Journal
CANCERS
Volume 12, Issue 8, Pages -Publisher
MDPI
DOI: 10.3390/cancers12082308
Keywords
cholangiocarcinoma; experimental models; animal models; cell lines; tumor microenvironment; spheroids; cancer stem cells; organoids
Categories
Funding
- Italian Ministry of Health-Ricerca Corrente 2020
- FPRC 5xmille 2015 Ministero Salute Cancer Im-Gen
- Fondazione Roche-Bando per la ricerca 2019
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Cholangiocarcinoma (CCA) is a rare, aggressive disease with poor overall survival. In advanced cases, surgery is often not possible or fails; in addition, there is a lack of effective and specific therapies. Multidisciplinary approaches and advanced technologies have improved the knowledge of CCA molecular pathogenesis, highlighting its extreme heterogeneity and high frequency of genetic and molecular aberrations. Effective preclinical models, therefore, should be based on a comparable level of complexity. In the past years, there has been a consistent increase in the number of available CCA models. The exploitation of even more complex CCA models is rising. Examples are the use of CRISPR/Cas9 or stabilized organoids forin vitrostudies, as well as patient-derived xenografts or transgenic mouse models forin vivoapplications. Here, we examine the available preclinical CCA models exploited to investigate: (i) carcinogenesis processes from initiation to progression; and (ii) tools for personalized therapy and innovative therapeutic approaches, including chemotherapy and immune/targeted therapies. For each model, we describe the potential applications, highlighting both its advantages and limits.
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