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cholangiocarcinoma

Etiology: - disease associations a) sclerosing cholangitis b) choledochal cysts c) liver fluke infection - Fasciola hepatica - Opisthorchis viverrini - Clonorchis sinensis [5] - 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: 1) liver function tests a) increased serum alkaline phosphatase b) increased serum gamma-glutamyl transferase c) conjugated bilirubin may be increased 2) serum carcinoembryonic antigen (CEA) may be increased 3) cancer antigen CA 19-9 may be increased 4) biopsy or fine-needle aspiration 5) albumin in situ hybridization assay if adenocacinoma specific for cholangiocarcinoma [5] Special laboratory: - ERCP - visualization, tissue sampling - may be combined with cholangiography Radiology: 1) ultrasound may show dilated bile ducts proximal to the tumor 2) computed tomography (CT) - intrahepatic biliary dilation - tumor enhances poorly with contrast agents (image) [5] 3) cholangiography may aid in identification & localization of the tumor Management: 1) surgical resection if possible - only 25-30% of patients present early with resectable disease 2) 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 3) relief of biliary obstruction a) ERCP b) percutaneous drainage c) placement of expandable metal stent 4) liver transplantation [2] - unresectablle hilar cholangiocarcinoma < 3 cm without metastases [1] - tumor recurrence after liver transplantation is frequent

Interactions

disease interactions

Related

bile duct cancer; extrahepatic biliary carcinoma primary sclerosing cholangitis (PSC)

General

adenocarcinoma biliary carcinoma liver cancer; hepatobiliary carcinoma

References

  1. Medical Knowledge Self Assessment Program (MKSAP) 11, 15, 16, 17, 18, 19. American College of Physicians, Philadelphia 1998, 2009, 2012, 2015, 2018, 2021
  2. Blechacz BR, Gores GJ. Cholangiocarcinoma. Clin Liver Dis. 2008 Feb;12(1):131-50 PMID: 18242501
  3. Gatto M, Alvaro D. Cholangiocarcinoma: risk factors and clinical presentation. Eur Rev Med Pharmacol Sci. 2010 Apr;14(4):363-7. PMID: 20496549
  4. Razumilava N, Gores GJ. Classification, diagnosis, and management of cholangiocarcinoma. Clin Gastroenterol Hepatol. 2013 Jan;11(1):13-21.e1 PMID: 22982100
  5. Rothaus C A Woman with Cholangiocarcinoma. NEJM Resident 360. March 17, 2021 https://resident360.nejm.org/clinical-pearls/a-woman-with-cholangiocarcinoma
  6. NEJM Knowledge+ Gastroenterology