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{{Multiple endocrine neoplasia type 1}}
{{Multiple endocrine neoplasia type 1}}
{{CMG}}; {{AE}} {{Ammu}}
{{CMG}}; {{AE}} {{Ammu}}
==Overview==
==Overview==
==Management==
Pharmacologic medical therapies for multiple endocrine neoplasia type 1 include management of individual components of MEN 1.
* Because the type of pancreatic endocrine cancer associated with MEN1 can be difficult to recognize, difficult to treat, and slow to progress, there are different views about the value of surgery in managing these tumors.
 
* One approach is to "watch and wait," using medical, or nonsurgical, treatmentsAccording to this school of thought, pancreatic surgery has serious complications, so it should not be attempted unless it will cure a tumor or cure a hormone excess state.
==Medical Therapy==
* Excessive release of certain hormones-mainly gastrin-from pancreatic endocrine cancer in MEN1 needs to be treated, and medications are often effective in blocking the effects of these hormones.<ref>[http://www.niddk.nih.gov/health-information/health-topics/endocrine/multiple-endocrine-neoplasia-type-1/Pages/fact-sheet.aspx] Multiple Endocrine Neoplasia Type 1</ref>
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:<ref name="pmid20585352">{{cite journal |vauthors=Moyes VJ, Monson JP, Chew SL, Akker SA |title=Clinical Use of Cinacalcet in MEN1 Hyperparathyroidism |journal=Int J Endocrinol |volume=2010 |issue= |pages=906163 |year=2010 |pmid=20585352 |pmc=2877200 |doi=10.1155/2010/906163 |url=}}</ref><ref>[http://www.niddk.nih.gov/health-information/health-topics/endocrine/multiple-endocrine-neoplasia-type-1/Pages/fact-sheet.aspx] Multiple Endocrine Neoplasia Type 1</ref><ref name="pmid2573957">{{cite journal |vauthors=Sheppard BC, Norton JA, Doppman JL, Maton PN, Gardner JD, Jensen RT |title=Management of islet cell tumors in patients with multiple endocrine neoplasia: a prospective study |journal=Surgery |volume=106 |issue=6 |pages=1108–17; discussion 1117–8 |year=1989 |pmid=2573957 |doi= |url=}}</ref><ref name="pmid8161665">{{cite journal |vauthors=Metz DC, Strader DB, Orbuch M, Koviack PD, Feigenbaum KM, Jensen RT |title=Use of omeprazole in Zollinger-Ellison syndrome: a prospective nine-year study of efficacy and safety |journal=Aliment. Pharmacol. Ther. |volume=7 |issue=6 |pages=597–610 |year=1993 |pmid=8161665 |doi= |url=}}</ref><ref name="pmid25713781">{{cite journal |vauthors=Sadowski SM, Triponez F |title=Management of pancreatic neuroendocrine tumors in patients with MEN 1 |journal=Gland Surg |volume=4 |issue=1 |pages=63–8 |year=2015 |pmid=25713781 |pmc=4321051 |doi=10.3978/j.issn.2227-684X.2014.12.01 |url=}}</ref><ref name="pmid1684067">{{cite journal |vauthors=Demeure MJ, Klonoff DC, Karam JH, Duh QY, Clark OH |title=Insulinomas associated with multiple endocrine neoplasia type I: the need for a different surgical approach |journal=Surgery |volume=110 |issue=6 |pages=998–1004; discussion 1004–5 |year=1991 |pmid=1684067 |doi= |url=}}</ref>
*'''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 [[Somatostatin|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.<ref name="Radiopaedia">Dr Amir Rezaee and Dr Yuranga Weerakkody http://radiopaedia.org/articles/pituitary-adenoma 2015. URL accessed on 9 30 2015</ref>
*[[Clomifene]] is contraindicated in patient with pituitary adenoma.
 
