Skip to main content

The Management of Neuro-Endocrine Neoplasms

  • Chapter
  • First Online:
The Spectrum of Neuroendocrine Neoplasia

Abstract

Neuroendocrine neoplasms arise in tissues of the endocrine system, including pituitary, thyroid, parathyroid, pancreas, respiratory system, gastrointestinal tract, and adrenal glands and rarely in other unusual sites. Surgery is the first line of treatment in many of these cases and can include a complete removal of the organ (i.e., total thyroidectomy or adrenalectomy), partial removal (i.e., hemithyroidectomy or distal pancreatectomy), or removal of only the tumor (i.e., pituitary tumor). Medical treatment is frequently used, and while this is usually reserved as adjuvant treatment to control symptoms or tumor growth in cases of residual disease following surgical intervention, it is sometimes used as a treatment of choice (i.e., prolactinoma), sometimes as neoadjuvant before surgery to improve outcomes (i.e., treatment to control growth hormone or cortisol excess before surgery), or as the sole primary treatment in patients who are poor candidates for surgery. Other treatment options can include radiotherapy and peptide receptor radionuclide therapy (PRRT); in some patients, additional therapies are intended for symptom control. Additional data on the molecular basis of neuroendocrine neoplasms may lead to providing treatment sequences personalized for each patient, based on the tumor biology and molecular and genetic patterns. Immunotherapy with immune checkpoint inhibitors may prove to be a successful option for selected neuroendocrine neoplasms.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

eBook
USD 16.99
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 119.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 169.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. Katznelson L, Laws ER, Melmed S, Molitch ME, Murad MH, Utz A, Wass JAH. Acromegaly: an endocrine society clinical practice guideline. J Clin Endocrinol Metab. 2014;99:3933–51.

    Article  CAS  Google Scholar 

  2. Nieman LK, Biller BMK, Findling JW, Murad MH, Newell-price J, Savage MO, Tabarin A. Treatment of cushing ’ s syndrome : an endocrine society clinical practice guideline. J Clin Endocrinol Metab. 2015;100:2807–31.

    Article  CAS  Google Scholar 

  3. Wells SA, Asa SL, Dralle H, Elisei R, Evans DB, Gagel RF, Lee N, MacHens A, Moley JF, Pacini F, et al. Revised American thyroid association guidelines for the management of medullary thyroid carcinoma. Thyroid. 2015;25:567–610.

    Article  Google Scholar 

  4. Bilezikian JP, Brandi ML, Eastell R, Silverberg SJ, Udelsman R, Marcocci C, Potts JT. Guidelines for the management of asymptomatic primary hyperparathyroidism: summary statement from the fourth international workshop. J Clin Endocrinol Metab. 2014;99:3561–9.

    Article  CAS  Google Scholar 

  5. Thakker RV, Newey PJ, Walls GV, Bilezikian J, Dralle H, Ebeling PR, Melmed S, Sakurai A, Tonelli F, Brandi ML. Clinical practice guidelines for multiple endocrine neoplasia type 1 (MEN1). J Clin Endocrinol Metab. 2012;97:2990–3011.

    Article  CAS  Google Scholar 

  6. Wilhelm SM, Wang TS, Ruan DT, Lee JA, Asa SL, Duh QY, Doherty GM, Herrera MF, Pasieka JL, Perrier ND, et al. The American association of endocrine surgeons guidelines for definitive management of primary hyperparathyroidism. JAMA Surg. 2016;151:959–68.

    Article  Google Scholar 

  7. Shah M, Goldner W, Halfdanarson T, Bergsland E, Berlin J, Halperin D, Chan J, Kulke M, Benson A, Blaszkowsky L, et al. NCCN Clinical Practice Guidelines in Oncology: Neuroendocrine and Adrenal Tumors. J Natl Compr Canc Netw. 2018;16:693–702.

    Article  CAS  Google Scholar 

  8. Garcia-Carbonero R, Sorbye H, Baudin E, Raymond E, Wiedenmann B, Niederle B, Sedlackova E, Toumpanakis C, Anlauf M, Cwikla JB, et al. ENETS consensus guidelines for high-grade gastroenteropancreatic neuroendocrine tumors and neuroendocrine carcinomas. Neuroendocrinology. 2016;103:186–94.

