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Inherited Pancreatic Endocrine Tumors

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Pancreatic Cancer

Abstract

Pancreatic neuroendocrine neoplasias (pNENs) may arise sporadically or in the setting of an inherited tumor syndrome. These syndromes comprise the multiple endocrine neoplasia type 1 (MEN1), the von Hippel-Lindau (VHL) syndrome, and the neurofibromatosis type 1 (NF-1). The prevalence and the different entities of pNENs differ significantly between these syndromes resulting in distinct treatment and screening recommendations.

Treatment of pNENs in the setting of an inherited tumor syndrome should consider the natural history of the disease, clinical symptoms, and the potential for malignant transformation which has to be considered individually for every patient.

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Authors and Affiliations

  1. Klinik für Visceral- Thorax- und Gefäßchirurgie, Universitätsklinikum Gießen und Marburg, Baldingerstraße, 35043, Marburg, Germany

    Jerena Manoharan, Jens Waldmann & Detlef K. Bartsch

  2. Klinikum Hanau Klinik für Allgemein-, Visceral- und Thoraxchirurgie, Leimenstr. 20, 63450, Hanau, Germany

    Peter Langer

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  1. Jerena Manoharan
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  2. Jens Waldmann
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  3. Peter Langer
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  4. Detlef K. Bartsch
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Corresponding author

Correspondence to Jerena Manoharan .

Editor information

Editors and Affiliations

  1. Div. Surgery & Oncology, University of Liverpool, Liverpool, United Kingdom

    John P. Neoptolemos

  2. Rochester, Minnesota, USA

    Raul Urrutia

  3. Dept. Gastrointestinal Medical Onco, Duke University Medical Center Dept. Gastrointestinal Medical Onco, Durham, North Carolina, USA

    James Abbruzzese

  4. Heidelberg, Germany

    Markus W. Büchler

Section Editor information

  1. Mayo Clinic Cancer Center, Mayo Clinic, 200 First Street SW, 55905, Rochester, MN, USA

    Raul A. Urrutia

  2. Department of General, Visceral and Transplantation Surgery, University of Heidelberg, Im Neuenheimer Feld 110, 69120, Heidelberg, Germany

    Markus W. Büchler MD

  3. Division of Surgery and Oncology, University of Liverpool, Daulby Street, Liverpool, UK

    John Neoptolemos

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Manoharan, J., Waldmann, J., Langer, P., Bartsch, D.K. (2016). Inherited Pancreatic Endocrine Tumors. In: Neoptolemos, J., Urrutia, R., Abbruzzese, J., Büchler, M. (eds) Pancreatic Cancer. Springer, New York, NY. https://doi.org/10.1007/978-1-4939-6631-8_25-2

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