Non-functional neuroendocrine tumors of the pancreas: Advances in diagnosis and management

World J Gastroenterol. 2015 Aug 28;21(32):9512-25. doi: 10.3748/wjg.v21.i32.9512.


Nonfunctional neuroendocrine tumors of the pancreas (NF-PNETs) are a heterogeneous group of neoplasms. Although rare, the incidence of NF-PNETs is increasing significantly. The classification of PNETs has evolved over the past decades and is now based on a proliferation grading system. While most NF-PNETs are slow growing, tumors with more aggressive biology may become incurable once they progress to unresectable metastatic disease. Tumors of higher grade can be suspected preoperatively based on the presence of calcifications, hypoenhancement on arterial phase computed tomography, positron emission technology avidity and lack of octreotide scan uptake. Surgery is the only curative treatment and is recommended for most patients for whom complete resection is possible. Liver-directed therapies (thermal ablation, transarterial embolization) can be useful in controlling unresectable hepatic metastatic disease. In the presence of unresectable progressive disease, somatostatin analogues, everolimus and sunitinib can prolong progression-free survival. This article provides a comprehensive review of NF-PNETs with special emphasis on recent advances in diagnosis and management.

Keywords: Islet cell; Neuroendocrine carcinoma; Neuroendocrine tumor; Octreotide; Pancreas.

Publication types

  • Review

MeSH terms

  • Antineoplastic Agents / adverse effects
  • Antineoplastic Agents / therapeutic use*
  • Diagnostic Imaging* / methods
  • Disease Progression
  • Disease-Free Survival
  • Humans
  • Liver Neoplasms / secondary
  • Liver Neoplasms / therapy
  • Neoplasm Grading
  • Neoplasm Staging
  • Neuroendocrine Tumors / diagnosis*
  • Neuroendocrine Tumors / mortality
  • Neuroendocrine Tumors / secondary
  • Neuroendocrine Tumors / therapy*
  • Pancreatectomy* / adverse effects
  • Pancreatectomy* / mortality
  • Pancreatic Neoplasms / diagnosis*
  • Pancreatic Neoplasms / mortality
  • Pancreatic Neoplasms / pathology
  • Pancreatic Neoplasms / therapy*
  • Positron-Emission Tomography
  • Predictive Value of Tests
  • Risk Factors
  • Tomography, X-Ray Computed
  • Treatment Outcome


  • Antineoplastic Agents