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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

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
Multiple Endocrine Neoplasia: Genetics and Clinical Management.
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
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

Curated

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

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
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
Multiple Endocrine Neoplasia: Genetics and Clinical Management.
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
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
Multiple Endocrine Neoplasia: Genetics and Clinical Management.
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
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

Approach to the Patient: Insulinoma.
Hofland J, Refardt JC, Feelders RA, Christ E, de Herder WW
J Clin Endocrinol Metab 2024 Mar 15;109(4):1109-1118. doi: 10.1210/clinem/dgad641. PMID: 37925662Free PMC Article
Molecular and Clinical Spectrum of Primary Hyperparathyroidism.
Jha S, Simonds WF
Endocr Rev 2023 Sep 15;44(5):779-818. doi: 10.1210/endrev/bnad009. PMID: 36961765Free PMC Article
Parathyroid Disorders.
Sell J, Ramirez S, Partin M
Am Fam Physician 2022 Mar 1;105(3):289-298. PMID: 35289573
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
Pharmacotherapy of Zollinger-Ellison syndrome.
Ito T, Igarashi H, Uehara H, Jensen RT
Expert Opin Pharmacother 2013 Feb;14(3):307-21. Epub 2013 Jan 30 doi: 10.1517/14656566.2013.767332. PMID: 23363383Free 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
The variable penetrance and spectrum of manifestations of multiple endocrine neoplasia type 1.
Carty SE, Helm AK, Amico JA, Clarke MR, Foley TP, Watson CG, Mulvihill JJ
Surgery 1998 Dec;124(6):1106-13; discussion 1113-4. doi: 10.1067/msy.1998.93107. PMID: 9854591

Clinical prediction guides

Clinical Factors Predicting Multiple Endocrine Neoplasia Type 1 and Type 4 in Patients with Neuroendocrine Tumors.
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
Expressions of Cushing's syndrome in multiple endocrine neoplasia type 1.
Simonds WF
Front Endocrinol (Lausanne) 2023;14:1183297. Epub 2023 Jun 20 doi: 10.3389/fendo.2023.1183297. PMID: 37409236Free PMC Article
Multiple Endocrine Neoplasia Type 1 Syndrome Pancreatic Neuroendocrine Tumor Genotype/Phenotype: Is There Any Advance on Predicting or Preventing?
Ramamoorthy B, Nilubol N
Surg Oncol Clin N Am 2023 Apr;32(2):315-325. doi: 10.1016/j.soc.2022.10.008. PMID: 36925188Free PMC Article
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

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
Cystic pancreatic neuroendocrine tumors (cPNETs): a systematic review and meta-analysis of case series.
Hurtado-Pardo L, Cienfuegos JA, Ruiz-Canela M, Panadero P, Benito A, Hernández Lizoain JL
Rev Esp Enferm Dig 2017 Nov;109(11):778-787. doi: 10.17235/reed.2017.5044/2017. PMID: 29072081
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

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

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

    Reviews

    Related information

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      Related medical variations
    • GTR
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    • GTR(Clinical)
      Clinical tests in GTR
    • MeSH
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