Primary Sclerosing Cholangitis and Cholangiocarcinoma

Publication Date: September 8, 2022
Last Updated: September 15, 2022

Primary Sclerosing Cholangitis

Diagnosis of PSC

  • In patients with suspected PSC, a 3D MRI/MRCP with T1w and T2w axial images and contrast enhancement should be obtained to evaluate for cholangiographic features of PSC, including intrahepatic and/orextrahepatic strictures alternating with normal or slightly dilated segments.
  • In patients with suspected PSC and a normal, high-quality MRI/MRCP, liver biopsy should be considered to rule out small-duct PSC. Patients with an equivocal MRI/MRCP should be referred to an experienced center for consideration of a repeat high-quality MRI/MRCP or liver biopsy. A repeat MRI/MRCP may be considered in 1 year if the diagnosis remains unclear.
  • ERCP should be avoided for the diagnosis of PSC.
  • In all patients with possible PSC, serum IgG4 levels should be measured to exclude IgG4-sclerosing cholangitis.
  • A liver biopsy should not be performed in patients with typical cholangiographic findings on MRI/MRCP, except when there is concern for autoimmune hepatitis overlap.
  • Ileocolonoscopy with biopsies should be performed in patients with a new diagnosis of PSC and no previous diagnosis of IBD. In patients without IBD, subsequent ileocolonoscopy should be considered at 5-year intervals or whenever symptoms suggestive of IBD occur.
  • Patients with small-duct PSC should be monitored by MRI/MRCP every 3–5 years for the development of large-duct disease.
  • Risk stratification and fibrosis staging should be done at diagnosis of PSC and regularly during follow-up. Clinical risk tools can be considered for this purpose, but specific probabilities of events should be interpreted with caution in the individual patient.
  • Liver stiffness measurement by transient elastography or magnetic resonance elastographyis currently the preferred method for estimation of fibrosis stage in PSC.
  • Liver biopsy is not recommended for fibrosis staging in clinical practice.

Cholangiocarcinoma

iCCA

Diagnosis

  • An elevated CA 19-9 alone should not be used to diagnose CCA.
  • Histopathological confirmation is required for definitive diagnosis of iCCA.
  • Cross-sectional imaging of the liver such as multiphasic CT or MRI are required to facilitate assessment of the primary mass, vascular invasion, presence of intra- or extrahepatic metastasis, and resectability.
  • Cross-sectional imaging of the chest and abdomen is necessary to stage the disease.
  • PET scan should not be used for diagnosis of primary tumor in CCA.

Surgical  resection

  • Surgical resection is the treatment of choice for patients with a single iCCA nodule in a resectable location without evidence of metastatic disease and who have adequate functional liver volume.
  • Patients diagnosed with iCCA should be referred to a center with surgical expertise in hepatobiliary malignancies.
  • Adjuvant capecitabine should be considered for all patients with CCA.

LT for iCCA

  • Liver transplantation for unresectable liver-limited iCCA should only be considered under research protocols.

LRT

  • Data are insufficient to recommend locoregional therapy as a standard therapy for locally advanced unresectable iCCA.

Overview

Title

Primary Sclerosing Cholangitis and Cholangiocarcinoma

Authoring Organization

American Association for the Study of Liver Diseases