Pathology and Surgical Treatment of High-Grade Pancreatic Neuroendocrine Carcinoma: an Evolving Landscape

  • Sven-Petter HaugvikEmail author
  • Daniel Kaemmerer
  • Sebastien Gaujoux
  • Knut Jørgen Labori
  • Caroline Sophie Verbeke
  • Ivar Prydz Gladhaug
Gastrointestinal Cancers (J Meyer, Section Editor)
Part of the following topical collections:
  1. Topical Collection on Gastrointestinal Cancers


Pancreatic neuroendocrine neoplasms (PNENs) are rare, accounting for less than 5 % of all pancreatic tumors. High-grade pancreatic neuroendocrine carcinomas (hgPNECs) represent about 5 % of all PNENs. They show highly aggressive behavior with dismal prognosis. Throughout the last two decades, there has been a notable progress in basic and clinical research of PNENs and a therapeutic trend towards both more aggressive and minimally invasive surgery. Despite these advances, hgPNECs as a distinct clinical entity remains largely unexplored among surgeons. This review of current development in pathology reporting and surgical treatment of hgPNECs aims at increasing the awareness of an evolving field in pancreatic surgery.


Neuroendocrine tumor Neuroendocrine neoplasm Gastroenteropancreatic neuroendocrine tumor Neuroendocrine carcinoma Pancreas Pancreatic neoplasm Pancreatic surgery Pancreatic cancer Review 


Compliance with Ethical Standards

Conflict of Interest

Sven-Petter Haugvik, Daniel Kaemmerer, Sebastien Gaujoux, Knut Jørgen Labori, Caroline Sophie Verbeke, and Ivar Prydz Gladhaug declare that they have no conflict of interest.

Human and Animal Rights and Informed Consent

This article does not contain any studies with human or animal subjects performed by any of the authors.


Papers of particular interest, published recently, have been highlighted as: •• Of major importance

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Copyright information

© Springer Science+Business Media New York 2016

Authors and Affiliations

  • Sven-Petter Haugvik
    • 1
    • 2
    • 3
    Email author
  • Daniel Kaemmerer
    • 4
  • Sebastien Gaujoux
    • 5
  • Knut Jørgen Labori
    • 1
  • Caroline Sophie Verbeke
    • 2
    • 6
  • Ivar Prydz Gladhaug
    • 1
    • 2
  1. 1.Department of Hepato-Pancreato-Biliary SurgeryOslo University HospitalNydalenNorway
  2. 2.Institute of Clinical MedicineUniversity of OsloBlindernNorway
  3. 3.Department of Surgery, Drammen HospitalVestre Viken Hospital TrustDrammenNorway
  4. 4.Department of General and Visceral SurgeryZentralklinik Bad BerkaBad BerkaGermany
  5. 5.Department of Digestive and Endocrine Surgery, Cochin Hospital, APHPUniversité Paris DescartesParisFrance
  6. 6.Department of PathologyOslo University HospitalNydalenNorway

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