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Molecular Pathology of Well-Differentiated Gastro-entero-pancreatic Neuroendocrine Tumors

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Abstract

Well differentiated neuroendocrine tumors (NETs) arising in the gastrointestinal and pancreaticobiliary system are the most common neuroendocrine neoplasms. Studies of the molecular basis of these lesions have identified genetic mutations that predispose to familial endocrine neoplasia syndromes and occur both as germline events and in sporadic tumors. The mutations often involve epigenetic regulators rather than the oncogenes and tumor suppressors that are affected in other malignancies. Somatic copy number alterations and miRNAs have also been implicated in the development and progression of some of these tumors. The molecular profiles differ by location, but many are shared by tumors in other sites, including those outside the gastroenteropancreatic system. The approach to therapy relies on both the neuroendocrine nature of these tumors and the identification of specific alterations that can serve as targets for precision oncologic approaches.

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Authors and Affiliations

  1. Department of Pathology, University Hospitals Cleveland Medical Center, Case Western Reserve University, Cleveland, OH, USA

    Sylvia L. Asa

  2. Institute of Pathology, University Hospital and University of Lausanne, Lausanne, Switzerland

    Stefano La Rosa

  3. Department of Pathology, Memorial Sloan Kettering Cancer Center, New York, NY, USA

    Olca Basturk

  4. Department of Pathology and Research Center for Translational Medicine (KUTTAM), Koç University Hospital, Istanbul, Turkey

    Volkan Adsay

  5. Department of Experimental Medicine, Sapienza University of Rome, Rome, Italy

    Marianna Minnetti

  6. Green Templeton College, University of Oxford and ENETS Centre of Excellence, Royal Free Hospital, London, UK

    Ashley B. Grossman

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Asa, S.L., La Rosa, S., Basturk, O. et al. Molecular Pathology of Well-Differentiated Gastro-entero-pancreatic Neuroendocrine Tumors. Endocr Pathol 32, 169–191 (2021). https://doi.org/10.1007/s12022-021-09662-5

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