=== Hyperparathyroidism ===
Medical therapy for  hyperparathyroidism should be considered in the following circumstances:<ref name="pmid23374743">{{cite journal| author=Khan AA| title=Medical management of primary hyperparathyroidism. | journal=J Clin Densitom | year= 2013 | volume= 16 | issue= 1 | pages= 60-3 | pmid=23374743 | doi=10.1016/j.jocd.2012.11.010 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23374743  }}</ref>
*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====
*1. Primary hyperparathyroidism
**1.1 Nutritional supplementation<ref name="pmid25162668">{{cite journal| author=Marcocci C, Bollerslev J, Khan AA, Shoback DM| title=Medical management of primary hyperparathyroidism: proceedings of the fourth International Workshop on the Management of Asymptomatic Primary Hyperparathyroidism. | journal=J Clin Endocrinol Metab | year= 2014 | volume= 99 | issue= 10 | pages= 3607-18 | pmid=25162668 | doi=10.1210/jc.2014-1417 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25162668  }}</ref>
***1.1.1 Low [[calcium]] intake<ref name="pmid12474069">{{cite journal| author=Jorde R, Szumlas K, Haug E, Sundsfjord J| title=The effects of calcium supplementation to patients with primary hyperparathyroidism and a low calcium intake. | journal=Eur J Nutr | year= 2002 | volume= 41 | issue= 6 | pages= 258-63 | pmid=12474069 | doi=10.1007/s00394-002-0383-1 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12474069  }}</ref>
****Preferred regimen (1): [[Elemental calcium]] 500 mg PO q24h
***:'''Note:''' Dietary [[calcium]] restriction is not necessary in primary hyperparathyroidism.
***1.1.2 [[Vitamin D]] depletion
****Preferred regimen (1): [[Cholecalciferol]] 600–1000 IU PO q24h
***:'''Note(1):''' [[Vitamin D deficiency]] is considered when serum level of [[25-hydroxy vitamin D]] is below 50 nM (20 ng/mL).<ref name="pmid21118827">{{cite journal| author=Ross AC, Manson JE, Abrams SA, Aloia JF, Brannon PM, Clinton SK et al.| title=The 2011 report on dietary reference intakes for calcium and vitamin D from the Institute of Medicine: what clinicians need to know. | journal=J Clin Endocrinol Metab | year= 2011 | volume= 96 | issue= 1 | pages= 53-8 | pmid=21118827 | doi=10.1210/jc.2010-2704 | pmc=3046611 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21118827  }}</ref>
***:'''Note(2):''' Serum [[calcium]] levels and [[urinary]] [[calcium]] [[excretion]] should be monitored during [[vitamin D]] supplementation. [[Vitamin D]] supplementation should be stopped if serum [[calcium]] levels is >11.6 mg/dL and/or [[urinary]] [[calcium]] [[excretion]] is >400 mg/24 h.
***:'''Note(3):''' The goal of [[vitamin D]] supplementation is to keep [[25-hydroxy vitamin D]] level between 50 nmol/L to 75 nmol/L.
**1.2 Pharmacotherapy
***1.2.1 [[Bisphosphonate|Bisphosphonates]]
****Preferred regimen (1): [[Alendronate]] 10 mg PO q24h<ref name="pmid12574184">{{cite journal| author=Chow CC, Chan WB, Li JK, Chan NN, Chan MH, Ko GT et al.