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Difference between revisions of "Cholangiocarcinoma"
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'''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]] | |||