Cholangiocarcinoma Guidelines

Updated: Apr 20, 2020
  • Author: Peter E Darwin, MD; Chief Editor: N Joseph Espat, MD, MS, FACS  more...
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Guidelines

Guidelines Summary

Guidelines Contributor:   Elwyn C Cabebe, MD Physician Partner, Valley Medical Oncology Consultants; Medical Director of Oncology, Clinical Liason Physician, Cancer Care Committee, Good Samaritan Hospital

Diagnosis

The National Comprehensive Cancer Network (NCCN) recommends the following intrahepatic cholangiocarcinoma workup in patients with an isolated intrahepatic mass that has imaging characteristics consistent with malignancy but not consistent with hepatocellular carcinoma [36] :

  • History and physical examination
  • Multiphasic abdominal/pelvic computed tomography (CT)/magnetic resonance imaging (MRI) with intravenous contrast
  • Chest CT with or without contrast
  • Consider baseline carcinoembryonic antigen (CEA) and CA 19-9 testing
  • Liver function tests (LFTs)
  • Surgical consultation
  • Esophagogastroduodenoscopy (EGD) and colonoscopy (to assess for T3 disease and rule out endobiliary metastasis from colorectal cancer mimicking intrahepatic cholangiocarcinoma [40] )
  • Consider viral hepatitis serologies
  • Consider biopsy
  • Consider alpha fetoprotein (AFP) testing

For the workup of extrahepatic cholangiocarcinoma, in patients who present with pain, jaundice, abnormal LFTs, and obstruction or abnormality on imaging, the NCCN recommends the following workup [36] :

  • History and physical examination
  • Multiphasic abdominal/pelvic CT/MRI (to assess for vascular invasion) with IV contrast
  • Chest CT with or without contrast
  • Cholangiography
  •  Consider baseline CEA and CA 19-9
  • LFTs
  • Consider endoscopic ultrasound (EUS) after surgical consultation
  • Consider serum IgG4 to rule out autoimmune cholangitis

Guidelines from the American Society for Gastrointestinal Endoscopy (ASGE) recommend magnetic resonance cholangiography (MRCP) to assess for resectability if a CT scan suggests cholangiocarcinoma. ERCP is recommended to obtain tissue or facilitate further evaluation of indeterminate strictures. [41]

Staging

Cholangiocarcinoma cancer staging follows the tumor-node-metastasis (TNM) classification of the American Joint Cancer Committee/Union for International Cancer Control/ (AJCC/UICC) and is staged separately for intrahepatic, perihilar, and distal bile duct tumors. [24]

TNM groupings by stage are as follows for each group: [24]

Table. 1 (Open Table in a new window)

Intrahepatic bile duct tumor

Stage

T

N

M

0

Tis

N0

M0

IA

T1a

N0

M0

IB

T1b

N0

M0

II

T2

N0

M0

IIIA

T3

N0

M0

IIIB

T4

N0

M0

 

Any T

N1

M0

IV

Any T

Any N

M1

Table. 2 (Open Table in a new window)

Perihilar bile duct tumor

Stage

T

N

M

0

Tis

N0

M0

I

T1

N0

M0

II

T2a-b

N0

M0

IIIA

T3

N0

M0

IIIB

T4

N0

M0

IIIC

Any T

N1

M0

IVA

T4

N2

M0

IVB

Any T

Any N

M1

Table. 3 (Open Table in a new window)

Distal bile duct tumor

Stage

T

N

M

0

Tis

N0

M0

I

T1

N0

M0

IIA

T1

N1

M0

 

T2

N0

M0

IIB

T2

N1

M0

 

T3

N0

M0

 

T3

N1

M0

IIIA

T1-3

N2

M0

IIIB

T4

N0-2

M0

IV

Any T

Any N

M1

Treatment

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, 42]

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