What are the diagnostic guidelines for cholangiocarcinoma (CCC)?

Updated: Dec 28, 2019
  • Author: Peter E Darwin, MD; Chief Editor: N Joseph Espat, MD, MS, FACS  more...
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The National Comprehensive Cancer Network (NCCN) recommends the following intrahepatic cholangiocarcinoma workup in patients with an isolated intrahepatic mass that has imaging characteristics consistent with malignancy but not consistent with hepatocellular carcinoma [36] :

  • History and physical examination
  • Multiphasic abdominal/pelvic computed tomography (CT)/magnetic resonance imaging (MRI) with intravenous contrast
  • Chest CT with or without contrast
  • Consider baseline carcinoembryonic antigen (CEA) and CA 19-9 testing
  • Liver function tests (LFTs)
  • Surgical consultation
  • Esophagogastroduodenoscopy (EGD) and colonoscopy (to assess for T3 disease and rule out endobiliary metastasis from colorectal cancer mimicking intrahepatic cholangiocarcinoma [39] )
  • Consider viral hepatitis serologies
  • Consider biopsy
  • Consider alpha fetoprotein (AFP) testing

For the workup of extrahepatic cholangiocarcinoma, in patients who present with pain, jaundice, abnormal LFTs, and obstruction or abnormality on imaging, the NCCN recommends the following workup [36] :

  • History and physical examination
  • Multiphasic abdominal/pelvic CT/MRI (to assess for vascular invasion) with IV contrast
  • Chest CT with or without contrast
  • Cholangiography
  •  Consider baseline CEA and CA 19-9
  • LFTs
  • Consider endoscopic ultrasound (EUS) after surgical consultation
  • Consider serum IgG4 to rule out autoimmune cholangitis

Guidelines from the American Society for Gastrointestinal Endoscopy (ASGE) recommend magnetic resonance cholangiography (MRCP) to assess for resectability if a CT scan suggests cholangiocarcinoma. ERCP is recommended to obtain tissue or facilitate further evaluation of indeterminate strictures. [40]

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