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Cholangiocarcinoma

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 2023; 2016), the European Association for the Study of the Liver (EASL/ILCA 2023), the Society for Immunotherapy of Cancer (SITC 2023), the Enhanced Recovery After Surgery Society (ERASS 2023), the European Association for the Study of the Liver (EASL 2023; 2022), the American Society for Radiation Oncology (ASTRO 2022), the American Society for Gastrointestinal Endoscopy (ASGE 2021; 2013), the International Liver Transplantation Society (ILTS 2020), the American Society of Clinical Oncology (ASCO 2019), the British Society of Gastroenterology (BSG 2019), the American College of Radiology (ACR 2019), and the American Association for the Study of Liver Diseases (AASLD 2014).
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Guidelines

1.Screening and diagnosis

Indications for screening, primary sclerosing cholangitis, ILCA/EASL: 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)

2.Classification and risk stratification

Classification
As per EASL 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

3.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)

4.Diagnostic procedures

Exploratory laparoscopy: 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

5.Medical management

General principles
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)

6.Therapeutic procedures

Radiotherapy, indications, ILCA/EASL: 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

7.Perioperative care

Preoperative care, counseling
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)

  • Postoperative care (thromboprophylaxis)

  • Postoperative care (early mobilization)

  • Postoperative care (nutrition)

  • Postoperative care (antiemetics)

  • Postoperative care (laxatives)

8.Surgical interventions

Surgical resection, indications, ILCA/EASL
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)

9.Specific circumstances

Pregnant patients: 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

10.Preventative measures

Primary prevention
As per EASL 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

11.Follow-up and surveillance

Assessment of treatment response
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