| title=Oral alendronate increases bone mineral density in postmenopausal women with primary hyperparathyroidism. | journal=J Clin Endocrinol Metab | year= 2003 | volume= 88 | issue= 2 | pages= 581-7 | pmid=12574184 | doi=10.1210/jc.2002-020890 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12574184  }}</ref><ref name="pmid15240609">{{cite journal| author=Khan AA, Bilezikian JP, Kung AW, Ahmed MM, Dubois SJ, Ho AY et al.| title=Alendronate in primary hyperparathyroidism: a double-blind, randomized, placebo-controlled trial. | journal=J Clin Endocrinol Metab | year= 2004 | volume= 89 | issue= 7 | pages= 3319-25 | pmid=15240609 | doi=10.1210/jc.2003-030908 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15240609  }}</ref>
***1.2.2 Calcimimetics
****Preferred regimen (1): [[Cinacalcet]] HCl 30-120 mg PO q12h<ref name="pmid15522938">{{cite journal| author=Peacock M, Bilezikian JP, Klassen PS, Guo MD, Turner SA, Shoback D| title=Cinacalcet hydrochloride maintains long-term normocalcemia in patients with primary hyperparathyroidism. | journal=J Clin Endocrinol Metab | year= 2005 | volume= 90 | issue= 1 | pages= 135-41 | pmid=15522938 | doi=10.1210/jc.2004-0842 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15522938  }}</ref><ref name="pmid23730501">{{cite journal| author=Luque-Fernández I, García-Martín A, Luque-Pazos A| title=Experience with cinacalcet in primary hyperparathyroidism: results after 1 year of treatment. | journal=Ther Adv Endocrinol Metab | year= 2013 | volume= 4 | issue= 3 | pages= 77-81 | pmid=23730501 | doi=10.1177/2042018813482344 | pmc=3666442 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23730501 }}</ref>
***:'''Note(1):''' [[Cinacalcet]] may be used in patients with familial primary hyperparathyroidism as a treatment option for patients having recurrent or persistent [[hypercalcemia]] after [[parathyroidectomy]].
***:'''Note(2):''' A combination of [[Bisphosphonate|bisphosphonates]] and calcimimetics may be used to reduce the serum [[calcium]] and improve [[bone mineral density]].<ref name="pmid21445714">{{cite journal| author=Faggiano A, Di Somma C, Ramundo V, Severino R, Vuolo L, Coppola A et al.| title=Cinacalcet hydrochloride in combination with alendronate normalizes hypercalcemia and improves bone mineral density in patients with primary hyperparathyroidism. | journal=Endocrine | year= 2011 | volume= 39 | issue= 3 | pages= 283-7 | pmid=21445714 | doi=10.1007/s12020-011-9459-0 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21445714  }}</ref>
***1.2.3 [[Estrogen receptor]]-targeted therapy ([[Postmenopausal|post-menopausal women]])
****Preferred regimen (1): Conjugated equine [[estrogen]] 0.625 mg q24h + [[medroxyprogesterone acetate]] 5mg q24h
***:'''Note(1):''' The risk-benefit ratio should be assessed with respect to known relative or absolute contraindication to use of [[estrogen]] in each patient.
*2. Secondary hyperparathyroidism<ref name="pmid18957950">{{cite journal| author=Wetmore JB, Quarles LD| title=Calcimimetics or vitamin D analogs for suppressing parathyroid hormone in end-stage renal disease: time for a paradigm shift? | journal=Nat Clin Pract Nephrol | year= 2009 | volume= 5 | issue= 1 | pages= 24-33 | pmid=18957950 | doi=10.1038/ncpneph0977 | pmc=3924719 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18957950  }}</ref>
