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Biliary Cancer

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Practical Medical Oncology Textbook

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Abstract

Biliary tract carcinoma is a collective term for a heterogeneous group of rare gastrointestinal cancers including cholangiocarcinoma (intrahepatic, hilar and distal) and gallbladder cancer with different pathogenesis and prognosis. Surgery with complete resection is the only curative chance, but a minority of patients present with resectable disease, but relapse rates are high. Cisplatin/gemcitabine combination chemotherapy represents the first-line reference cytotoxic treatment for patients with advanced/metastatic unresectable disease, while there is no standard second-line therapy. Selected patients may benefit from liver transplantation combined with neoadjuvant chemoradiotherapy or may be suitable for liver-directed therapy (e.g. radioembolization or external beam radiation). Emerging data from next-generation sequencing analyses have identified several genomic alterations (e.g. FGFR fusion rearrangements, IDH-1 and -2 mutations, chromatin modifiers alterations) that are correlated with the disease and potentially druggable by targeted agents. These drugs are currently in early phases of development in BTCs with encouraging results, but further investigation should be warranted to confirm their potential clinical utility.

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Authors and Affiliations

  1. Medical Oncology Department, University Campus Bio-Medico, Rome, Italy

    Giuseppe Tonini, Michele Iuliani, Giulia Ribelli, Sonia Simonetti & Francesco Pantano

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  1. Giuseppe Tonini
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  2. Michele Iuliani
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Correspondence to Giuseppe Tonini .

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  1. Department of Surgical, Oncological and Oral Sciences, University of Palermo, Palermo, Italy

    Antonio Russo

  2. Oncology Department, University of Antwerp, Edegem, Belgium

    Marc Peeters

  3. Department of Biomedicine, Neuroscience and Advanced Diagnostics, University of Palermo, Palermo, Italy

    Lorena Incorvaia

  4. Center for Thoracic Oncology, Tisch Cancer Institute, Mount Sinai System & Icahn School of Medicine, Mount Sinai, NY, USA

    Christian Rolfo

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Tonini, G., Iuliani, M., Ribelli, G., Simonetti, S., Pantano, F. (2021). Biliary Cancer. In: Russo, A., Peeters, M., Incorvaia, L., Rolfo, C. (eds) Practical Medical Oncology Textbook. UNIPA Springer Series. Springer, Cham. https://doi.org/10.1007/978-3-030-56051-5_41

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