    Article  CAS  Google Scholar 

  9. Lenders JW, Duh Q-Y, Eisenhofer G, Gimenez-Roqueplo A-P, Grebe SKG, Murad MH, Naruse M, Pacak K, Young WF. Guidelines pheochromocytoma and paraganglioma: an endocrine society clinical practice guideline. J Clin Endocrinol Metab. 2014;99:1915–42.

    Article  CAS  Google Scholar 

  10. Krieger MD, Couldwell WT, Weiss MH. Assessment of long-term remission of acromegaly following surgery. J Neurosurg. 2003;98:719–24.

    Article  Google Scholar 

  11. Karavitaki N, Turner HE, Adams CBT, Cudlip S, Byrne JV, Fazal-Sanderson V, Rowlers S, Trainer PJ, Wass JAH. Surgical debulking of pituitary macroadenomas causing acromegaly improves control by lanreotide. Clin Endocrinol. 2008;68:970–5.

    Article  CAS  Google Scholar 

  12. Rinke A, Müller HH, Schade-Brittinger C, Klose KJ, Barth P, Wied M, Mayer C, Aminossadati B, Pape UF, Bläker M, et al. Placebo-controlled, double-blind, prospective, randomized study on the effect of octreotide LAR in the control of tumor growth in patients with metastatic neuroendocrine midgut tumors: a report from the PROMID study group. J Clin Oncol. 2009;27:4656–63.

    Article  CAS  Google Scholar 

  13. Bevan JS, Newell-Price J, Wass JAH, Atkin SL, Bouloux PM, Chapman J, Davis JRE, Howlett TA, Randeva HS, Stewart PM, et al. Home administration of lanreotide Autogel® by patients with acromegaly, or their partners, is safe and effective. Clin Endocrinol. 2008;68:343–9.

    Article  CAS  Google Scholar 

  14. Salvatori R, Woodmansee WW, Molitch M, Gordon MB, Lomax KG. Lanreotide extended-release aqueous-gel formulation, injected by patient, partner or healthcare provider in patients with acromegaly in the United States: 1-year data from the SODA registry. Pituitary. 2014;17:13–21.

    Article  CAS  Google Scholar 

  15. Freda PU, Katznelson L, Van Der Lely AJ, Reyes CM, Zhao S, Rabinowitz D. Long-acting somatostatin analog therapy of acromegaly: a meta-analysis. J Clin Endocrinol Metab. 2005;90:4465–73.

    Article  CAS  Google Scholar 

  16. Caplin ME, Pavel M, Ćwikła JB, Phan AT, Raderer M, Sedláčková E, Cadiot G, Wolin EM, Capdevila J, Wall L, et al. Lanreotide in metastatic enteropancreatic neuroendocrine tumors. N Engl J Med. 2014;371:224–33.

    Article  Google Scholar 

  17. Gadelha MR, Bronstein MD, Brue T, Coculescu M, Fleseriu M, Guitelman M, Pronin V, Raverot G, Shimon I, Lievre KK, et al. Pasireotide versus continued treatment with octreotide or lanreotide in patients with inadequately controlled acromegaly (PAOLA): a randomised, phase 3 trial. Lancet Diabetes Endocrinol. 2014;2:875–84.

    Article  CAS  Google Scholar 

  18. Gatto F, Feelders RA, Franck SE, Van Koetsveld PM, Dogan F, Kros JM, Neggers SJCMM, Van Der Lely AJ, Lamberts SWJ, Ferone D, et al. In vitro head-to-head comparison between octreotide and pasireotide in gh-secreting pituitary adenomas. J Clin Endocrinol Metab. 2017;102:2009–18.

    Article  Google Scholar 

  19. Ibáñez-Costa A, Rivero-Cortés E, Vázquez-Borrego MC, Gahete MD, Jiménez-Reina L, Venegas-Moreno E, de la Riva A, Arráez MÁ, González-Molero I, Schmid HA, et al. Octreotide and pasireotide (dis)similarly inhibit pituitary tumor cells in vitro. J Endocrinol. 2016;231:135–45.

    Article  Google Scholar 

  20. Colao A, Petersenn S, Newell-Price J, Findling JW, Gu F, Maldonado M, Schoenherr U, Mills D, Salgado LR, Biller BMK. A 12-month phase 3 study of pasireotide in cushing’s disease. N Engl J Med. 2012;366:914–24.

