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Multiple endocrine neoplasia

MedGen UID:
45036
•Concept ID:
C0027662
•
Neoplastic Process
Synonyms: Adenomatoses, Familial Endocrine; Adenomatoses, Multiple Endocrine; Adenomatosis, Familial Endocrine; Adenomatosis, Multiple Endocrine; Adenopathies, Multiple Endocrine; Adenopathy, Multiple Endocrine; Endocrine Adenomatoses, Familial; Endocrine Adenomatoses, Multiple; Endocrine Adenomatosis, Familial; Endocrine Adenomatosis, Multiple; Endocrine Adenopathies, Multiple; Endocrine Adenopathy, Multiple; Endocrine Neoplasia, Multiple; Endocrine Neoplasms, Multiple; Familial Endocrine Adenomatoses; Familial Endocrine Adenomatosis; Multiple Endocrine Adenomatoses; Multiple Endocrine Adenomatosis; Multiple Endocrine Adenopathies; Multiple Endocrine Adenopathy; Multiple Endocrine Neoplasia; Multiple Endocrine Neoplasia Syndrome; Multiple Endocrine Neoplasia Syndromes; Multiple Endocrine Neoplasms; Neoplasia, Multiple Endocrine; Neoplasms, Multiple Endocrine
SNOMED CT: Multiple endocrine adenomas (60549007); Endocrine adenomatosis (60549007); MEN - Multiple endocrine neoplasia (46724008); Multiple endocrine tumasia (46724008); MEA - Multiple endocrine adenomatosis (46724008); Familial polyendocrine adenomatosis (46724008); Multiple endocrine neoplasia (46724008)
Modes of inheritance:
Autosomal dominant inheritance
MedGen UID:
141047
•Concept ID:
C0443147
•
Intellectual Product
Source: Orphanet
A mode of inheritance that is observed for traits related to a gene encoded on one of the autosomes (i.e., the human chromosomes 1-22) in which a trait manifests in heterozygotes. In the context of medical genetics, an autosomal dominant disorder is caused when a single copy of the mutant allele is present. Males and females are affected equally, and can both transmit the disorder with a risk of 50% for each child of inheriting the mutant allele.
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Autosomal dominant inheritance (Orphanet)
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0017169
OMIM® Phenotypic series: PS131100
Orphanet: ORPHA276161

Definition

Multiple endocrine neoplasia (MEN) is a group of rare inherited cancer syndromes characterized by the development of two or more endocrine gland tumors, sometimes with tumor development in other tissues or organs. [from ORDO]

Professional guidelines

PubMed

Update on the clinical management of multiple endocrine neoplasia type 1.
Pieterman CRC, Valk GD
Clin Endocrinol (Oxf) 2022 Oct;97(4):409-423. Epub 2022 Apr 1 doi: 10.1111/cen.14727. PMID: 35319130Free PMC Article
Neuroendocrine and Adrenal Tumors, Version 2.2021, NCCN Clinical Practice Guidelines in Oncology.
Shah MH, Goldner WS, Benson AB, Bergsland E, Blaszkowsky LS, Brock P, Chan J, Das S, Dickson PV, Fanta P, Giordano T, Halfdanarson TR, Halperin D, He J, Heaney A, Heslin MJ, Kandeel F, Kardan A, Khan SA, Kuvshinoff BW, Lieu C, Miller K, Pillarisetty VG, Reidy D, Salgado SA, Shaheen S, Soares HP, Soulen MC, Strosberg JR, Sussman CR, Trikalinos NA, Uboha NA, Vijayvergia N, Wong T, Lynn B, Hochstetler C
J Natl Compr Canc Netw 2021 Jul 28;19(7):839-868. doi: 10.6004/jnccn.2021.0032. PMID: 34340212
Clinical practice guidelines for multiple endocrine neoplasia type 1 (MEN1).
Thakker RV, Newey PJ, Walls GV, Bilezikian J, Dralle H, Ebeling PR, Melmed S, Sakurai A, Tonelli F, Brandi ML; Endocrine Society
J Clin Endocrinol Metab 2012 Sep;97(9):2990-3011. Epub 2012 Jun 20 doi: 10.1210/jc.2012-1230. PMID: 22723327

