepPar-1 -ve, AFP -ve, EMA +ve
Site bile ducts (including pancreas, liver - see liver neoplasms)
Associated Dx liver flukes (Clonorchis sinensis, Opisthorchis viverrini), Caroli disease, primary sclerosing cholangitis - esp. in ulcerative colitis
Signs +/-jaundice
Prevalence uncommon
Blood work +/-findings of cholestasis
Prognosis poor
Clin. DDx other liver tumours - hepatocellular carcinoma, metastases
Treatment surgical resection if possible

It is also known as bile duct carcinoma.[1]

General

  • Malignancy of the biliary tree.
  • May be intrahepatic, i.e. intrahepatic cholangiocarcinoma (abbreviated ICC), or extrahepatic.

Epidemiology

  • Rare - approximately 1/5 the incidence of HCC.[2]
  • More common among asians.

Risks:

Gross

  • Classically one large mass - outline described as cauliflower-like.[7]
    • May have satellite nodules.

Image

  • Cholangiocarcinoma. (WC)

Microscopic

Features:[8]

  • Usually an adenocarcinoma, i.e. gland forming with:
    • Cuboidal or columnar mucin producing cells, and
    • A dense fibrous (desmoplastic) stroma.

Notes:

  • Biliary stents lead to reactive changes,[9] these can be confused for malignancy. One must always check whether a biliary stent was in situ at time of biopsy.[10]
  • Usually abundant desmoplasia, ergo hard to get good, i.e. diagnositic, endoluminal brushing specimens.[11]
  • May have hyaline inclusions.[12]

DDx:

Images

  • Cholangiocarcinoma - low mag. Shows the typical desmoplastic stroma. (WC/Nephron)

  • Cholangiocarcinoma - intermed. mag. (WC/Nephron)

  • Cholangiocarcinoma - high mag. Shows a normal portal triad adjacent to atypical glandular cells within the interlobular septum and obvious tumour. (WC/Nephron)

  • Cholangiocarcinoma - very high mag. (WC/Nephron)

www:

IHC

Classic IHC pattern:[13]

ICC vs. HCC:[14]

  • ICC: CK19 (92.5%), MUC-1 (73.8%) +ve.
  • HCC: HepPar-1 (85.6%), CD34 (87.8%) +ve.

HCC vs. ICC:[15]

  • TTF-1: ~90-100% +ve (cytoplasmic) in HCC vs. ~10% in cholangiocarcinoma.

Note:

  • MUC-1 is also known as EMA.

Molecular

  • Approx. 10% of the tumors carry a IDH-1 or IDH-2 hotspot mutation.[16]
  • FGFR2 gene fusions have been reported.[17]

Sign out

MASS, PANCREAS, CORE BIOPSY:
- ADENOCARCINOMA, MODERATELY DIFFERENTIATED.

Note:

Micro

The sections show an atypical gland-forming lesion (adenocarcinoma) in a fibrous background. This lesion is separate from the benign pancreatic glands that are present. The atypical glands are unequally spaced. Moderate-to-marked cytologic atypia is present. Mitotic activity is not readily apparent.

