Difference between revisions of "Cholangiocarcinoma"

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#redirect [[Liver_neoplasms#Cholangiocarcinoma]]
'''Cholangiocarcinoma''' is a [[malignant]] [[liver neoplasms|primary tumour of the liver]].
 
It is also known as '''bile duct carcinoma'''.<ref>URL: [http://www.cancer.org/cancer/bileductcancer/detailedguide/bile-duct-cancer-what-is-bile-duct-cancer http://www.cancer.org/cancer/bileductcancer/detailedguide/bile-duct-cancer-what-is-bile-duct-cancer]. Access on: 23 May 2013.</ref>
 
==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.<ref>{{Ref GLP|608}}</ref>
*More common among asians.
 
Risks:
*Infection - liver flukes (endemic to Southeast Asia):
**''Clonorchis sinensis'' ([[AKA]] ''Opisthorchis sinensis''<ref name=Ref_PBoD926>{{Ref PBoD|926}}</ref>).<ref name=pmid18420495>{{Cite journal  | last1 = Park | first1 = do H. | last2 = Son | first2 = HY. | title = Images in clinical medicine. Clonorchis sinensis. | journal = N Engl J Med | volume = 358 | issue = 16 | pages = e18 | month = Apr | year = 2008 | doi = 10.1056/NEJMicm054461 | PMID = 18420495 |URL = http://www.nejm.org/doi/full/10.1056/NEJMicm054461 }}</ref>
**''Opisthorchis viverrini''.<ref name=pmid20202771>{{cite journal |author=de Martel C, Plummer M, Franceschi S |title=Cholangiocarcinoma: Descriptive epidemiology and risk factors |journal=Gastroenterol Clin Biol |volume= |issue= |pages= |year=2010 |month=March |pmid=20202771 |doi=10.1016/j.gcb.2010.01.008 |url=}}</ref>
*[[Caroli disease]] - rare congenital disease.<ref name=pmid17418061>{{cite journal |author=Ananthakrishnan AN, Saeian K |title=Caroli's disease: identification and treatment strategy |journal=Curr Gastroenterol Rep |volume=9 |issue=2 |pages=151–5 |year=2007 |month=April |pmid=17418061 |doi= |url=}}</ref>
*[[Primary sclerosing cholangitis]] - may be assoc. with inflammatory bowel disease (IBD), esp. [[ulcerative colitis]] (UC).
 
==Gross==
*Classically one large mass - outline described as ''cauliflower-like''.<ref name=pmid18425077>{{Cite journal  | last1 = Nakanishi | first1 = Y. | last2 = Zen | first2 = Y. | last3 = Kawakami | first3 = H. | last4 = Kubota | first4 = K. | last5 = Itoh | first5 = T. | last6 = Hirano | first6 = S. | last7 = Tanaka | first7 = E. | last8 = Nakanuma | first8 = Y. | last9 = Kondo | first9 = S. | title = Extrahepatic bile duct carcinoma with extensive intraepithelial spread: a clinicopathological study of 21 cases. | journal = Mod Pathol | volume = 21 | issue = 7 | pages = 807-16 | month = Jul | year = 2008 | doi = 10.1038/modpathol.2008.65 | PMID = 18425077 | URL = http://www.nature.com/modpathol/journal/v21/n7/full/modpathol200865a.html }}</ref>
**May have satellite nodules.
 
===Image===
<gallery>
Image:Cholangiocarcinoma.png | Cholangiocarcinoma. (WC)
</gallery>
==Microscopic==
Features:<ref name=Ref_GLP609>{{Ref GLP|609}}</ref>
*Usually an ''adenocarcinoma'', i.e. gland forming with:
**Cuboidal or columnar mucin producing cells, and
**A dense fibrous ([[desmoplastic stroma|desmoplastic]]) stroma.
 
