Inherited Pancreatic Endocrine Tumors

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


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

Treatment of PETs 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 taken into account individually for every patient.


Liver Metastasis Pancreatic Cyst Predictive Genetic Testing Distal Pancreatic Resection Pancreatic Head Tumor 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


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Copyright information

© Springer Science+Business Media, LLC 2010

Authors and Affiliations

  • Jens Waldmann
    • 1
  • Peter Langer
    • 1
  • Detlef K. Bartsch
    • 1
  1. 1.Department of SurgeryUniversity Hospital Marburg and GiessenMarburgGermany

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