title: Update on the Diagnosis and Treatment of Cholangiocarcinoma.
abstract: OBJECTIVE
  Cholangiocarcinoma is a rare biliary adenocarcinoma associated with poor outcomes. Cholangiocarcinoma is subdivided into extrahepatic and intrahepatic variants. Intrahepatic cholangiocarcinoma is then further differentiated into (1) peripheral mass-forming tumors and (2) central periductal infiltrating tumors. We aimed to review the currently known risk factors, diagnostic tools, and treatment options, as well as highlight the need for further clinical trials and research to improve overall survival rates.
  
  
  RESULTS
  Cholangiocarcinoma has seen significant increase in incidence rates over the last several decades. Most patients do not carry the documented risk factors, which include infections and inflammatory conditions, but cholangiocarcinoma typically forms in the setting of cholestasis and chronic inflammation. Management strategies include multispecialty treatments, with consideration of surgical resection, systemic chemotherapy, and targeted radiation therapy. Surgically resectable disease is the only curable treatment option, which may involve liver transplantation in certain selected cases. Referrals to centers of excellence, along with enrollment in novel clinical trials are recommended for patients with unresectable or recurrent disease. This article provides an overview of cholangiocarcinoma and discusses the current diagnosis and treatment options. While incidence is increasing and more risk factors are being discovered, much more work remains to improve outcomes of this ominous disease.
authors.affiliation: Grand Strand Medical Center, Myrtle Beach, SC, 29572, USA.
authors.forename: Bryan
authors.initials: B
authors.lastname: Doherty
authors.affiliation: Grand Strand Medical Center, Myrtle Beach, SC, 29572, USA.
authors.forename: Vinod E
authors.initials: VE
authors.lastname: Nambudiri
authors.affiliation: Division of Gastroenterology and Hepatology, Mayo Clinic, 4500 San Pablo Road, Jacksonville, FL, 32224, USA. Palmer.william@mayo.edu.
authors.forename: William C
authors.initials: WC
authors.lastname: Palmer
mesh_terms: D001650:Bile Duct Neoplasms / Q000175:diagnosis* / Q000453:epidemiology / Q000209:etiology / Q000628:therapy; D014408:Biomarkers, Tumor / Q000097:blood; D018281:Cholangiocarcinoma / Q000175:diagnosis* / Q000453:epidemiology / Q000209:etiology / Q000628:therapy; D003131:Combined Modality Therapy; D019468:Disease Management; D006801:Humans; D011379:Prognosis; D012307:Risk Factors
chemical_list: D014408:Biomarkers, Tumor
keywords: Biliary adenocarcinoma; Biliary tract cancer; Cholangiocarcinoma; Diagnosis and treatment; Gall bladder; Rare hepatic tumor