Treatment of symptomatic neuroendocrine tumor syndromes: recent advances and controversies

Expert Opin Pharmacother. 2016 Nov;17(16):2191-2205. doi: 10.1080/14656566.2016.1236916. Epub 2016 Sep 23.


Neuroendocrine tumors(NETs), once thought rare, are increasing in frequency in most countries and receiving increasing-attention. NETs present two-treatment problems. A proportion is aggressive and a proportion has a functional, hormone-excess-state(F-NET), each of which must be treated. Recently, there have been many advances, well-covered in reviews/consensus papers on imaging-NETs; new, novel anti-tumor treatments and understanding their pathogenesis. However, little attention has been paid to advances in the treatment of the hormone-excess-state. These advances are usually reported in case-series, and case-reports with few large studies. In this paper these advances are reviewed. Areas covered: Advances in the last 5-years are concentrated on, but a review of literature from the last 10-years was performed. PubMed and other databases (Cochrane, etc.) were searched for F-NET-syndromes including carcinoid-syndrome, as well as meeting-abstracts on NETs. All advances that controlled hormone-excess-states or facilitated-control were covered. These include new medical-therapies [serotonin-synthesis inhibitors(telotristat), Pasireotide, new agents for treating ACTHomas], increased dosing with conventional therapies (octreotide-LAR, Lanreotide-Autogel), mTor inhibitors(everolimus), Tyrosine-kinase inhibitors(sunitinib),cytoreductive surgery, liver-directed therapies (embolization, chemoembolization, radioembolization, RFA), peptide radio-receptor-therapy(PRRT) and 131I-MIBG, ablation of primary F-NETs. Expert opinion: Although many of the newer therapies controlling the hormone-excess-states in F-NETs are reported in relatively few patients, all the approaches show promise. Their description also generates some controversies/unresolved areas,such as the order of these new treatments, their longterm-efficacy, and effectiveness of combinations which may require large,controlled studies.

Keywords: Acid hypersecretion; Lanreotide; SIRT; Zollinger-Ellison syndrome; carcinoid syndrome; chemoembolization; chemotherapy; cytoreductive surgery; embolization; gastrinoma; insulinoma; multiple endocrine Neoplasia type 1; neuroendocrine tumor; octreotide; pancreatic endocrine tumor; peptide radio-receptor therapy; radio-frequency ablation; radioembolization; somatostatin analogues; telotristat.

Publication types

  • Review

MeSH terms

  • Animals
  • Antineoplastic Combined Chemotherapy Protocols / administration & dosage*
  • Everolimus / therapeutic use
  • Humans
  • Indoles / therapeutic use
  • Molecular Targeted Therapy / methods
  • Molecular Targeted Therapy / trends
  • Neuroendocrine Tumors / diagnosis
  • Neuroendocrine Tumors / drug therapy*
  • Octreotide / therapeutic use
  • Pyrroles / therapeutic use
  • Somatostatin / administration & dosage*
  • Somatostatin / analogs & derivatives*
  • Somatostatin / therapeutic use
  • Sunitinib
  • Syndrome
  • Treatment Outcome


  • Indoles
  • Pyrroles
  • Somatostatin
  • pasireotide
  • Everolimus
  • Octreotide
  • Sunitinib