    Article  CAS  Google Scholar 

  21. Wolin EM, Jarzab B, Eriksson B, Walter T, Toumpanakis C, Morse MA, Tomassetti P, Weber MM, Fogelman DR, Ramage J, et al. Phase III study of pasireotide long-acting release in patients with metastatic neuroendocrine tumors and carcinoid symptoms refractory to available somatostatin analogues. Drug Des Devel Ther. 2015;9:5075–86.

    Article  CAS  Google Scholar 

  22. Yao J, Shah M, Ito T, Bohas C, Wolin EM, Van Custem E, Hobday T, Okusaka T, Capdevila J, de Vries E, et al. Everolimus for advanced pancreatic neuroendocrine tumors. N Engl J Med. 2011;364:514–23.

    Article  CAS  Google Scholar 

  23. Yao JC, Fazio N, Singh S, Buzzoni R, Carnaghi C, Wolin E, Tomasek J, Raderer M, Lahner H, Voi M, et al. Everolimus for the treatment of advanced, non-functional neuroendocrine tumours of the lung or gastrointestinal tract (RADIANT-4): a randomised, placebo-controlled, phase 3 study. Lancet. 2016;387:968–77.

    Article  CAS  Google Scholar 

  24. Raymond E, Dahan L, Raoul J-L, Bang Y-J, Borbath I, Lombard-Bohas C, Valle J, Metrakos P, Smith D, Vinik A, et al. Sunitinib malate for the treatment of pancreatic neuroendocrine tumors. N Engl J Med. 2011;364:501–13.

    Article  CAS  Google Scholar 

  25. O’Kane GM, Ezzat S, Joshua AM, Bourdeau I, Leibowitz-Amit R, Olney HJ, Krzyzanowska M, Reuther D, Chin S, Wang L, et al. A phase 2 trial of sunitinib in patients with progressive paraganglioma or pheochromocytoma: the SNIPP trial. Br J Cancer. 2019;120:1113–9.

    Article  Google Scholar 

  26. Phan AT, Halperin DM, Chan JA, Fogelman DR, Hess KR, Malinowski P, Regan E, Ng CS, Yao JC, Kulke MH. Pazopanib and depot octreotide in advanced, well-differentiated neuroendocrine tumours: a multicentre, single-group, phase 2 study. Lancet Oncol. 2015;16:695–703.

    Article  CAS  Google Scholar 

  27. Strosberg J, Wolin E, Hendifar A, Yao J, Chasen B, Mittra E, Kunz PL, Kulke MH, Jacene H, Bushnell D, et al. Phase 3 trial of 177 Lu-Dotatate for Midgut neuroendocrine tumors. N Engl J Med. 2017;376:125–35.

    Article  CAS  Google Scholar 

  28. Kulke MH, Hörsch D, Caplin ME, Anthony LB, Bergsland E, Öberg K, Welin S, Warner RRP, Lombard-Bohas C, Kunz PL, et al. Telotristat ethyl, a tryptophan hydroxylase inhibitor for the treatment of carcinoid syndrome. J Clin Oncol. 2017;35:14–23.

    Article  CAS  Google Scholar 

  29. Gubergrits N, Malecka-Panas E, Lehman GA, Vasileva G, Shen Y, Sander-Struckmeier S, Caras S, Whitcomb DC. A 6-month, open-label clinical trial of pancrelipase delayed-release capsules (Creon) in patients with exocrine pancreatic insufficiency due to chronic pancreatitis or pancreatic surgery. Aliment Pharmacol Ther. 2011;33:1152–61.

    Article  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Shereen Ezzat .

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2021 Springer Nature Switzerland AG

About this chapter

Check for updates. Verify currency and authenticity via CrossMark

Cite this chapter

Akirov, A., Amer, L., Chbat, J., Ezzat, S. (2021). The Management of Neuro-Endocrine Neoplasms. In: Asa, S.L., La Rosa, S., Mete, O. (eds) The Spectrum of Neuroendocrine Neoplasia. Springer, Cham. https://doi.org/10.1007/978-3-030-54391-4_19

Download citation

  • DOI: https://doi.org/10.1007/978-3-030-54391-4_19

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-030-54390-7

  • Online ISBN: 978-3-030-54391-4

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics