Multiple endocrine neoplasia type 1 medical therapy

Jump to navigation Jump to search

Multiple endocrine neoplasia type 1 Microchapters


Patient Information


Historical Perspective




Differentiating Multiple endocrine neoplasia type 1 from other Diseases

Epidemiology and Demographics

Risk Factors


Natural History, Complications and Prognosis


History and Symptoms

Physical Examination

Laboratory Findings


X Ray




Other Imaging Findings

Other Diagnostic Studies


Medical Therapy


Primary Prevention

Secondary Prevention

Cost-Effectiveness of Therapy

Future or Investigational Therapies

Case Studies

Case #1

Multiple endocrine neoplasia type 1 medical therapy On the Web

Most recent articles

Most cited articles

Review articles

CME Programs

Powerpoint slides


American Roentgen Ray Society Images of Multiple endocrine neoplasia type 1 medical therapy

All Images
Echo & Ultrasound
CT Images

Ongoing Trials at Clinical

US National Guidelines Clearinghouse

NICE Guidance

FDA on Multiple endocrine neoplasia type 1 medical therapy

CDC on Multiple endocrine neoplasia type 1 medical therapy

Multiple endocrine neoplasia type 1 medical therapy in the news

Blogs on Multiple endocrine neoplasia type 1 medical therapy

Directions to Hospitals Treating Multiple endocrine neoplasia type 1

Risk calculators and risk factors for Multiple endocrine neoplasia type 1 medical therapy

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [2]; Associate Editor(s)-in-Chief: Ammu Susheela, M.D. [3]


Pharmacologic medical therapies for multiple endocrine neoplasia type 1 include management of individual components of MEN 1.

Medical Therapy

Pharmacologic medical therapies for multiple endocrine neoplasia type 1 include management of individual components of MEN 1 .

Pituitary adenoma

Treatment options depends on the type of tumor and on its size:[1][2][3][4][5][6]

  • Prolactinomas are most often treated with dopamine agonists such as bromocriptine and cabergoline. The latter, decreases tumor size as well as alleviates symptoms. Dopamine agonists are followed by serial imaging to detect the recurrence. If the adenoma is large, treatment may include radiation therapy and surgery. Efforts have been made to use a progesterone antagonist for the treatment of prolactinomas, but so far have not proved successful.
  • Thyrotrophic adenomas respond to octreotide, a long-acting somatostatin analog, in many but not all cases according to a review of the medical literature. Unlike prolactinomas, thyrotrophic adenomas characteristically respond poorly to dopamine agonist treatment.
  • Somatotrophic adenomas can be treated with somatostatin analogues, dopamine analogues, and the newer GH-receptor antagonists, such as pegvisomant.
  • Adrenocorticotropic adenomas can be treated with ketoconazole, an inhibitor of steroidogenesis, it's considered as a drug of choice in adjunctive medical therapy for ACTH-producing adenomas.
  • Recurrent macroadenoma can be treated with octreotide, a long-acting somatostatin analogue. This can result in both reduction of the size of the tumour and reduction in the serum levels of growth hormone.[7]
  • Clomifene is contraindicated in patient with pituitary adenoma.


Medical therapy for hyperparathyroidism should be considered in the following circumstances:[8]

  • Patients with hyperparathyroidism not meeting the guidelines for surgery.
  • Patients with hyperparathyroidism having contraindications to surgery.
  • Patient with hyperparathyroidism who have previous unsuccessful neck exploration.
  • Patient with hyperparathyroidism who have not been cured by surgery.
  • Patient with hyperparathyroidism refuses surgery.

Medical Management

Zollinger-Ellison Syndrome

  • Pharmacotherapy of Zollinger-Ellison syndrome (ZES) includes aspects of management such as medical control of acid hypersecretion, diagnosis, localization and treatment directed at the gastrinoma.
  • Widespread use of PPIs for many GI complaints is making the diagnosis of ZES more difficult and is delaying the diagnosis.
  • Certain aspects of ZES require modifications of standard antisecretory treatment and are discussed (pregnancy, parenteral therapy, complicated disease)
  • Patients with advanced disease require treatments directed against the gastrinoma, a number of which are recently shown effective or promising including new chemotherapy regimens, molecular targeted therapies, biotherapies, and peptide-radioreceptor therapy.
  • As the widespread use of pharmacotherapy has become more prevalent, the management of ZES has transformed from a surgical therapy to medical therapy which has been observed to play a major role. [23]
  • Medical management is to treat symptoms and prevent complications from peptic ulcer disease. The preferred medical therapy is the use of high doses of proton pump inhibitors. PPI’s are preferred over H2 receptor blockers due to their higher potency and longer duration of action. [24]

