Sporadic Pancreatic Endocrine Tumors

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Abstract

Pancreatic endocrine neoplasias (pNENs) are uncommon but fascinating tumors with an annual incidence of 1 per 100,000 people. pNENs present as either functional tumors, causing specific hormonal syndromes like Zollinger-Ellison syndrome (ZES) or organic hyperinsulinism, or as pancreatic endocrine nonfunctional tumors (NF-pNENs). The natural history of pNENs is highly variable. Ninety percent of all insulinomas or small NF-pNENs are readily curable by surgical resection. Most other functional and late detected NF-pNENs have a less favorable chance for cure. Patients with completely resected tumors generally have a good prognosis, and an aggressive surgical approach combined with conservative treatment options in patients with advanced disease often results in long-term survival.

Keywords

Neuroendocrine tumors of the pancreas Insulinoma Gastrinoma Pancreatic endocrine nonfunctional tumors Surgical therapy 
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Copyright information

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

Authors and Affiliations

  1. 1.Department of SurgeryUniversity Hospital Marburg and GiessenMarburgGermany
  2. 2.Klinik für Visceral- Thorax- und GefäßchirurgieUniversitätsklinikum Gießen und Marburg, BaldingerstraßeMarburgGermany

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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