Cholangiocarcinoma - Symptoms, diagnosis and treatment | BMJ Best Practice

Last reviewed:September 2019

Last updated:December 2017

Summary

Painless jaundice, weight loss, and abdominal pain in patient >55 years old.

Liver enzymes, blood levels of CA 19-9, CEA, CA-125; abdominal ultrasound, abdominal CT/MRI, MR angiography, and cholangiography (ERCP, MRCP, percutaneous transhepatic catheterization) are used for evaluation.

Surgical resection offers only potential cure.

Chemotherapy has been shown to be relatively ineffective.

Liver transplant is indicated in a small subset of patients.

Definition

Cholangiocarcinomas are cancers arising from the bile duct epithelium. These can be divided depending on their location in the biliary tree: intrahepatic or extrahepatic (perihilar and distal). Perihilar tumors involving the bifurcation of the ducts are also known as Klatskin tumors. More than 95% are adenocarcinomas. Most are of the infiltrating nodular or diffusely infiltrating type. Purely nodular or papillary are less frequent subtypes.

History and exam

Key diagnostic factors

painless jaundice
weight loss
abdominal pain

Other diagnostic factors

pruritus
triad of fever, jaundice, and RUQ pain
palpable gallbladder
hepatomegaly
dark urine
pale stools

Risk factors

age >50 years
cholangitis
choledocholithiasis
cholecystolithiasis
other structural disorders of the biliary tract
ulcerative colitis (UC)
primary sclerosing cholangitis (PSC)
nonspecific cirrhosis
alcoholic liver disease
liver fluke infection
chronic typhoid carrier
hepatitis C virus (HCV)
HIV
hepatitis B virus (HBV)
exposure to thorium dioxide
diabetes
cigarette smoking
exposure to toxins/medications
male sex

Diagnostic investigations

1st investigations to order

serum bilirubin
serum alk phos
serum gamma-GT
serum aminotransferase
serum prothrombin time
serum CA 19-9
serum CEA
serum CA 19-9 + (40 times CEA)
serum CA-125
abdominal ultrasound

Investigations to consider

abdominal CT
abdominal MRI
MR angiography
ERCP
MRCP
percutaneous transhepatic catheterization (PTC)
positron emission tomography (PET)
Immunostaining

Emerging tests

optical coherence tomography (OCT)
peroral cholangioscopy
duodenoscope-assisted cholangioscopy

Treatment algorithm

ACUTE

resectable disease

unresectable disease

Contributors

AuthorsVIEW ALL 

N. Joseph Espat, MD, MS, FACS

Chairman of Surgery

Professor and Chief Surgical Oncology

Director Cancer Center

Department of Surgery

Roger Williams Medical Center

Boston University School of Medicine

Providence

RI

Disclosures

NJE has taught faculty courses for Medtronic and Sirtex.

Ponnandai Somasundar, MD, MPH, FACS

Associate Professor and Associate Chief

Surgical Oncology

Department of Surgery

Roger Williams Medical Center

Boston University School of Medicine

Providence

RI

Disclosures

PS declares that he has no competing interests.

Peer reviewersVIEW ALL 

Kevin Watkins, MD

Assistant Professor

Department of Surgery

Health Science Center

SUNY

Stony Brook

NY

Disclosures

KW declares that he has no competing interests.

Savio Reddymasu, MD

GI Fellow

Department of Medicine

Center for Gastrointestinal Motility

Division of Gastroenterology and Hepatology

University of Kansas Medical Center

Kansas City

KS

Disclosures

SR declares that he has no competing interests.

Satvinder Mudan, MBBS, BSc, MD, FRCS

Consultant in Surgical Oncology

The Royal Marsden Hospital

London

UK

Disclosures

SM declares that he has no competing interests.

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