What are the treatment 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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Answer

The NCCN and ESMO guidelines concur that the only potentially curative treatment for cholangiocarcinoma is complete surgical resection with negative margins. However, few patients are diagnosed with early-stage resectable tumors. [36, 41]

For intrahepatic cholangiocarcinomas that are resected with microscopic margins or extrahepatic cholangiocarcinomas that are resected with negative margins and negative regional nodes, the NCCN recommends fluoropyrimidine chemoradiation, or fluoropyrimidine-based or gemcitabine-based chemotherapy.

For intrahepatic resections with residual local disease, gemcitabine/cisplatin combination therapy is a category 1 recommendation by the NCCN. Enrollment in clinical trials, and fluoropyrimidine-based or gemcitabine-based chemotherapy are also options. [36]

For extrahepatic resections with positive margins, residual disease or positive regional nodes, the NCCN recommends fluoropyrimidine chemoradiation followed by fluoropyrimidine-based or gemcitabine-based chemotherapy. For positive regional nodes, fluoropyrimidine-based or gemcitabine-based chemotherapy is also an option.

For management of patients with unresectable or metastatic disease, NCCN makes the following recommendations [36] :

  • Gemcitabine/cisplatin combination therapy (category 1)
  • Enrollment in clinical trial
  • Fluoropyrimidine or gemcitabine chemotherapy
  • Fluoropyrimidine chemoradiation Locoregional therapy
  • Supportive care

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