**2.1 Secondary hyperparathyroidism due to [[vitamin D deficiency]]
***2.1.1 Vitamin D analogs
****Preferred regimen (1): [[Calcitriol]]
****Preferred regimen (2): [[Paricalcitol]]
****Preferred regimen (3): [[Doxercalciferol]]
**2.2 Secondary hyperparathyroidism due to [[Chronic renal failure]]
***2.2.1 Calcimimetics<ref name="pmid16632010">{{cite journal| author=Strippoli GF, Palmer S, Tong A, Elder G, Messa P, Craig JC| title=Meta-analysis of biochemical and patient-level effects of calcimimetic therapy. | journal=Am J Kidney Dis | year= 2006 | volume= 47 | issue= 5 | pages= 715-26 | pmid=16632010 | doi=10.1053/j.ajkd.2006.01.015 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16632010  }}</ref><ref name="pmid15673327">{{cite journal| author=Moe SM, Chertow GM, Coburn JW, Quarles LD, Goodman WG, Block GA et al.| title=Achieving NKF-K/DOQI bone metabolism and disease treatment goals with cinacalcet HCl. | journal=Kidney Int | year= 2005 | volume= 67 | issue= 2 | pages= 760-71 | pmid=15673327 | doi=10.1111/j.1523-1755.2005.67139.x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15673327  }}</ref>
****Preferred regimen (1): [[Cinacalcet]] HCL 30-180 mg PO q24h
***2.2.2 [[Phosphate binders]]/[[phosphate]] restriction
***2.2.3 Vitamin D analogs<ref name="pmid18310602">{{cite journal| author=Block GA, Zeig S, Sugihara J, Chertow GM, Chi EM, Turner SA et al.| title=Combined therapy with cinacalcet and low doses of vitamin D sterols in patients with moderate to severe secondary hyperparathyroidism. | journal=Nephrol Dial Transplant | year= 2008 | volume= 23 | issue= 7 | pages= 2311-8 | pmid=18310602 | doi=10.1093/ndt/gfn026 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18310602  }}</ref><ref name="pmid17699221">{{cite journal| author=Chertow GM, Blumenthal S, Turner S, Roppolo M, Stern L, Chi EM et al.| title=Cinacalcet hydrochloride (Sensipar) in hemodialysis patients on active vitamin D derivatives with controlled PTH and elevated calcium x phosphate. | journal=Clin J Am Soc Nephrol | year= 2006 | volume= 1 | issue= 2 | pages= 305-12 | pmid=17699221 | doi=10.2215/CJN.00870805 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17699221  }}</ref>
****Preferred regimen (1): [[Calcitriol]] 0.5 μg thrice weekly
****Preferred regimen (2): [[Paricalcitol]] 2 μg thrice weekly
****Preferred regimen (3): [[Doxercalciferol]] 1 μg thrice weekly
*3. Tertiary hyperparathyroidism
**3.1 Calcimimetic drugs<ref name="pmid28518414">{{cite journal| author=Dulfer RR, Franssen GJH, Hesselink DA, Hoorn EJ, van Eijck CHJ, van Ginhoven TM| title=Systematic review of surgical and medical treatment for tertiary hyperparathyroidism. | journal=Br J Surg | year= 2017 | volume= 104 | issue= 7 | pages= 804-813 | pmid=28518414 | doi=10.1002/bjs.10554 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=28518414  }}</ref>
**:*Preferred regimen (1): [[Cinacalcet]] HCL
 
