TY - CHAP M1 - Book, Section TI - Biliary Tract Cancer A1 - Makawita, Shalini A1 - Lee, Sunyoung A1 - Chun, Yun Shin A1 - Roach, Millicent A. A1 - Koay, Eugene J. A1 - Javle, Milind A2 - Kantarjian, Hagop M. A2 - Wolff, Robert A. A2 - Rieber, Alyssa G. Y1 - 2022 N1 - T2 - The MD Anderson Manual of Medical Oncology, 4e AB - KEY CONCEPTSBiliary tract cancers are rare tumors (prevalence <6/100,000 persons/year) with significant geographic variability.Risk factors include cholelithiasis, polyps, porcelain gallbladder, chronic inflammatory states such as inflammatory bowel disease, primary sclerosing cholangitis and nonalcoholic steatohepatitis, genetic syndromes (Peutz-Jeghers or Gardner syndrome), and chronic infection (Salmonella typhi carriers, Opisthorchis viverrine, and Clonorchis sinensis, particularly in Southeast Asia, hepatitis B and C).Five-year overall survival rates post R0 resection range from 40% to 50%. High-risk features include positive lymph nodes/margins, vascular invasion, and multifocal disease.In localized disease, a multimodality approach is often used:In early-stage intrahepatic cholangiocarcinoma, surgical resection with nodal dissection is followed by adjuvant capecitabine or gemcitabine-cisplatin; radiation therapy is given postoperatively for positive margins.Neoadjuvant chemotherapy is considered for high-risk features.In clinical practice, combination chemotherapy (gemcitabine-cisplatin or gemcitabine-capecitabine) is used adjuvantly, particularly in node-positive disease or R1 resection.Systemic therapy in metastatic disease is guided largely by molecular profiling and actionable targets. FGFR, IDH1, HER2, and BRAF alterations are among the most enriched, and numerous clinical trial options are available for these patients.Cholangiocarcinoma has low rates of high microsatellite instability and programmed cell death ligand 1 positivity. It remains overall a “cold tumor,” though methods to improve immune cell infiltration and predict responders to immunotherapy are underway. SN - PB - McGraw Hill Education CY - New York, NY Y2 - 2024/03/29 UR - accessmedicine.mhmedical.com/content.aspx?aid=1190835403 ER -