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Inherited Pancreatic Endocrine Tumors

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Abstract:

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.

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  1. Department of Surgery, University Hospital Marburg and Giessen, Marburg, Germany

    Jens Waldmann, Peter Langer & Detlef K. Bartsch

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  3. Detlef K. Bartsch
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Waldmann, J., Langer, P., Bartsch, D.K. (2010). Inherited Pancreatic Endocrine Tumors. In: Pancreatic Cancer. Springer, New York, NY. https://doi.org/10.1007/978-0-387-77498-5_25

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