Pharmacotherapy for Zollinger-Ellison syndrome may includes the following: [23] [25] [26]

Hormonal therapy

  • In patients who are not suitable for surgery or in patients with widespread metastasis, conservative management with PPIs is also recommended. [24]
  • In patients with metastatic disease a limited efficacy has been observed with current treatment modalities. Chemotherapy may be advised for patients with widespread metastasis. The first-line treatment suggested is combined therapy with streptozotocin and 5-fluorouracil or doxorubicin. However, these treatments have been shown to result in limited responses and considerable toxicity. [24]


  1. Moyes VJ, Monson JP, Chew SL, Akker SA (2010). "Clinical Use of Cinacalcet in MEN1 Hyperparathyroidism". Int J Endocrinol. 2010: 906163. doi:10.1155/2010/906163. PMC 2877200. PMID 20585352.
  2. [1] Multiple Endocrine Neoplasia Type 1
  3. Sheppard BC, Norton JA, Doppman JL, Maton PN, Gardner JD, Jensen RT (1989). "Management of islet cell tumors in patients with multiple endocrine neoplasia: a prospective study". Surgery. 106 (6): 1108–17, discussion 1117–8. PMID 2573957.
  4. Metz DC, Strader DB, Orbuch M, Koviack PD, Feigenbaum KM, Jensen RT (1993). "Use of omeprazole in Zollinger-Ellison syndrome: a prospective nine-year study of efficacy and safety". Aliment. Pharmacol. Ther. 7 (6): 597–610. PMID 8161665.
  5. Sadowski SM, Triponez F (2015). "Management of pancreatic neuroendocrine tumors in patients with MEN 1". Gland Surg. 4 (1): 63–8. doi:10.3978/j.issn.2227-684X.2014.12.01. PMC 4321051. PMID 25713781.
  6. Demeure MJ, Klonoff DC, Karam JH, Duh QY, Clark OH (1991). "Insulinomas associated with multiple endocrine neoplasia type I: the need for a different surgical approach". Surgery. 110 (6): 998–1004, discussion 1004–5. PMID 1684067.
  7. Dr Amir Rezaee and Dr Yuranga Weerakkody 2015. URL accessed on 9 30 2015
  8. Khan AA (2013). "Medical management of primary hyperparathyroidism". J Clin Densitom. 16 (1): 60–3. doi:10.1016/j.jocd.2012.11.010. PMID 23374743.
  9. Marcocci C, Bollerslev J, Khan AA, Shoback DM (2014). "Medical management of primary hyperparathyroidism: proceedings of the fourth International Workshop on the Management of Asymptomatic Primary Hyperparathyroidism". J Clin Endocrinol Metab. 99 (10): 3607–18. doi:10.1210/jc.2014-1417. PMID 25162668.
  10. Jorde R, Szumlas K, Haug E, Sundsfjord J (2002). "The effects of calcium supplementation to patients with primary hyperparathyroidism and a low calcium intake". Eur J Nutr. 41 (6): 258–63. doi:10.1007/s00394-002-0383-1. PMID 12474069.
  11. Ross AC, Manson JE, Abrams SA, Aloia JF, Brannon PM, Clinton SK; et al. (2011). "The 2011 report on dietary reference intakes for calcium and vitamin D from the Institute of Medicine: what clinicians need to know". J Clin Endocrinol Metab. 96 (1): 53–8. doi:10.1210/jc.2010-2704. PMC 3046611. PMID 21118827.
  12. Chow CC, Chan WB, Li JK, Chan NN, Chan MH, Ko GT; et al. (2003). "Oral alendronate increases bone mineral density in postmenopausal women with primary hyperparathyroidism". J Clin Endocrinol Metab. 88 (2): 581–7. doi:10.1210/jc.2002-020890. PMID 12574184.
  13. Khan AA, Bilezikian JP, Kung AW, Ahmed MM, Dubois SJ, Ho AY; et al. (2004). "Alendronate in primary hyperparathyroidism: a double-blind, randomized, placebo-controlled trial". J Clin Endocrinol Metab. 89 (7): 3319–25. doi:10.1210/jc.2003-030908. PMID 15240609.
  14. Peacock M, Bilezikian JP, Klassen PS, Guo MD, Turner SA, Shoback D (2005). "Cinacalcet hydrochloride maintains long-term normocalcemia in patients with primary hyperparathyroidism". J Clin Endocrinol Metab. 90 (1): 135–41. doi:10.1210/jc.2004-0842. PMID 15522938.
