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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.
 
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

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
Norton JA, Krampitz G, Jensen RT
Surg Oncol Clin N Am 2015 Oct;24(4):795-832. Epub 2015 Jul 27 doi: 10.1016/j.soc.2015.06.008. PMID: 26363542Free PMC Article
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

Curated

NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®), Neuroendocrine and Adrenal Tumors, 2023

Recent clinical studies

Etiology

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
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
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
Norton JA, Krampitz G, Jensen RT
Surg Oncol Clin N Am 2015 Oct;24(4):795-832. Epub 2015 Jul 27 doi: 10.1016/j.soc.2015.06.008. PMID: 26363542Free PMC Article
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

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
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
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
Norton JA, Krampitz G, Jensen RT
Surg Oncol Clin N Am 2015 Oct;24(4):795-832. Epub 2015 Jul 27 doi: 10.1016/j.soc.2015.06.008. PMID: 26363542Free PMC Article
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

Sell J, Ramirez S, Partin M
Am Fam Physician 2022 Mar 1;105(3):289-298. PMID: 35289573
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
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
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
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

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
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
Castinetti F
Aging (Albany NY) 2019 Jun 12;11(11):3416-3417. doi: 10.18632/aging.102030. PMID: 31188782Free PMC Article
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
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

Clinical prediction guides

Faggiano A, Fazzalari B, Mikovic N, Russo F, Zamponi V, Mazzilli R, Guarnieri V, Piane M, Visco V, Petrucci S
Genes (Basel) 2023 Sep 10;14(9) doi: 10.3390/genes14091782. PMID: 37761922Free PMC Article
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
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
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
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

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
Zhang ZW, Guo X, Qi XP
Endocr Metab Immune Disord Drug Targets 2021;21(3):534-543. doi: 10.2174/1871530320666200910112230. PMID: 32914730
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
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
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

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
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      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Curated

    • NCCN, 2023
      NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®), Neuroendocrine and Adrenal Tumors, 2023

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