Journal of Gastroenterology

, Volume 47, Issue 9, pp 941–960 | Cite as

Therapy of metastatic pancreatic neuroendocrine tumors (pNETs): recent insights and advances

  • Tetsuhide Ito
  • Hisato Igarashi
  • Robert T. Jensen


Neuroendocrine tumors (NETs) [carcinoids, pancreatic neuroendocrine tumors (pNETs)] are becoming an increasing clinical problem because not only are they increasing in frequency, but they can frequently present with advanced disease that requires diagnostic and treatment approaches different from those used in the neoplasms that most physicians are used to seeing and treating. In the past few years there have been numerous advances in all aspects of NETs including: an understanding of their unique pathogenesis; specific classification systems developed which have prognostic value; novel methods of tumor localization developed; and novel treatment approaches described. In patients with advanced metastatic disease these include the use of newer chemotherapeutic approaches, an increased understanding of the role of surgery and cytoreductive methods, the development of methods for targeted delivery of cytotoxic agents, and the development of targeted medical therapies (everolimus, sunitinib) based on an increased understanding of the disease biology. Although pNETs and gastrointestinal NETs share many features, recent studies show they differ in pathogenesis and in many aspects of diagnosis and treatment, including their responsiveness to different therapies. Because of limited space, this review will be limited to the advances made in the management and treatment of patients with advanced metastatic pNETs over the past 5 years.


Neuroendocrine tumor Pancreatic endocrine tumor Liver metastases Gastrinoma Insulinoma Surgery Chemotherapy Streptozotocin Everolimus Sunitinib Somatostatin Octreotide Lanreotide Hepatic transarterial embolization Liver transplantation Peptide receptor radionuclide therapy Somatostatin receptor SIRT Chemoembolization Unresectable liver metastases 



ACTH-secreting pancreatic neuroendocrine tumors


Central nervous system


European Neuroendocrine Tumor Society


European Society for Medical Oncology




Gastrointestinal neuroendocrine tumors (carcinoids of gastrointestinal tract)


Transarterial embolization/chemoembolization


Insulin-like growth factor


International Union for Cancer Control/American Joint Cancer Committee/World Health Organization


Magnetic resonance imaging


North American Neuroendocrine Tumor Society


National Comprehensive Cancer Network


Neuroendocrine tumors (carcinoids, pancreatic neuroendocrine tumors)


Nonfunctional pancreatic neuroendocrine tumors


Pancreatic neuroendocrine tumors


Platelet-derived growth factor receptors


Peptide receptor radionuclide therapy


Radiofrequency ablation


Selective internal radiation


Somatostatin receptor scintigraphy


Vascular endothelial growth factor receptor


Vasoactive intestinal peptide-secreting pancreatic neuroendocrine tumors



This work was partially supported by intramural funds of NIDDK, NIH.

Conflict of interest

The authors declare that they have no conflict of interest.


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Copyright information

© Springer (outside the USA) 2012

Authors and Affiliations

  • Tetsuhide Ito
    • 1
  • Hisato Igarashi
    • 1
  • Robert T. Jensen
    • 2
  1. 1.Department of Medicine and Bioregulatory Science, Graduate School of Medical SciencesKyushu UniversityFukuokaJapan
  2. 2.Digestive Diseases BranchNIDDK, NIHBethesdaUSA

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