Consensus: Guidelines for diagnosis and therapy of MEN type 1 and type 2

M. L. Brandi, R. F. Gagel, A. Angeli, J. P. Bilezikian, P. Beck-Peccoz, C. Bordi, B. Conte-Devolx, A. Falchetti, R. G. Gheri, A. Libroia, C. J M Lips, G. Lombardi, M. Mannelli, F. Pacini, B. A J Ponder, F. Raue, B. Skogseid, G. Tamburrano, R. V. Thakker, N. W. ThompsonP. Tomassetti, F. Tonelli, Jr Wells S.A., S. J. Marx

Research output: Contribution to journalArticle

Abstract

This is a consensus statement from an international group, mostly of clinical endocrinologists. MEN1 and MEN2 are hereditary cancer syndromes. The commonest tumors secrete PTH or gastrin in MEN1, and calcitonin or catecholamines in MEN2. Management strategies improved after the discoveries of their genes. MEN1 has no clear syndromic variants. Tumor monitoring in MEN1 carriers includes biochemical tests yearly and imaging tests less often. Neck surgery includes subtotal or total parathyroidectomy, parathyroid cryopreservation, and thymectomy. Proton pump inhibitors or somatostatin analogs are the main management for oversecretion of entero-pancreatic hormones, except insulin. The roles for surgery of most entero-pancreatic tumors present several controversies: exclusion of most operations on gastrinomas and indications for surgery on other tumors. Each MEN1 family probably has an inactivating MEN1 germline mutation. Testing for a germline MEN1 mutation gives useful information, but rarely mandates an intervention. The most distinctive MEN2 variants are MEN2A, MEN2B, and familial medullary thyroid cancer (MTC). They vary in aggressiveness of MTC and spectrum of disturbed organs. Mortality in MEN2 is greater from MTC than from pheochromocytoma. Thyroidectomy, during childhood if possible, is the goal in all MEN2 carriers to prevent or cure MTC. Each MEN2 index case probably has an activating germline RET mutation. RET testing has replaced calcitonin testing to diagnose the MEN2 carrier state. The specific RET codon mutation correlates with the MEN2 syndromic variant, the age of onset of MTC, and the aggressiveness of MTC; consequently, that mutation should guide major management decisions, such as whether and when to perform thyroidectomy.

Original languageEnglish
Pages (from-to)5658-5671
Number of pages14
JournalJournal of Clinical Endocrinology and Metabolism
Volume86
Issue number12
DOIs
Publication statusPublished - 2001

Fingerprint

Multiple Endocrine Neoplasia Type 2a
Multiple Endocrine Neoplasia Type 1
Tumors
Consensus
Guidelines
Surgery
Calcitonin
Germ-Line Mutation
Testing
Pancreatic Hormones
Thyroidectomy
Proton Pump Inhibitors
Gastrins
Therapeutics
Somatostatin
Neoplasms
Catecholamines
Hereditary Neoplastic Syndromes
Gastrinoma
Genes

ASJC Scopus subject areas

  • Biochemistry
  • Endocrinology, Diabetes and Metabolism

Cite this

Brandi, M. L., Gagel, R. F., Angeli, A., Bilezikian, J. P., Beck-Peccoz, P., Bordi, C., ... Marx, S. J. (2001). Consensus: Guidelines for diagnosis and therapy of MEN type 1 and type 2. Journal of Clinical Endocrinology and Metabolism, 86(12), 5658-5671. https://doi.org/10.1210/jc.86.12.5658

Consensus : Guidelines for diagnosis and therapy of MEN type 1 and type 2. / Brandi, M. L.; Gagel, R. F.; Angeli, A.; Bilezikian, J. P.; Beck-Peccoz, P.; Bordi, C.; Conte-Devolx, B.; Falchetti, A.; Gheri, R. G.; Libroia, A.; Lips, C. J M; Lombardi, G.; Mannelli, M.; Pacini, F.; Ponder, B. A J; Raue, F.; Skogseid, B.; Tamburrano, G.; Thakker, R. V.; Thompson, N. W.; Tomassetti, P.; Tonelli, F.; Wells S.A., Jr; Marx, S. J.

In: Journal of Clinical Endocrinology and Metabolism, Vol. 86, No. 12, 2001, p. 5658-5671.

Research output: Contribution to journalArticle

Brandi, ML, Gagel, RF, Angeli, A, Bilezikian, JP, Beck-Peccoz, P, Bordi, C, Conte-Devolx, B, Falchetti, A, Gheri, RG, Libroia, A, Lips, CJM, Lombardi, G, Mannelli, M, Pacini, F, Ponder, BAJ, Raue, F, Skogseid, B, Tamburrano, G, Thakker, RV, Thompson, NW, Tomassetti, P, Tonelli, F, Wells S.A., J & Marx, SJ 2001, 'Consensus: Guidelines for diagnosis and therapy of MEN type 1 and type 2', Journal of Clinical Endocrinology and Metabolism, vol. 86, no. 12, pp. 5658-5671. https://doi.org/10.1210/jc.86.12.5658
Brandi, M. L. ; Gagel, R. F. ; Angeli, A. ; Bilezikian, J. P. ; Beck-Peccoz, P. ; Bordi, C. ; Conte-Devolx, B. ; Falchetti, A. ; Gheri, R. G. ; Libroia, A. ; Lips, C. J M ; Lombardi, G. ; Mannelli, M. ; Pacini, F. ; Ponder, B. A J ; Raue, F. ; Skogseid, B. ; Tamburrano, G. ; Thakker, R. V. ; Thompson, N. W. ; Tomassetti, P. ; Tonelli, F. ; Wells S.A., Jr ; Marx, S. J. / Consensus : Guidelines for diagnosis and therapy of MEN type 1 and type 2. In: Journal of Clinical Endocrinology and Metabolism. 2001 ; Vol. 86, No. 12. pp. 5658-5671.
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AU - Beck-Peccoz, P.

AU - Bordi, C.

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