This is the sixth part of our series about the condition based on our patient booklet “Fast Facts for Patient and Supporters: Cholangiocarcinoma”. This article lists the treatments and schedules for cholangiocarcinoma.

Adjuvant Therapy

Typically, adjuvant treatment is with capecitabine given by mouth for 6 months, or gemcitabine given in combination with capecitabine for 3 months, followed by radiation therapy with capecitabine as radiosensitization.

Capecitabine may be used as adjuvant therapy for cholangiocarcinomas located anywhere in the bile duct system, while gemcitabine/capecitabine with capecitabine/radiation is typically used for people with extrahepatic cholangiocarcinomas.

Neoadjuvant Therapy

Neoadjuvant therapy uses the same medications as those used for palliative treatment (see below) and/or involves radiation therapy given with radiosensitizing chemotherapy.

Palliative Therapy

The aim is to control the growth of tumors that cannot be fully removed.

First-Line Therapy

First-line therapy is the first treatment you have. It typically involves two chemotherapy drugs: cisplatin and gemcitabine. Treatment is given by vein weekly for two consecutive weeks, followed by a week’s break (a 3-week cycle).

Some people with kidney or liver problems may not be able to tolerate one or both drugs. In this case, oxaliplatin may be used in place of cisplatin. 5-fluorouracil (5-FU) or capecitabine can be used in place of gemcitabine. In addition, nab-paclitaxel may be added to cisplatin and gemcitabine, or used in place of platinum drugs (cisplatin/oxaliplatin) in certain circumstances. These drugs may have different treatment schedules.

Second-Line Therapy

Second-line therapy is given after your tumor grows during or after first-line therapy or if you are not able to tolerate first-line therapy.

Biomarker testing may identify second-line immunotherapy or targeted therapy options. The treatments described below may not be available in some areas.

There are several immunotherapy medications that may be used in place of chemotherapy for tumors described as microsatellite instability-high (MSI-H) or mismatch repair deficient (dMMR). They may also be used in tumors that have been identified to have a high tumor mutational burden (TMB).

For tumors with an NTRK gene fusion, targeted therapies have been developed that help stop the effects of the fusion on tumor growth. Larotrectinib and entrectinib are examples.

Similarly, targeted therapies for abnormalities in the FGFR2 gene (fusions or other rearrangements) can stop the genetic changes from promoting tumor growth. Pemigatinib is an example.

If no molecular signature is identified or a targeted drug is not available, 5-FU or capecitabine is typically used, together with oxaliplatin. Other options include 5-FU or capecitabine if gemcitabine was used as first-line therapy, or gemcitabine if 5-FU or capecitabine was used initially. Other options may include 5-FU in combination with irinotecan, or regorafenib, which targets blood vessels within the tumor.

Third-Line Therapy

Third-line therapy may be available. The options will depend on the therapies you received in the past and the availability of therapies for your particular type of cancer.

Please check out the other posts of our series here:

• What Is Cholangiocarcinoma, How Does It Develop and What Are Its Symptoms?
• Cholangiocarcinoma: Seeing Your Doctor & Who Is Who
• Cholangiocarcinoma: Diagnostic Tests, Procedures and Staging
• How Can Cholangiocarcinoma Be Treated by Surgery and Radiotherapy?
• How Can Cholangiocarcinoma Be Treated by Systemic Therapy and Infusion Therapy?

Information based on Fast Facts for Patients and their Supporters: Cholangiocarcinoma (Karger, 2021).