See also

References

  1. ↑ URL: http://www.cancer.org/cancer/bileductcancer/detailedguide/bile-duct-cancer-what-is-bile-duct-cancer. Access on: 23 May 2013.
  2. ↑ Iacobuzio-Donahue, Christine A.; Montgomery, Elizabeth A. (2005). Gastrointestinal and Liver Pathology: A Volume in the Foundations in Diagnostic Pathology Series (1st ed.). Churchill Livingstone. pp. 608. ISBN 978-0443066573.
  3. ↑ Cotran, Ramzi S.; Kumar, Vinay; Fausto, Nelson; Nelso Fausto; Robbins, Stanley L.; Abbas, Abul K. (2005). Robbins and Cotran pathologic basis of disease (7th ed.). St. Louis, Mo: Elsevier Saunders. pp. 926. ISBN 0-7216-0187-1.
  4. ↑ Park, do H.; Son, HY. (Apr 2008). "Images in clinical medicine. Clonorchis sinensis.". N Engl J Med 358 (16): e18. doi:10.1056/NEJMicm054461. PMID 18420495.
  5. ↑ de Martel C, Plummer M, Franceschi S (March 2010). "Cholangiocarcinoma: Descriptive epidemiology and risk factors". Gastroenterol Clin Biol. doi:10.1016/j.gcb.2010.01.008. PMID 20202771.
  6. ↑ Ananthakrishnan AN, Saeian K (April 2007). "Caroli's disease: identification and treatment strategy". Curr Gastroenterol Rep 9 (2): 151–5. PMID 17418061.
  7. ↑ Nakanishi, Y.; Zen, Y.; Kawakami, H.; Kubota, K.; Itoh, T.; Hirano, S.; Tanaka, E.; Nakanuma, Y. et al. (Jul 2008). "Extrahepatic bile duct carcinoma with extensive intraepithelial spread: a clinicopathological study of 21 cases.". Mod Pathol 21 (7): 807-16. doi:10.1038/modpathol.2008.65. PMID 18425077.
  8. ↑ Iacobuzio-Donahue, Christine A.; Montgomery, Elizabeth A. (2005). Gastrointestinal and Liver Pathology: A Volume in the Foundations in Diagnostic Pathology Series (1st ed.). Churchill Livingstone. pp. 609. ISBN 978-0443066573.
  9. ↑ Carrasco, CH; Wallace, S; Charnsangavej, C; Richli, W; Wright, KC; Fanning, T; Gianturco, C (Dec 1985). "Expandable biliary endoprosthesis: an experimental study.". AJR Am J Roentgenol 145 (6): 1279-81. PMID 3877438.
  10. ↑ STC. 2 October 2009.
  11. ↑ 11.0 11.1 STC. 6 December 2010.
  12. ↑ Aishima, S.; Fujita, N.; Mano, Y.; Iguchi, T.; Taketomi, A.; Maehara, Y.; Oda, Y.; Tsuneyoshi, M. (Sep 2010). "p62+ Hyaline inclusions in intrahepatic cholangiocarcinoma associated with viral hepatitis or alcoholic liver disease.". Am J Clin Pathol 134 (3): 457-65. doi:10.1309/AJCP53YVVJCNDZIR. PMID 20716803.
  13. ↑ Iacobuzio-Donahue, Christine A.; Montgomery, Elizabeth A. (2005). Gastrointestinal and Liver Pathology: A Volume in the Foundations in Diagnostic Pathology Series (1st ed.). Churchill Livingstone. pp. 609. ISBN 978-0443066573.
  14. ↑ [Evaluation of immunohistochemical markers for differential diagnosis of hepatocellular carcinoma from intrahepatic cholangiocarcinoma] Dong H, Cong WL, Zhu ZZ, Wang B, Xian ZH, Yu H. Zhonghua Zhong Liu Za Zhi. 2008 Sep;30(9):702-5. Chinese. PMID 19173916.
  15. ↑ Lei JY, Bourne PA, diSant'Agnese PA, Huang J (April 2006). "Cytoplasmic staining of TTF-1 in the differential diagnosis of hepatocellular carcinoma vs cholangiocarcinoma and metastatic carcinoma of the liver". Am. J. Clin. Pathol. 125 (4): 519–25. doi:10.1309/59TN-EFAL-UL5W-J94M. PMID 16627262.
  16. ↑ Wang, P.; Dong, Q.; Zhang, C.; Kuan, PF.; Liu, Y.; Jeck, WR.; Andersen, JB.; Jiang, W. et al. (Jun 2013). "Mutations in isocitrate dehydrogenase 1 and 2 occur frequently in intrahepatic cholangiocarcinomas and share hypermethylation targets with glioblastomas.". Oncogene 32 (25): 3091-100. doi:10.1038/onc.2012.315. PMID 22824796.
  17. ↑ Wu, YM.; Su, F.; Kalyana-Sundaram, S.; Khazanov, N.; Ateeq, B.; Cao, X.; Lonigro, RJ.; Vats, P. et al. (Jun 2013). "Identification of targetable FGFR gene fusions in diverse cancers.". Cancer Discov 3 (6): 636-47. doi:10.1158/2159-8290.CD-13-0050. PMID 23558953.
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