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The Journal of Clinical Endocrinology & Metabolism Vol. 86, No. 12 5658-5671
Copyright © 2001 by The Endocrine Society

Special Features

CONSENSUS: Guidelines for Diagnosis and Therapy of MEN Type 1 and Type 2

Maria Luisa Brandi, Robert F. Gagel, Alberto Angeli, John P. Bilezikian, Paolo Beck-Peccoz, Cesare Bordi, Bernard Conte-Devolx, Alberto Falchetti, Riccardo Gionata Gheri, Alfonso Libroia, Cornelius J. M. Lips, Gaetano Lombardi, Massimo Mannelli, Furio Pacini, Bruce A. J. Ponder, Frank Raue, Britt Skogseid, Guido Tamburrano, Rajesh V. Thakker, Norman W. Thompson, Paola Tomassetti, Francesco Tonelli, Samuel A. Wells, Jr. and Stephen J. Marx

Department of Internal Medicine, University of Florence (M.L.B., A.F., M.M., F.T.), 50139 Florence, Italy; University of Texas, M. D. Anderson Cancer Center (R.F.G.), Houston, Texas 77030; Dipartimento di Scienze Cliniche e Biologiche, Medicina Interna I, University of Torino (A.A.), 10100 Torino, Italy; Department of Medicine, Columbia University College of Physicians and Surgeons (J.P.B.), New York, New York 10032; Institute of Endocrine Sciences, University of Milan, Ospedale Maggiore IRCCS (P.B.-P.), 20100 Milan, Italy; Department of Pathology and Laboratory Medicine, University of Parma (C.B.), 43100 Parma, Italy; Endocrinologie et Maladies Metaboliques, Timone Hospital (B.C.-D.), 13385 Marseilles, France; Unità di Endocrinologia, Azienda Ospedaliera Careggi (R.G.G.), Florence, Italy; Divisione di Endocrinologia, Ospedale Niguarda (A.L.), Milan, Italy; Department of Internal Medicine, University Medical Center (C.J.M.L.), 3508 GA Utrecht, The Netherlands; Department of Molecular and Clinical Endocrinology, Federico II University (G.L.), 80100 Naples, Italy; Department of Clinical Physiopathology, University of Florence (M.M., F.T.), Florence, Italy; Department of Endocrinology and Metabolism, Section of Endocrinology, University of Pisa (F.P.), 56100 Pisa, Italy; Department of Genetics, University of Cambridge (B.A.J.P.), Cambridge CB2 2XZ, United Kingdom; Endokrinologische Gemeinschaftspraxis (F.R.), 69111 Heidelberg, Germany; Endocrine Oncology Unit, University Hospital (B.S.), S-751 85 Uppsala, Sweden; Department of Clinical Science, Endocrine Section, University of Rome La Sapienza (G.T.), 00100 Rome, Italy; Nuffield Department of Medicine, University of Oxford, John Radcliffe Hospital (R.V.T.), Oxford OX3 9DU, United Kingdom; Division of Endocrine Surgery, University of Michigan Medical Center (N.W.T.), Ann Arbor, Michigan 48109-0331; Department of Internal Medicine and Gastroenterology, University of Bologna (P.T.), 40100 Bologna, Italy; Department of Surgery, Washington University School of Medicine (S.A.W.), St. Louis, Missouri 63110; and Metabolic Diseases Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health (S.J.M.), Bethesda, Maryland 20862

Address all correspondence and requests for reprints to: Maria Luisa Brandi, M.D., Ph.D., Department of Internal Medicine and Regional Center for Hereditary Endocrine Tumors, Viale G. Pieraccini 6, 50139 Florence, Italy. E-mail: m.brandi{at}dmi.unifi.it

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.




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Adrenal Ganglioneuromas in Children with Multiple Endocrine Neoplasia Type 2: A Report of Two Cases
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[Abstract] [Full Text] [PDF]

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 J. Clin. Endocrinol. Metab.Home page
K. Frank-Raue, S. Rondot, W. Hoeppner, P. Goretzki, F. Raue, and W. Meng
Coincidence of Multiple Endocrine Neoplasia Types 1 and 2: Mutations in the RET Protooncogene and MEN1 Tumor Suppressor Gene in a Family Presenting with Recurrent Primary Hyperparathyroidism
J. Clin. Endocrinol. Metab., July 1, 2005; 90(7): 4063 - 4067.
[Abstract] [Full Text] [PDF]

