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Cholangiocarcinoma

What's new

The European Society of Medical Oncology (ESMO) has released an interim update on the management of advanced biliary tract cancer. For first-line therapy, cisplatin-gemcitabine-durvalumab and cisplatin-gemcitabine-pembrolizumab regimens are recommended (TOPAZ-1 and KEYNOTE-966 trials). For second- and later-line therapy, options include futibatinib or pemigatinib for FGFR2 fusions or rearrangements (FOENIX and FIGHT-202 trials), entrectinib, larotrectinib, or repotrectinib for NTRK fusions (phase 1/2 trials), selpercatinib for RET fusions (LIBRETTO-001 trial), trastuzumab deruxtecan for HER2 overexpression or amplification (MyPathway, KCSG-HB19-14, DESTINY-PanTumor02, and SGNTUC-019 trials; FDA-approved), and zanidatamab for previously treated HER2-positive disease (HERIZON-BTC-01 trial; FDA-approved). .

Guidelines

Key sources

The following summarized guidelines for the evaluation and management of cholangiocarcinoma are prepared by our editorial team based on guidelines from the European Society of Medical Oncology (ESMO 2025,2023,2016), the Surgical Infection Society (SIS 2024), the Enhanced Recovery After Surgery Society (ERASS 2023), the European Association for the Study of the Liver (EASL 2023,2022), the European Association for the Study ...
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Screening and diagnosis

Indications for screening, PSC: as per EASL/ILCA 2023 guidelines, consider obtaining annual surveillance for cholangiocarcinoma with noninvasive radiologic modalities, preferably with MRI plus MRCP, in patients with PSC.
C
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  • Indications for screening (liver cirrhosis)

  • Indications for screening (liver flukes)

Classification and risk stratification

Classification
As per EASL/ILCA 2023 guidelines:
Consider subclassifying intrahepatic cholangiocarcinoma into large duct type and small duct type, as this may have clinical utility based on its prognostic and therapeutic implications.
C
Consider obtaining intrahepatic cholangiocarcinoma macro classification in combination with pathological subclassification.
C
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  • Prognosis

Diagnostic investigations

Diagnostic imaging: as per ESMO 2023 guidelines, obtain radiological imaging before ERCP or percutaneous transhepatic cholangiography in patients with jaundice.
B
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  • Imaging for staging (CT/MRI)

  • Imaging for staging (PET)

  • Imaging for staging (EUS)

Diagnostic procedures

Exploratory laparoscopy: as per ESMO 2016 guidelines, consider performing staging laparoscopy on an individual basis to exclude the presence of peritoneal metastases, if it will influence the decision to proceed with major resection.
E
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  • Biopsy and histopathology

  • Molecular testing

Medical management

General principles
As per ESMO 2016 guidelines:
Provide patients with biliary tract cancer with a designated point of contact within the multidisciplinary team for advice and support (such as a nurse specialist).
E
Ensure full access to palliative care and symptom management (including pain control) for patients with biliary tract cancer.
E
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  • Neoadjuvant chemotherapy

  • Adjuvant chemotherapy

  • Definitive systemic therapy (first-line therapy)

  • Definitive systemic therapy (second- and later-line therapy)

Therapeutic procedures

Radiotherapy, indications: as per EASL/ILCA 2023 guidelines, insufficient evidence to recommend for or against external beam ablative dose radiotherapy as an alternative to systemic therapy in patients with unresectable liver-limited intrahepatic cholangiocarcinoma.
I
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  • Radiotherapy (technical considerations)

  • Local ablation

  • Intra-arterial therapies

  • Right portal vein embolization

  • Photodynamic therapy

  • Biliary stenting

Perioperative care

Preoperative care, counseling
As per ERASS 2023 guidelines:
Provide preoperative information and counseling regarding the upcoming liver surgery. Consider using brochures and multimedia support to improve verbal counseling.
B
Advise preoperative smoking cessation at least 4 weeks before hepatectomy. Advise alcohol cessation in heavy drinkers (> 24 g/day for females or > 36 g/day for males) 4-8 weeks before surgery.
A
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  • Preoperative care (nutrition)

  • Preoperative care (rehabilitation)

  • Preoperative care (biliary drainage)

  • Preoperative care (antibiotic prophylaxis)

  • Preoperative care (corticosteroids)

  • Preoperative care (preanesthetic medication)

  • Intraoperative care (anesthesia and analgesia)

  • Intraoperative care (temperature management)

  • Intraoperative care (fluid management)

  • Intraoperative care (bleeding management)

  • Postoperative care (thromboprophylaxis)

  • Postoperative care (early mobilization)

  • Postoperative care (nutrition)

  • Postoperative care (antiemetics)

  • Postoperative care (laxatives)

Surgical interventions

Surgical resection, indications
As per EASL/ILCA 2023 guidelines:
Consider performing tumor resection in selected patients with multifocal, unilobar intrahepatic cholangiocarcinoma.
C
Insufficient evidence to recommend resection over locoregional and/or systemic treatments in patients with intrahepatic cholangiocarcinoma and macroscopic vascular involvement of the IVC, hepatic vein, or portal vein.
I
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  • Surgical resection (technical considerations)

  • Liver transplantation (indications)

  • Liver transplantation (technical considerations)

Specific circumstances

Pregnant patients: as per EASL 2023 guidelines, obtain a case-by-case evaluation by a multidisciplinary team to consider diagnostic and therapeutic strategies based on symptoms and prognosis in pregnant patients with cholangiocarcinoma.
B
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  • Patients with gastric outlet obstruction

Preventative measures

Primary prevention
As per EASL/ILCA 2023 guidelines:
Consider developing educational campaigns toward changing behavior to prevent liver fluke infection and re-infection.
C
Insufficient evidence to recommend hepatic resection as a strategy to prevent intrahepatic cholangiocarcinoma in patients with hepatolithiasis.
I
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Follow-up and surveillance

Assessment of treatment response
As per ESMO 2023 guidelines:
Consider obtaining CA 19-9 for the assessment of treatment response.
C
Re-assess patients by a multidisciplinary team to discuss surgery in case of response following locoregional or systemic treatment of locally advanced tumors.
B
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  • Follow-up

  • Rehabilitation