Pancreatic Neuroendocrine Tumors (pNETs)

  • Alessandro Sanguinetti
  • Andrea Polistena
  • Louis Banka Johnson
  • Nicola Avenia
Chapter

Abstract

This chapter briefly summarizes the epidemiology, pathophysiology, clinical management, and outcomes of patients with pancreatic neuroendocrine tumors (PNETs) and highlights recent advances in PNET research. PNETs are rare neoplasms, compared with carcinomas arising from pancreatic exocrine tissue. They, like other neuroendocrine tumor types, display variable malignant potential, hormone-related syndromes (functionality), localization, and genetic background. Although tumor origin and molecular pathogenesis remain poorly understood, recently established grading and staging systems facilitate patient risk stratification and thereby directly impact clinical decision-making. Although the optimal clinical management of PNETs involves a multidisciplinary approach, surgery remains the only curative treatment for early-stage disease. Surgery may also have a role in patients with advanced stage disease, including those with hepatic metastases. Alternative therapeutic approaches applied to PNETs, including chemotherapy, radiofrequency ablation, transarterial chemoembolization, biotherapy, polypeptide radionuclide receptor therapy, antiangiogenic therapy, and selective internal radiotherapy, have failed to demonstrate a long-term survival benefit. Surgery remains the primary therapeutic option for patients with PNETs. Research on PNETs is desperately needed to improve the therapeutic options for patients with this disease.

Keywords

Pancreatic neuroendocrine tumors Insulinoma Gastrinoma VIPoma Glucagonoma Nonfunctioning Multiple endocrine neoplasia syndrome type 1 Mahvash disease 

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Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  • Alessandro Sanguinetti
    • 1
  • Andrea Polistena
    • 1
  • Louis Banka Johnson
    • 2
  • Nicola Avenia
    • 1
  1. 1.Unit of Endocrine Surgery, Department of Surgical SciencesSchool of Medicine University of Perugia, Azienda Ospedaliera “S. MariaTerniItaly
  2. 2.Department of Colorectal SurgeryUniversity Hospital of Malmoe-Lund, SUSLundSweden

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