Recent clinical studies

Etiology

Update on the clinical management of multiple endocrine neoplasia type 1.
Pieterman CRC, Valk GD
Clin Endocrinol (Oxf) 2022 Oct;97(4):409-423. Epub 2022 Apr 1 doi: 10.1111/cen.14727. PMID: 35319130Free PMC Article
Multiple endocrine neoplasia type 2: A review.
Mathiesen JS, Effraimidis G, Rossing M, Rasmussen ÅK, Hoejberg L, Bastholt L, Godballe C, Oturai P, Feldt-Rasmussen U
Semin Cancer Biol 2022 Feb;79:163-179. Epub 2021 Apr 1 doi: 10.1016/j.semcancer.2021.03.035. PMID: 33812987
Dermal hyperneury.
Marušić Z, Korša L, Calonje E
Clin Dermatol 2021 Mar-Apr;39(2):291-294. Epub 2020 Oct 16 doi: 10.1016/j.clindermatol.2020.10.008. PMID: 34272024
Multiple endocrine neoplasia: an update.
McDonnell JE, Gild ML, Clifton-Bligh RJ, Robinson BG
Intern Med J 2019 Aug;49(8):954-961. doi: 10.1111/imj.14394. PMID: 31387156
Clinical practice guidelines for multiple endocrine neoplasia type 1 (MEN1).
Thakker RV, Newey PJ, Walls GV, Bilezikian J, Dralle H, Ebeling PR, Melmed S, Sakurai A, Tonelli F, Brandi ML; Endocrine Society
J Clin Endocrinol Metab 2012 Sep;97(9):2990-3011. Epub 2012 Jun 20 doi: 10.1210/jc.2012-1230. PMID: 22723327

Diagnosis

Update on the clinical management of multiple endocrine neoplasia type 1.
Pieterman CRC, Valk GD
Clin Endocrinol (Oxf) 2022 Oct;97(4):409-423. Epub 2022 Apr 1 doi: 10.1111/cen.14727. PMID: 35319130Free PMC Article
Clinical aspects of multiple endocrine neoplasia type 1.
Al-Salameh A, Cadiot G, Calender A, Goudet P, Chanson P
Nat Rev Endocrinol 2021 Apr;17(4):207-224. Epub 2021 Feb 9 doi: 10.1038/s41574-021-00468-3. PMID: 33564173
Multiple endocrine neoplasia: an update.
McDonnell JE, Gild ML, Clifton-Bligh RJ, Robinson BG
Intern Med J 2019 Aug;49(8):954-961. doi: 10.1111/imj.14394. PMID: 31387156
Treatment of Pancreatic Neuroendocrine Tumors in Multiple Endocrine Neoplasia Type 1: Some Clarity But Continued Controversy.
Jensen RT, Norton JA
Pancreas 2017 May/Jun;46(5):589-594. doi: 10.1097/MPA.0000000000000825. PMID: 28426491Free PMC Article
Clinical practice guidelines for multiple endocrine neoplasia type 1 (MEN1).
Thakker RV, Newey PJ, Walls GV, Bilezikian J, Dralle H, Ebeling PR, Melmed S, Sakurai A, Tonelli F, Brandi ML; Endocrine Society
J Clin Endocrinol Metab 2012 Sep;97(9):2990-3011. Epub 2012 Jun 20 doi: 10.1210/jc.2012-1230. PMID: 22723327

Therapy

Parathyroid Disorders.
Sell J, Ramirez S, Partin M
Am Fam Physician 2022 Mar 1;105(3):289-298. PMID: 35289573
Molecular targets of tyrosine kinase inhibitors in thyroid cancer.
Fallahi P, Ferrari SM, Galdiero MR, Varricchi G, Elia G, Ragusa F, Paparo SR, Benvenga S, Antonelli A
Semin Cancer Biol 2022 Feb;79:180-196. Epub 2020 Nov 26 doi: 10.1016/j.semcancer.2020.11.013. PMID: 33249201
American Association of Clinical Endocrinology Disease State Clinical Review on the Evaluation and Management of Adrenocortical Carcinoma in an Adult: a Practical Approach.
Kiseljak-Vassiliades K, Bancos I, Hamrahian A, Habra M, Vaidya A, Levine AC, Else T
Endocr Pract 2020 Nov;26(11):1366-1383. Epub 2020 Dec 14 doi: 10.4158/DSCR-2020-0567. PMID: 33875173Free PMC Article
Treatment of symptomatic neuroendocrine tumor syndromes: recent advances and controversies.
Ito T, Lee L, Jensen RT
Expert Opin Pharmacother 2016 Nov;17(16):2191-2205. Epub 2016 Sep 23 doi: 10.1080/14656566.2016.1236916. PMID: 27635672Free PMC Article
Revised American Thyroid Association guidelines for the management of medullary thyroid carcinoma.
Wells SA Jr, Asa SL, Dralle H, Elisei R, Evans DB, Gagel RF, Lee N, Machens A, Moley JF, Pacini F, Raue F, Frank-Raue K, Robinson B, Rosenthal MS, Santoro M, Schlumberger M, Shah M, Waguespack SG; American Thyroid Association Guidelines Task Force on Medullary Thyroid Carcinoma
Thyroid 2015 Jun;25(6):567-610. doi: 10.1089/thy.2014.0335. PMID: 25810047Free PMC Article