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 J. Clin. Endocrinol. Metab.Home page
A. Machens, M. Brauckhoff, H.-J. Holzhausen, P. N. Thanh, H. Lehnert, and H. Dralle
Codon-Specific Development of Pheochromocytoma in Multiple Endocrine Neoplasia Type 2
J. Clin. Endocrinol. Metab., July 1, 2005; 90(7): 3999 - 4003.
[Abstract] [Full Text] [PDF]

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 Jpn J Clin OncolHome page
T. Snabboon, W. Plengpanich, S. Siriwong, N. Wisedopas, S. Suwanwalaikorn, W. Khovidhunkit, and V. Shotelersuk
A Novel Germline Mutation, 1793delG, of the MEN1 Gene Underlying Multiple Endocrine Neoplasia Type 1
Jpn. J. Clin. Oncol., May 1, 2005; 35(5): 280 - 282.
[Abstract] [Full Text] [PDF]

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 Arch SurgHome page
L. A. Lambert, S. E. Shapiro, J. E. Lee, N. D. Perrier, M. Truong, M. J. Wallace, A. O. Hoff, R. F. Gagel, and D. B. Evans
Surgical Treatment of Hyperparathyroidism in Patients With Multiple Endocrine Neoplasia Type 1
Arch Surg, April 1, 2005; 140(4): 374 - 382.
[Abstract] [Full Text] [PDF]

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 JCOHome page
J. E. Garber and K. Offit
Hereditary Cancer Predisposition Syndromes
J. Clin. Oncol., January 10, 2005; 23(2): 276 - 292.
[Abstract] [Full Text] [PDF]

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M. Benito, S. L. Asa, V. A. LiVolsi, V. A. West, and P. J. Snyder
Gonadotroph Tumor Associated with Multiple Endocrine Neoplasia Type 1
J. Clin. Endocrinol. Metab., January 1, 2005; 90(1): 570 - 574.
[Abstract] [Full Text] [PDF]

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 J. Med. Genet.Home page
J W Cardinal, L Bergman, N Hayward, A Sweet, J Warner, L Marks, D Learoyd, T Dwight, B Robinson, M Epstein, et al.
A report of a national mutation testing service for the MEN1 gene: clinical presentations and implications for mutation testing
J. Med. Genet., January 1, 2005; 42(1): 69 - 74.
[Abstract] [Full Text] [PDF]

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 EndocrinologyHome page
M. Santoro, R. M. Melillo, F. Carlomagno, G. Vecchio, and A. Fusco
Minireview: RET: Normal and Abnormal Functions
Endocrinology, December 1, 2004; 145(12): 5448 - 5451.
[Abstract] [Full Text] [PDF]

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 J. Clin. Endocrinol. Metab.Home page
B. Asgharian, M. L. Turner, F. Gibril, L. K. Entsuah, J. Serrano, and R. T. Jensen
Cutaneous Tumors in Patients with Multiple Endocrine Neoplasm Type 1 (MEN1) and Gastrinomas: Prospective Study of Frequency and Development of Criteria with High Sensitivity and Specificity for MEN1
J. Clin. Endocrinol. Metab., November 1, 2004; 89(11): 5328 - 5336.
[Abstract] [Full Text] [PDF]

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 J. Clin. Endocrinol. Metab.Home page
W. Hao, M. C. Skarulis, W. F. Simonds, L. S. Weinstein, S. K. Agarwal, C. Mateo, L. James-Newton, G. R. Hobbs, F. Gibril, R. T. Jensen, et al.
Multiple Endocrine Neoplasia Type 1 Variant with Frequent Prolactinoma and Rare Gastrinoma
J. Clin. Endocrinol. Metab., August 1, 2004; 89(8): 3776 - 3784.
[Abstract] [Full Text] [PDF]

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 J. Clin. Endocrinol. Metab.Home page
C. A. Carrasco, A. A. Gonzalez, C. A. Carvajal, C. Campusano, E. Oestreicher, E. Arteaga, N. Wohllk, and C. E. Fardella
Novel Intronic Mutation of MEN1 Gene Causing Familial Isolated Primary Hyperparathyroidism
J. Clin. Endocrinol. Metab., August 1, 2004; 89(8): 4124 - 4129.
[Abstract] [Full Text] [PDF]

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 J. Clin. Endocrinol. Metab.Home page
C. Jimenez, M. A. Habra, S.-C. E. Huang, A. El-Naggar, S. E. Shapiro, D. B. Evans, G. Cote, and R. F. Gagel
Pheochromocytoma and Medullary Thyroid Carcinoma: A New Genotype-Phenotype Correlation of the RET Protooncogene 891 Germline Mutation
J. Clin. Endocrinol. Metab., August 1, 2004; 89(8): 4142 - 4145.
[Abstract] [Full Text] [PDF]

