Clinical Presentation and Diagnosis of Neuroendocrine Tumors

Hematol Oncol Clin North Am. 2016 Feb;30(1):21-48. doi: 10.1016/j.hoc.2015.08.006. Epub 2015 Oct 24.


Neuroendocrine tumors (NETs) are slow-growing neoplasms capable of storing and secreting different peptides and neuroamines. Some of these substances cause specific symptom complexes, whereas others are silent. They usually have episodic expression, and the diagnosis is often made at a late stage. Although considered rare, the incidence of NETs is increasing. For these reasons, a high index of suspicion is needed. In this article, the different clinical syndromes and the pathophysiology of each tumor as well as the new and emerging biochemical markers and imaging techniques that should be used to facilitate an early diagnosis, follow-up, and prognosis are reviewed.

Keywords: Biomarkers; Diagnosis; GEP-NETs; PNETs; Syndromes and their tumors.

Publication types

  • Review

MeSH terms

  • Biomarkers, Tumor / genetics
  • Biomarkers, Tumor / metabolism
  • Diagnosis, Differential
  • Diagnostic Imaging / methods*
  • Endocrine System / metabolism
  • Endocrine System / pathology*
  • Gastrointestinal Neoplasms / diagnosis*
  • Gastrointestinal Neoplasms / genetics
  • Gastrointestinal Neoplasms / metabolism
  • Genetic Predisposition to Disease / genetics
  • Hormones / metabolism
  • Humans
  • Mutation
  • Neuroendocrine Tumors / diagnosis*
  • Neuroendocrine Tumors / genetics
  • Neuroendocrine Tumors / metabolism


  • Biomarkers, Tumor
  • Hormones