What is the management for neuroendocrine tumors (NETs)?

Medical Advisory BoardAll articles are reviewed for accuracy by our Medical Advisory Board
Article Review Status
Submitted
Under Review
Approved

Last updated: March 5, 2025 • View editorial policy

From the Guidelines

Management of neuroendocrine tumors (NETs) requires a multidisciplinary approach tailored to tumor location, grade, stage, and functional status, with the primary goal of improving morbidity, mortality, and quality of life. The most recent and highest quality study, published in 2020 1, provides guidance on the diagnosis, treatment, and follow-up of gastroenteropancreatic neuroendocrine neoplasms.

Key Considerations

  • Initial management involves surgical resection when possible, as complete removal offers the best chance for cure.
  • For localized disease, surgery remains the primary treatment, while metastatic disease may benefit from debulking surgery to reduce hormone production and tumor burden.
  • Medical therapy includes somatostatin analogs like octreotide LAR (20-30 mg IM every 4 weeks) or lanreotide (120 mg deep SC every 4 weeks), which control symptoms in functional tumors and may slow disease progression in well-differentiated NETs.
  • For progressive disease, targeted therapies such as everolimus (10 mg daily) or sunitinib (37.5 mg daily) can be effective, as shown in studies published in 2013 2 and 2015 3.
  • Peptide receptor radionuclide therapy (PRRT) with lutetium-177 dotatate is valuable for somatostatin receptor-positive tumors that have progressed on other therapies.
  • Chemotherapy regimens (temozolomide/capecitabine or cisplatin/etoposide) are typically reserved for higher-grade or rapidly progressing tumors.
  • Symptom management is crucial, particularly for functional tumors causing carcinoid syndrome, where telotristat ethyl (250 mg TID) may help control diarrhea resistant to somatostatin analogs.
  • Regular monitoring with imaging and biomarkers (chromogranin A, 5-HIAA) is essential to assess treatment response and disease progression.

Treatment Options

  • Surgical resection: complete removal offers the best chance for cure.
  • Debulking surgery: reduces hormone production and tumor burden in metastatic disease.
  • Somatostatin analogs: control symptoms in functional tumors and may slow disease progression.
  • Targeted therapies: everolimus and sunitinib are effective for progressive disease.
  • PRRT: valuable for somatostatin receptor-positive tumors that have progressed on other therapies.
  • Chemotherapy: reserved for higher-grade or rapidly progressing tumors.
  • Symptom management: crucial for functional tumors causing carcinoid syndrome.

From the FDA Drug Label

1. 2 Gastroenteropancreatic Neuroendocrine Tumors

Lanreotide Injection is indicated for the treatment of adult patients with unresectable, well or moderately differentiated, locally advanced or metastatic gastroenteropancreatic neuroendocrine tumors (GEP-NETs) to improve progression-free survival.

2.1 Recommended Dosage Gastroenteropancreatic Neuroendocrine Tumors (GEP-NETs) The recommended dosage of Lanreotide Injection is 120 mg administered every 4 weeks by deep subcutaneous injection.

The management for neuroendocrine tumors (NETs), specifically gastroenteropancreatic neuroendocrine tumors (GEP-NETs), is Lanreotide Injection 120 mg administered every 4 weeks by deep subcutaneous injection, with the goal of improving progression-free survival 4.

From the Research

Management of Neuroendocrine Tumors (NETs)

The management of NETs involves a multidisciplinary approach, with the primary goal of achieving a cure, and secondary goals of symptom control and limitation of tumor progression 5, 6, 7, 8, 9.

  • Surgical Management: Surgery is the only possible curative approach and represents the traditional first-line therapy 5, 8. However, most patients with NETs are diagnosed once metastases have occurred, making curative surgery generally not possible 5, 8.
  • Medical Management: Patients require chronic postoperative medical management to relieve symptoms and suppress tumor growth and spread 5, 6, 7, 9.
    • Somatostatin Analogues: Somatostatin analogues, such as octreotide long-acting repeatable (LAR), can improve symptoms of carcinoid syndrome and stabilize tumor growth in many patients 5, 6, 7, 9.
    • Antiproliferative Treatment: Octreotide LAR has been shown to be an effective antiproliferative treatment in patients with newly diagnosed, functionally active or inactive, well-differentiated metastatic midgut NETs 5, 9.
    • Combination Therapy: Combination therapy with octreotide LAR and everolimus has shown synergistic antiproliferative activity 5, 6, 9.
    • Peptide Receptor Radionuclide Therapy: Peptide receptor radiotherapy with 90yttrium-DOTATOC or 177lutetium-DOTATE is a new interesting treatment option for NETs 5, 7, 9.
  • Targeted Therapies: Targeted therapies, such as sunitinib, a tyrosine kinase inhibitor, are active in pancreatic NETs 5, 7.
  • Future Therapies: Pasireotide, a multi-receptor targeted somatostatin analogue, has the potential to be an effective therapy for de novo or octreotide-refractory carcinoid syndrome and for inhibiting tumor cell proliferation 5, 9.

References

Guideline

neuroendocrine tumors, version 1.2015.

Journal of the National Comprehensive Cancer Network : JNCCN, 2015

Research

Management of neuroendocrine tumors: current and future therapies.

Expert review of endocrinology & metabolism, 2011

Research

Surgical Management of Pancreatic Neuroendocrine Tumors.

Surgical oncology clinics of North America, 2016

Research

The management of neuroendocrine tumours: current and future medical therapy options.

Clinical oncology (Royal College of Radiologists (Great Britain)), 2012

Professional Medical Disclaimer

This information is intended for healthcare professionals. Any medical decision-making should rely on clinical judgment and independently verified information. The content provided herein does not replace professional discretion and should be considered supplementary to established clinical guidelines. Healthcare providers should verify all information against primary literature and current practice standards before application in patient care. Dr.Oracle assumes no liability for clinical decisions based on this content.