Prognosis

Cancer statistics, 2023.
Siegel RL, Miller KD, Wagle NS, Jemal A
CA Cancer J Clin 2023 Jan;73(1):17-48. doi: 10.3322/caac.21763. PMID: 36633525
Update on the clinical management of multiple endocrine neoplasia type 1.
Pieterman CRC, Valk GD
Clin Endocrinol (Oxf) 2022 Oct;97(4):409-423. Epub 2022 Apr 1 doi: 10.1111/cen.14727. PMID: 35319130Free PMC Article
Age and MEN2 outcome.
Castinetti F
Aging (Albany NY) 2019 Jun 12;11(11):3416-3417. doi: 10.18632/aging.102030. PMID: 31188782Free PMC Article
Causes of death and prognostic factors in multiple endocrine neoplasia type 1: a prospective study: comparison of 106 MEN1/Zollinger-Ellison syndrome patients with 1613 literature MEN1 patients with or without pancreatic endocrine tumors.
Ito T, Igarashi H, Uehara H, Berna MJ, Jensen RT
Medicine (Baltimore) 2013 May;92(3):135-181. doi: 10.1097/MD.0b013e3182954af1. PMID: 23645327Free PMC Article
Insulinoma.
Grant CS
Best Pract Res Clin Gastroenterol 2005 Oct;19(5):783-98. doi: 10.1016/j.bpg.2005.05.008. PMID: 16253900

Clinical prediction guides

How to treat gastrinomas in patients with multiple endocrine neoplasia type1: surgery or long-term proton pump inhibitors?
Imamura M, Komoto I, Taki Y
Surg Today 2023 Dec;53(12):1325-1334. Epub 2022 Dec 7 doi: 10.1007/s00595-022-02627-z. PMID: 36473964Free PMC Article
Update on the clinical management of multiple endocrine neoplasia type 1.
Pieterman CRC, Valk GD
Clin Endocrinol (Oxf) 2022 Oct;97(4):409-423. Epub 2022 Apr 1 doi: 10.1111/cen.14727. PMID: 35319130Free PMC Article
Update on multiple endocrine neoplasia Type 1 and 2.
Al-Salameh A, Baudry C, Cohen R
Presse Med 2018 Sep;47(9):722-731. Epub 2018 Jun 13 doi: 10.1016/j.lpm.2018.03.005. PMID: 29909163
Patient quality of life and prognosis in multiple endocrine neoplasia type 2.
Grey J, Winter K
Endocr Relat Cancer 2018 Feb;25(2):T69-T77. Epub 2017 Oct 24 doi: 10.1530/ERC-17-0335. PMID: 29066504
Clinicopathological correlates of hyperparathyroidism.
Duan K, Gomez Hernandez K, Mete O
J Clin Pathol 2015 Oct;68(10):771-87. Epub 2015 Jul 10 doi: 10.1136/jclinpath-2015-203186. PMID: 26163537

Recent systematic reviews

Less Than Subtotal Parathyroidectomy for Multiple Endocrine Neoplasia Type 1 Primary Hyperparathyroidism: A Systematic Review and Meta-Analysis.
Bouriez D, Gronnier C, Haissaguerre M, Tabarin A, Najah H
World J Surg 2022 Nov;46(11):2666-2675. Epub 2022 Jun 29 doi: 10.1007/s00268-022-06633-7. PMID: 35767091
Molecular Diagnosis and Treatment of Multiple Endocrine Neoplasia Type 2B in Ethnic Han Chinese.
Zhang ZW, Guo X, Qi XP
Endocr Metab Immune Disord Drug Targets 2021;21(3):534-543. doi: 10.2174/1871530320666200910112230. PMID: 32914730
Clinical features and prognosis of thymic neuroendocrine tumours associated with multiple endocrine neoplasia type 1: A single-centre study, systematic review and meta-analysis.
Ye L, Wang W, Ospina NS, Jiang L, Christakis I, Lu J, Zhou Y, Zhu W, Cao Y, Wang S, Perrier ND, Young WF Jr, Ning G, Wang W
Clin Endocrinol (Oxf) 2017 Dec;87(6):706-716. Epub 2017 Oct 16 doi: 10.1111/cen.13480. PMID: 28940393
Risk-reduction surgery in pediatric surgical oncology: A perspective.
Sandoval JA, Fernandez-Pineda I, Malkan AD
J Pediatr Surg 2016 Apr;51(4):675-87. Epub 2016 Feb 3 doi: 10.1016/j.jpedsurg.2016.01.004. PMID: 26898681
The association between Hirschsprung's disease and multiple endocrine neoplasia type 2a: a systematic review.
Coyle D, Friedmacher F, Puri P
Pediatr Surg Int 2014 Aug;30(8):751-6. Epub 2014 Jun 28 doi: 10.1007/s00383-014-3538-2. PMID: 24972642

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