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Molecular biology of neuroendocrine tumors: from pathways to biomarkers and targets

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Abstract

Neuroendocrine tumors (NETs) represent a heterogeneous group of diseases with varied natural history and prognosis depending upon the organ of origin and grade of aggressiveness. The most widely used biomarker to determine disease burden and monitor response to treatment is chromogranin A (CgA), but it is far from being the optimal predictive and prognostic biomarker in NETs. Biological understanding and derived treatment options for NETs have changed markedly in recent years. Over the last decade, the genomic landscape of these tumors has been extensively investigated. This has resulted in the discovery of mutations and expression anomalies in genes and pathways such as the PI3K/Akt/mTOR, DAXX/ATRX, and MEN1, which are promising predictive and prognostic biomarkers and future candidates for targeted therapies. Additionally, the study of tumor stroma and environment are one of the most promising fields for discovery of potential new targets and biomarkers.

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Authors and Affiliations

  1. Department of Medical Oncology, Vall d’Hebron University Hospital, Universitat Autònoma de Barcelona, Center affiliated to the Red Temática de Investigación Cooperativa en Cancer (RTICC), Instituto Carlos III, Spanish Ministry of Science and Innovation, Barcelona, Spain

    J. Capdevila

  2. Department of Pathology and Oncology, Johns Hopkins University School of Medicine, Baltimore, MD, USA

    A. Meeker

  3. Department of Medical Oncology, Hospital Universitario Virgen del Rocío, Instituto de Biomedicina de Sevilla (IBIS) [Universidad de Sevilla, CSIC, HUVR], Center affiliated to the Red Temática de Investigación Cooperativa en Cancer (RTICC), Instituto Carlos III, Spanish Ministry of Science and Innovation, Sevilla, Spain

    R. García-Carbonero

  4. Department of Laboratory Medicine, Center for Molecular Pathology, Lund University, Lund, Sweden

    K. Pietras

  5. Department of Pathology, Hospital Universitario Central de Asturias, Asturias, Spain

    A. Astudillo

  6. Laboratori de Recerca Traslacional, InstitutCatalàd’Oncología-IDIBELL, L’Hospitalet, Center affiliated to the Red Temática de Investigación Cooperativa en Cancer (RTICC), Instituto Carlos III, Spanish Ministry of Science and Innovation, Barcelona, Spain

    O. Casanovas

  7. ARC-NET Research Centre and Department of Pathology and Diagnostics, University of Verona, Verona, Italy

    A. Scarpa

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  1. J. Capdevila
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  2. A. Meeker
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  3. R. García-Carbonero
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  4. K. Pietras
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  6. O. Casanovas
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  7. A. Scarpa
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Correspondence to J. Capdevila.

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Capdevila, J., Meeker, A., García-Carbonero, R. et al. Molecular biology of neuroendocrine tumors: from pathways to biomarkers and targets. Cancer Metastasis Rev 33, 345–351 (2014). https://doi.org/10.1007/s10555-013-9468-y

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