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

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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				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]

				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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				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. [Full Text] [PDF]

				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. 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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				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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				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]

				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]

				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]

				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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				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]