  15. Luque-Fernández I, García-Martín A, Luque-Pazos A (2013). "Experience with cinacalcet in primary hyperparathyroidism: results after 1 year of treatment". Ther Adv Endocrinol Metab. 4 (3): 77–81. doi:10.1177/2042018813482344. PMC 3666442. PMID 23730501.
  16. Faggiano A, Di Somma C, Ramundo V, Severino R, Vuolo L, Coppola A; et al. (2011). "Cinacalcet hydrochloride in combination with alendronate normalizes hypercalcemia and improves bone mineral density in patients with primary hyperparathyroidism". Endocrine. 39 (3): 283–7. doi:10.1007/s12020-011-9459-0. PMID 21445714.
  17. Wetmore JB, Quarles LD (2009). "Calcimimetics or vitamin D analogs for suppressing parathyroid hormone in end-stage renal disease: time for a paradigm shift?". Nat Clin Pract Nephrol. 5 (1): 24–33. doi:10.1038/ncpneph0977. PMC 3924719. PMID 18957950.
  18. Strippoli GF, Palmer S, Tong A, Elder G, Messa P, Craig JC (2006). "Meta-analysis of biochemical and patient-level effects of calcimimetic therapy". Am J Kidney Dis. 47 (5): 715–26. doi:10.1053/j.ajkd.2006.01.015. PMID 16632010.
  19. Moe SM, Chertow GM, Coburn JW, Quarles LD, Goodman WG, Block GA; et al. (2005). "Achieving NKF-K/DOQI bone metabolism and disease treatment goals with cinacalcet HCl". Kidney Int. 67 (2): 760–71. doi:10.1111/j.1523-1755.2005.67139.x. PMID 15673327.
  20. Block GA, Zeig S, Sugihara J, Chertow GM, Chi EM, Turner SA; et al. (2008). "Combined therapy with cinacalcet and low doses of vitamin D sterols in patients with moderate to severe secondary hyperparathyroidism". Nephrol Dial Transplant. 23 (7): 2311–8. doi:10.1093/ndt/gfn026. PMID 18310602.
  21. Chertow GM, Blumenthal S, Turner S, Roppolo M, Stern L, Chi EM; et al. (2006). "Cinacalcet hydrochloride (Sensipar) in hemodialysis patients on active vitamin D derivatives with controlled PTH and elevated calcium x phosphate". Clin J Am Soc Nephrol. 1 (2): 305–12. doi:10.2215/CJN.00870805. PMID 17699221.
  22. Dulfer RR, Franssen GJH, Hesselink DA, Hoorn EJ, van Eijck CHJ, van Ginhoven TM (2017). "Systematic review of surgical and medical treatment for tertiary hyperparathyroidism". Br J Surg. 104 (7): 804–813. doi:10.1002/bjs.10554. PMID 28518414.
  23. 23.0 23.1 Ito T, Igarashi H, Uehara H, Jensen RT (2013). "Pharmacotherapy of Zollinger-Ellison syndrome". Expert Opin Pharmacother. 14 (3): 307–21. doi:10.1517/14656566.2013.767332. PMC 3580316. PMID 23363383.
  24. 24.0 24.1 24.2 Cingam S, Karanchi H. PMID 28722872. Missing or empty |title= (help)
  25. Metz DC, Comer GM, Soffer E, Forsmark CE, Cryer B, Chey W; et al. (2006). "Three-year oral pantoprazole administration is effective for patients with Zollinger-Ellison syndrome and other hypersecretory conditions". Aliment Pharmacol Ther. 23 (3): 437–44. doi:10.1111/j.1365-2036.2006.02762.x. PMID 16423003.
  26. Hirschowitz BI, Simmons J, Mohnen J (2005). "Clinical outcome using lansoprazole in acid hypersecretors with and without Zollinger-Ellison syndrome: a 13-year prospective study". Clin Gastroenterol Hepatol. 3 (1): 39–48. PMID 15645403.
  27. Saijo F, Naito H, Funayama Y, Fukushima K, Shibata C, Hashimoto A; et al. (2003). "Octreotide in control of multiple liver metastases from gastrinoma". J Gastroenterol. 38 (9): 905–8. doi:10.1007/s00535-002-1170-8. PMID 14564638.
  28. Burgess JR, Greenaway TM, Parameswaran V, Shepherd JJ (1999). "Octreotide improves biochemical, radiologic, and symptomatic indices of gastroenteropancreatic neoplasia in patients with multiple endocrine neoplasia type 1 (MEN-1). Implications for an integrated model of MEN-1 tumorigenesis". Cancer. 86 (10): 2154–9. PMID 10570446.
  29. Shojamanesh H, Gibril F, Louie A, Ojeaburu JV, Bashir S, Abou-Saif A; et al. (2002). "Prospective study of the antitumor efficacy of long-term octreotide treatment in patients with progressive metastatic gastrinoma". Cancer. 94 (2): 331–43. doi:10.1002/cncr.10195. PMID 11900219.

Template:WS Template:WH