Notes:
*Biliary stents lead to reactive changes,<ref name=pmid3877438>{{Cite journal  | last1 = Carrasco | first1 = CH | last2 = Wallace | first2 = S | last3 = Charnsangavej | first3 = C | last4 = Richli | first4 = W | last5 = Wright | first5 = KC | last6 = Fanning | first6 = T | last7 = Gianturco | first7 = C | title = Expandable biliary endoprosthesis: an experimental study. | journal = AJR Am J Roentgenol | volume = 145 | issue = 6 | pages = 1279-81 | month = Dec | year = 1985 | doi =  | PMID = 3877438 }}</ref> these can be confused for malignancy.  One must always check whether a biliary stent was in situ at time of biopsy.<ref>STC. 2 October 2009.</ref>
*Usually abundant desmoplasia, ergo hard to get good, i.e. diagnositic, endoluminal brushing specimens.<ref>STC. 6 December 2010.</ref>
*May have hyaline inclusions.<ref name=pmid20716803>{{Cite journal  | last1 = Aishima | first1 = S. | last2 = Fujita | first2 = N. | last3 = Mano | first3 = Y. | last4 = Iguchi | first4 = T. | last5 = Taketomi | first5 = A. | last6 = Maehara | first6 = Y. | last7 = Oda | first7 = Y. | last8 = Tsuneyoshi | first8 = M. | title = p62+ Hyaline inclusions in intrahepatic cholangiocarcinoma associated with viral hepatitis or alcoholic liver disease. | journal = Am J Clin Pathol | volume = 134 | issue = 3 | pages = 457-65 | month = Sep | year = 2010 | doi = 10.1309/AJCP53YVVJCNDZIR | PMID = 20716803 | URL = http://ajcp.ascpjournals.org/content/134/3/457.full.pdf }}
</ref>
 
DDx:
*Metastatic adenocarcinoma.
**[[Pancreatic adenocarcinoma]].
*Fulminant hepatic [[necrosis]].
**Bile ducts usu. left behind... look like well-differentiated adenocarcinoma.
*[[Bile duct adenoma]].
**No necrosis, no mitotic activity, no significant [[nuclear pleomorphism]].
 
===Images===
<gallery>
Image:Cholangiocarcinoma_-_low_mag.jpg | Cholangiocarcinoma - low mag. Shows the typical [[desmoplastic stroma]]. (WC/Nephron)
Image:Cholangiocarcinoma_-_high_mag.jpg |Cholangiocarcinoma - high mag. Shows a normal portal triad adjacent to atypical glandular cells within the interlobular septum and obvious tumour. (WC/Nephron)
</gallery>
www:
*[http://path.upmc.edu/cases/case296.html Cholangiocarcinoma & liver flukes - several images (upmc.edu)].
*[http://path.upmc.edu/cases/case420.html Cholangiocarcinoma - several images (upmc.edu)].
 
==IHC==
Classic IHC pattern:<ref>{{Ref GLP|609}}</ref>
*CK7 +ve.
*CK20 +ve/-ve.
*HepPar-1 -ve.
*AFP -ve.<ref>STC. 6 December 2010.</ref>
 
ICC vs. HCC:<ref name=pmid19173916>[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.</ref>
*ICC: CK19 (92.5%), MUC-1 (73.8%) +ve.
*HCC: HepPar-1 (85.6%), CD34 (87.8%) +ve.
 
HCC vs. ICC:<ref name=pmid16627262>{{cite journal |author=Lei JY, Bourne PA, diSant'Agnese PA, Huang J |title=Cytoplasmic staining of TTF-1 in the differential diagnosis of hepatocellular carcinoma vs cholangiocarcinoma and metastatic carcinoma of the liver |journal=Am. J. Clin. Pathol. |volume=125 |issue=4 |pages=519–25 |year=2006 |month=April |pmid=16627262 |doi=10.1309/59TN-EFAL-UL5W-J94M |url=}}</ref>
*TTF-1: ~90-100% +ve (cytoplasmic) in HCC vs. ~10% in cholangiocarcinoma.
 
==Sign out==
<pre>
MASS, PANCREAS, CORE BIOPSY:
- ADENOCARCINOMA, MODERATELY DIFFERENTIATED.
</pre>
 
Note:
*On biopsy, it isn't possible to cleanly separate from [[pancreatic adenocarcinoma]]. Thus, it is better to stay vague.
===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==
*[[Liver neoplasms]].
 
==References==
{{Reflist|2}}
 


[[Category:Diagnosis]]
[[Category:Diagnosis]]
[[Category:Liver pathology]]

Revision as of 19:02, 26 January 2014

Cholangiocarcinoma is a malignant primary tumour of the liver.

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

www:

IHC

Classic IHC pattern:[13]

  • CK7 +ve.
  • CK20 +ve/-ve.
  • HepPar-1 -ve.
  • AFP -ve.[14]

ICC vs. HCC:[15]

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

HCC vs. ICC:[16]

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

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. ↑ 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. ↑ STC. 6 December 2010.
  15. ↑ [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.
  16. ↑ 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.
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