cholangiocarcinoma
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Etiology
- disease associations
- idiopathic (most cases)
Epidemiology
- accounts for 1/3 of primary liver cancer in USA
- 2nd most common liver cancer after hepatocellular carcinoma[1]
- 8000 cases/year in U.S.[5]
Pathology
- adenocarcinoma
- immunohistochemistry: CK7+, CK19+, CK20-*
* also seen in patients with metastatic cancers of the pancreas, stomach, small bowel, & less commonly colon[5]
Genetics
- implicated gene: RASGEF1A
- FGFR2 fusion or rearrangement present in 13-14%
Clinical manifestations
- jaundice with or without abdominal pain
- +/- weight loss, nausea, anorexia, pruritus
Laboratory
- liver function tests
- increased serum alkaline phosphatase
- increased serum gamma-glutamyl transferase
- conjugated bilirubin may be increased
- serum carcinoembryonic antigen (CEA) may be increased
- cancer antigen CA 19-9 may be increased
- biopsy or fine-needle aspiration
- albumin in situ hybridization assay if adenocacinoma specific for cholangiocarcinoma[5]
Diagnostic procedures
- ERCP
- visualization, tissue sampling
- may be combined with cholangiography
Radiology
- ultrasound may show dilated bile ducts proximal to the tumor
- computed tomography (CT)
- intrahepatic biliary dilation
- tumor enhances poorly with contrast agents (image)[5]
- cholangiography may aid in identification & localization of the tumor
Management
- surgical resection if possible
- only 25-30% of patients present early with resectable disease
- responds poorly to chemotherapy & radiation
- pemigatinib is the 1st FDA-approved agent for cholangiocarcinoma with FGFR2 fusion or rearrangement[5]
- futibatinib (Lytgobi) FDA-approved for previously treated, unresectable, locally advanced or metastatic intrahepatic cholangiocarcinoma harboring FGFR2 gene fusions or other rearrangements
- relief of biliary obstruction
- ERCP
- percutaneous drainage
- placement of expandable metal stent
- liver transplantation[2]
- unresectablle hilar cholangiocarcinoma < 3 cm without metastases[1]
- tumor recurrence after liver transplantation is frequent
More general terms
Additional terms
References
- ↑ Jump up to: 1.0 1.1 1.2 Medical Knowledge Self Assessment Program (MKSAP) 11, 15, 16, 17, 18, 19. American College of Physicians, Philadelphia 1998, 2009, 2012, 2015, 2018, 2021
- ↑ Jump up to: 2.0 2.1 Blechacz BR, Gores GJ. Cholangiocarcinoma. Clin Liver Dis. 2008 Feb;12(1):131-50 PMID: https://www.ncbi.nlm.nih.gov/pubmed/18242501
- ↑ Gatto M, Alvaro D. Cholangiocarcinoma: risk factors and clinical presentation. Eur Rev Med Pharmacol Sci. 2010 Apr;14(4):363-7. PMID: https://www.ncbi.nlm.nih.gov/pubmed/20496549
- ↑ Razumilava N, Gores GJ. Classification, diagnosis, and management of cholangiocarcinoma. Clin Gastroenterol Hepatol. 2013 Jan;11(1):13-21.e1 PMID: https://www.ncbi.nlm.nih.gov/pubmed/22982100
- ↑ Jump up to: 5.0 5.1 5.2 5.3 5.4 5.5 5.6 Rothaus C A Woman with Cholangiocarcinoma. NEJM Resident 360. March 17, 2021 https://resident360.nejm.org/clinical-pearls/a-woman-with-cholangiocarcinoma
- ↑ NEJM Knowledge+ Gastroenterology