Inherited Pancreatic Endocrine Tumors

  • Jerena Manoharan
  • Jens Waldmann
  • Peter Langer
  • Detlef K. Bartsch
Reference work entry


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.


Pancreatic neuroendocrine neoplasia Multiple endocrine neoplasia type 1 Neurofibromatosis type 1 Von Hippel-Lindau syndrome Screening Practice guidelines 


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Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2018

Authors and Affiliations

  • Jerena Manoharan
    • 1
  • Jens Waldmann
    • 1
  • Peter Langer
    • 2
  • Detlef K. Bartsch
    • 1
  1. 1.Klinik für Visceral- Thorax- und Gefäßchirurgie, Universitätsklinikum Gießen und Marburg, BaldingerstraßeMarburgGermany
  2. 2.Klinikum Hanau Klinik für Allgemein-, Visceral- und ThoraxchirurgieHanauGermany

Section editors and affiliations

  • Raul A. Urrutia
    • 1
  • Markus W. Büchler
    • 2
  • John Neoptolemos
    • 3
  1. 1.Mayo Clinic Cancer CenterMayo ClinicRochesterUSA
  2. 2.Department of General, Visceral and Transplantation SurgeryUniversity of HeidelbergHeidelbergGermany
  3. 3.Division of Surgery and OncologyUniversity of LiverpoolLiverpoolUK

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