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 Am. J. Pathol.Home page
R. M. Melillo, A. M. Cirafici, V. De Falco, M. Bellantoni, G. Chiappetta, A. Fusco, F. Carlomagno, A. Picascia, D. Tramontano, G. Tallini, et al.
The Oncogenic Activity of RET Point Mutants for Follicular Thyroid Cells May Account for the Occurrence of Papillary Thyroid Carcinoma in Patients Affected by Familial Medullary Thyroid Carcinoma
Am. J. Pathol., August 1, 2004; 165(2): 511 - 521.
[Abstract] [Full Text] [PDF]

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 J. Clin. Endocrinol. Metab.Home page
T. Ebeling, O. Vierimaa, S. Kytola, J. Leisti, and P. I. Salmela
Effect of Multiple Endocrine Neoplasia Type 1 (MEN1) Gene Mutations on Premature Mortality in Familial MEN1 Syndrome with Founder Mutations
J. Clin. Endocrinol. Metab., July 1, 2004; 89(7): 3392 - 3396.
[Abstract] [Full Text] [PDF]

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 J. Clin. Endocrinol. Metab.Home page
C. Jimenez, G. T. Dang, P. N. Schultz, A. El-Naggar, S. Shapiro, E. A. Barnes, D. B. Evans, R. Vassilopoulou-Sellin, R. F. Gagel, G. J. Cote, et al.
A Novel Point Mutation of the RET Protooncogene Involving the Second Intracellular Tyrosine Kinase Domain in a Family with Medullary Thyroid Carcinoma
J. Clin. Endocrinol. Metab., July 1, 2004; 89(7): 3521 - 3526.
[Abstract] [Full Text] [PDF]

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 Endocr. Rev.Home page
G. A. Kaltsas, G. M. Besser, and A. B. Grossman
The Diagnosis and Medical Management of Advanced Neuroendocrine Tumors
Endocr. Rev., June 1, 2004; 25(3): 458 - 511.
[Abstract] [Full Text] [PDF]

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 Cancer Res.Home page
S. Jain, M. A. Watson, M. K. DeBenedetti, Y. Hiraki, J. F. Moley, and J. Milbrandt
Expression Profiles Provide Insights into Early Malignant Potential and Skeletal Abnormalities in Multiple Endocrine Neoplasia Type 2B Syndrome Tumors
Cancer Res., June 1, 2004; 64(11): 3907 - 3913.
[Abstract] [Full Text] [PDF]

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 J. Clin. Endocrinol. Metab.Home page
K. L. Becker, E. S. Nylen, J. C. White, B. Muller, and R. H. Snider Jr.
Procalcitonin and the Calcitonin Gene Family of Peptides in Inflammation, Infection, and Sepsis: A Journey from Calcitonin Back to Its Precursors
J. Clin. Endocrinol. Metab., April 1, 2004; 89(4): 1512 - 1525.
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J Warner, M Epstein, A Sweet, D Singh, J Burgess, S Stranks, P Hill, D Perry-Keene, D Learoyd, B Robinson, et al.
Genetic testing in familial isolated hyperparathyroidism: unexpected results and their implications
J. Med. Genet., March 1, 2004; 41(3): 155 - 160.
[Abstract] [Full Text] [PDF]

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A. M. Alvares Da Silva, R. M. B. Maciel, M. R. Dias Da Silva, S. R. C. Toledo, M. B. De Carvalho, and J. M. Cerutti
A Novel Germ-Line Point Mutation in RET Exon 8 (Gly533Cys) in a Large Kindred with Familial Medullary Thyroid Carcinoma
J. Clin. Endocrinol. Metab., November 1, 2003; 88(11): 5438 - 5443.
[Abstract] [Full Text] [PDF]

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 NEJMHome page
A. Machens, P. Niccoli-Sire, J. Hoegel, K. Frank-Raue, T. J. van Vroonhoven, H.-D. Roeher, R. A. Wahl, P. Lamesch, F. Raue, B. Conte-Devolx, et al.
Early Malignant Progression of Hereditary Medullary Thyroid Cancer
N. Engl. J. Med., October 16, 2003; 349(16): 1517 - 1525.
[Abstract] [Full Text] [PDF]

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 NEJMHome page
G. J. Cote and R. F. Gagel
Lessons Learned from the Management of a Rare Genetic Cancer
N. Engl. J. Med., October 16, 2003; 349(16): 1566 - 1568.
[Full Text] [PDF]

