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Biliary cancer

Last updated: February 9, 2021

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Biliary tract cancers include cholangiocarcinoma and gallbladder carcinoma, which are both rare diseases with very poor prognoses. Cholangiocarcinoma is classified as either intrahepatic or extrahepatic according to the anatomical site of the tumor. Extrahepatic carcinoma, which is the more common form, can be further classified as perihilar (Klatskin tumor; occurs at the bifurcation of the hepatic duct) and distal extrahepatic carcinoma. Risk factors for cholangiocarcinoma include primary sclerosing cholangitis and chronic biliary tract inflammation. The greatest risk factor for gallbladder carcinoma is cholelithiasis. Patients are often initially asymptomatic or only present with nonspecific symptoms (e.g., abdominal pain, fever, weight loss) until late stages of disease, meaning that most tumors are already advanced at the time of diagnosis. Extrahepatic cholangiocarcinoma may manifest with signs of cholestasis (e.g., jaundice, dark urine, pruritus) and a painless, enlarged gallbladder (Courvoisier sign). If liver transaminases and tumor markers are raised and/or ultrasound imaging suggests bile duct or gallbladder carcinoma, MRCP or MDCT are recommended for diagnosis. Although surgical resection of early-stage tumors is curative, approximately 90% of patients have more advanced, unresectable tumors at the time of diagnosis.

Cholangiocarcinoma [2][4]

Gallbladder carcinoma [5]

  • Incidence: ∼ 2/100,000 per year in the US but significantly higher in India, Chile, and Eastern Europe
  • Sex: >

References:[4][6]

Epidemiological data refers to the US, unless otherwise specified.

Chronic gallbladder inflammation increases the risk of gallbladder carcinoma.

Clinical features depend on the location and stage of the tumor.

Cholangiocarcinoma [2]

Gallbladder carcinoma [4][5]

The diagnostic approach varies depending on the location of the tumor and whether PSC is present. If the patient's presentation, laboratory findings (especially tumor markers), and/or transabdominal ultrasound suggest biliary cancer, then MRCP or MDCT is generally recommended for diagnosis. Biopsy is generally unnecessary.

Gallbladder carcinoma is usually diagnosed incidentally after elective cholecystectomy.

References:[4][9]

The differential diagnoses listed here are not exhaustive.

The prognosis for cholangiocarcinoma and gallbladder cancer is generally poor, especially for gallbladder cancer and intrahepatic cholangiocarcinoma. Treatment is determined by tumor resectability.

Resectable disease [4]

  • < 10% of cases are resectable.
  • Resection is the only curative therapy.
  • Procedure: radical resection plus lymphadenectomy
    • Preoperative biliary drainage is performed in some cases.
    • Adjuvant chemotherapy: usually a fluoropyrimidine-based regimen
Surgical approach Contraindications
Intrahepatic cholangiocarcinoma
  • Hepatic resection: usually partial resection (in rare cases, liver transplant)
Extrahepatic cholangiocarcinoma
  • Complete resection and regional lymphadenectomy
Gallbladder carcinoma
  • Cholecystectomy and
    • Resection of hepatic segments IVb and V
    • Lymphadenectomy
    • With or without bile duct excision

Unresectable disease [4]

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  2. Nestor F. Esnaola, Joshua E. Meyer, Andreas Karachristos, Jennifer L. Maranki, E. Ramsay Camp, Crystal S. Denlinger. Evaluation and management of intrahepatic and extrahepatic cholangiocarcinoma. Cancer. 2016; 122 (9): p.1349-1369. doi: 10.1002/cncr.29692 . | Open in Read by QxMD
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  5. Valle JW, Borbath I, Khan SA, Huguet F, Gruenberger T, Arnold D. Biliary cancer: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up†. Annals of Oncology. 2016; 27 (suppl_5): p.v28-v37. doi: 10.1093/annonc/mdw324 . | Open in Read by QxMD
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  13. What are the risk factors for bile duct cancer?. http://www.cancer.org/cancer/bileductcancer/detailedguide/bile-duct-cancer-risk-factors. Updated: January 11, 2014. Accessed: December 22, 2016.
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