=== 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 [[Proton pump inhibitor|PPI]]<nowiki/>s for many [[Gastrointestinal tract|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. <ref name="pmid23363383">{{cite journal |vauthors=Ito T, Igarashi H, Uehara H, Jensen RT |title=Pharmacotherapy of Zollinger-Ellison syndrome |journal=Expert Opin Pharmacother |volume=14 |issue=3 |pages=307–21 |year=2013 |pmid=23363383 |pmc=3580316 |doi=10.1517/14656566.2013.767332 |url=}}</ref>
*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]]. [[Proton pump inhibitor|PPI]]’s are preferred over [[H2 receptor]] blockers due to their higher potency and longer duration of action. <ref name="pmid28722872">{{cite journal |vauthors=Cingam S, Karanchi H |title= |journal= |volume= |issue= |pages= |year= |pmid=28722872 |doi= |url=}}</ref>
Pharmacotherapy for Zollinger-Ellison syndrome may includes the following: <ref name="pmid23363383">{{cite journal |vauthors=Ito T, Igarashi H, Uehara H, Jensen RT |title=Pharmacotherapy of Zollinger-Ellison syndrome |journal=Expert Opin Pharmacother |volume=14 |issue=3 |pages=307–21 |year=2013 |pmid=23363383 |pmc=3580316 |doi=10.1517/14656566.2013.767332 |url=}}</ref> <ref name="pmid16423003">{{cite journal| author=Metz DC, Comer GM, Soffer E, Forsmark CE, Cryer B, Chey W et al.| title=Three-year oral pantoprazole administration is effective for patients with Zollinger-Ellison syndrome and other hypersecretory conditions. | journal=Aliment Pharmacol Ther | year= 2006 | volume= 23 | issue= 3 | pages= 437-44 | pmid=16423003 | doi=10.1111/j.1365-2036.2006.02762.x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16423003  }}</ref> <ref name="pmid15645403">{{cite journal| author=Hirschowitz BI, Simmons J, Mohnen J| title=Clinical outcome using lansoprazole in acid hypersecretors with and without Zollinger-Ellison syndrome: a 13-year prospective study. | journal=Clin Gastroenterol Hepatol | year= 2005 | volume= 3 | issue= 1 | pages= 39-48 | pmid=15645403 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15645403  }}</ref>
*[[Proton pump inhibitor]]s
:*Preferred regimen (1): [[Omeprazole]] 60 mg per day
:*Preferred regimen (2):[[Esomeprazole]] 120 mg per day
:*Preferred regimen (3):[[Lansoprazole]] 45 mg per day
:*Preferred regimen (4):[[Rabeprazole]] 60 mg per day
:*Preferred regimen (5):[[Pantoprazole]] 120 mg per day
*[[H2-receptor antagonist]]s
:*Preferred regimen (1):[[Famotidine]]
:*Preferred regimen (2):[[Ranitidine]]
*[[Chemotherapy]] used for [[tumors ]]that can not be surgically resected
:*Preferred regimen (1): [[Streptozotocin]]
:*Preferred regimen (2)[[5-fluorouracil]]
:*Preferred regimen (3): [[Doxorubicin]]
===[[Hormonal therapy]]===
:*[[Octreotide]] can be used to slow down [[acid secretion]]. [[Somatostatin]] analogue [[octreotide]] is effective in controlling systemic effects related to multiple [[liver metastases]] from a [[gastrinoma]]. <ref name="pmid14564638">{{cite journal| author=Saijo F, Naito H, Funayama Y, Fukushima K, Shibata C, Hashimoto A et al.| title=Octreotide in control of multiple liver metastases from gastrinoma. | journal=J Gastroenterol | year= 2003 | volume= 38 | issue= 9 | pages= 905-8 | pmid=14564638 | doi=10.1007/s00535-002-1170-8 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14564638  }}</ref>
:*In the management of gastroenteropancreatic (GEP) [[neoplasia]] in [[hypergastrinemic]] [[MEN]]-1 patients, [[octreotide]] is considered as a safe and effective adjunct to surgical strategies.  <ref name="pmid10570446">{{cite journal| author=Burgess JR, Greenaway TM, Parameswaran V, Shepherd JJ| title=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. | journal=Cancer | year= 1999 | volume= 86 | issue= 10 | pages= 2154-9 | pmid=10570446 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10570446  }}</ref>
:*In patients with progressive [[malignant]] [[gastrinoma]], [[octreotide]] is an effective [[antitumor]] treatment. In patients with progressive [[malignant]] [[gastrinoma]],  [[octreotide]] treatment helps replace [[chemotherapy]] as the standard treatment especially in patients with slow-growing [[tumors]]. <ref name="pmid11900219">{{cite journal| author=Shojamanesh H, Gibril F, Louie A, Ojeaburu JV, Bashir S, Abou-Saif A et al.| title=Prospective study of the antitumor efficacy of long-term octreotide treatment in patients with progressive metastatic gastrinoma. | journal=Cancer | year= 2002 | volume= 94 | issue= 2 | pages= 331-43 | pmid=11900219 | doi=10.1002/cncr.10195 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11900219  }}</ref>
*In patients who are not suitable for surgery or in patients with widespread [[metastasis]], conservative management with PPIs is also recommended.  <ref name="pmid28722872">{{cite journal |vauthors=Cingam S, Karanchi H |title= |journal= |volume= |issue= |pages= |year= |pmid=28722872 |doi= |url=}}</ref>
*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. <ref name="pmid28722872">{{cite journal |vauthors=Cingam S, Karanchi H |title= |journal= |volume= |issue= |pages= |year= |pmid=28722872 |doi= |url=}}</ref>
 
==References==
==References==
{{Reflist|2}}
{{reflist|2}}
 
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Latest revision as of 02:45, 27 November 2017

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

Overview

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.

Hyperparathyroidism 

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]

References

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  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 http://radiopaedia.org/articles/pituitary-adenoma 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.
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  24. 24.0 24.1 24.2 Cingam S, Karanchi H. PMID 28722872. Missing or empty |title= (help)
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