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 JNCI J Natl Cancer InstHome page
J. Bryant, J. Farmer, L. J. Kessler, R. R. Townsend, and K. L. Nathanson
Pheochromocytoma: The Expanding Genetic Differential Diagnosis
J Natl Cancer Inst, August 20, 2003; 95(16): 1196 - 1204.
[Abstract] [Full Text] [PDF]

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 J. Clin. Endocrinol. Metab.Home page
M. K. Punales, H. Graf, J. L. Gross, and A. L. Maia
RET Codon 634 Mutations in Multiple Endocrine Neoplasia Type 2: Variable Clinical Features and Clinical Outcome
J. Clin. Endocrinol. Metab., June 1, 2003; 88(6): 2644 - 2649.
[Abstract] [Full Text] [PDF]

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 J. Med. Genet.Home page
A Cebrian, S Ruiz-Llorente, A Cascon, M Pollan, J J Diez, A Pico, D Telleria, J Benitez, and M Robledo
Mutational and gross deletion study of the MEN1 gene and correlation with clinical features in Spanish patients
J. Med. Genet., May 1, 2003; 40(5): e72 - 72.
[Full Text] [PDF]

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 Arch SurgHome page
L. Yip, G. J. Cote, S. E. Shapiro, G. D. Ayers, C. E. Herzog, R. V. Sellin, S. I. Sherman, R. F. Gagel, J. E. Lee, and D. B. Evans
Multiple Endocrine Neoplasia Type 2: Evaluation of the Genotype-Phenotype Relationship
Arch Surg, April 1, 2003; 138(4): 409 - 416.
[Abstract] [Full Text] [PDF]

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 J. Clin. Endocrinol. Metab.Home page
F. Gibril, Y.-J. Chen, D. S. Schrump, A. Vortmeyer, Z. Zhuang, I. A. Lubensky, J. C. Reynolds, A. Louie, L. K. Entsuah, K. Huang, et al.
Prospective Study of Thymic Carcinoids in Patients with Multiple Endocrine Neoplasia Type 1
J. Clin. Endocrinol. Metab., March 1, 2003; 88(3): 1066 - 1081.
[Abstract] [Full Text] [PDF]

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 J. Clin. Endocrinol. Metab.Home page
C. A. Koch, K. Pacak, and G. P. Chrousos
The Molecular Pathogenesis of Hereditary and Sporadic Adrenocortical and Adrenomedullary Tumors
J. Clin. Endocrinol. Metab., December 1, 2002; 87(12): 5367 - 5384.
[Abstract] [Full Text] [PDF]

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 Clin. Chem.Home page
C. Sturgeon
Practice Guidelines for Tumor Marker Use in the Clinic
Clin. Chem., August 1, 2002; 48(8): 1151 - 1159.
[Abstract] [Full Text] [PDF]

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 Mayo Clin Proc.Home page
S. Dionisi, S. Minisola, J. Pepe, S. De Geronimo, F. Paglia, L. Memeo, and L. A. Fitzpatrick
Concurrent Parathyroid Adenomas and Carcinoma in the Setting of Multiple Endocrine Neoplasia Type 1: Presentation as Hypercalcemic Crisis
Mayo Clin. Proc., August 1, 2002; 77(8): 866 - 869.
[Abstract] [PDF]

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 J. Clin. Endocrinol. Metab.Home page
P. Igaz, A. Patocs, K. Racz, I. Klein, A. Varadi, and O. Esik
Occurrence of Pheochromocytoma in a MEN2A Family with Codon 609 Mutation of the RET Protooncogene
J. Clin. Endocrinol. Metab., June 1, 2002; 87(6): 2994 - 2994.
[Full Text] [PDF]

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 Arch SurgHome page
M. A. Kouvaraki, J. E. Lee, S. E. Shapiro, R. F. Gagel, S. I. Sherman, R. V. Sellin, G. J. Cote, and D. B. Evans
Genotype-Phenotype Analysis in Multiple Endocrine Neoplasia Type 1
Arch Surg, June 1, 2002; 137(6): 641 - 647.
[Abstract] [Full Text] [PDF]

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 J. Clin. Endocrinol. Metab.Home page
S. J. Marx and L. K. Nieman
Aggressive Pituitary Tumors in MEN1: Do They Refute the Two-Hit Model of Tumorigenesis?
J. Clin. Endocrinol. Metab., February 1, 2002; 87(2): 453 - 456.
[Full Text] [PDF]


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