title: MEN1 disease occurring before 21 years old: a 160-patient cohort study from the Groupe d'étude des Tumeurs Endocrines.
abstract: BACKGROUND
  Multiple endocrine neoplasia Type-1 (MEN1) in young patients is only described by case reports.
  
  
  OBJECTIVE
  To improve the knowledge of MEN1 natural history before 21 years old.
  
  
  METHODS
  Obtain a description of the first symptoms occurring before 21 years old (clinical symptoms, biological or imaging abnormalities), surgical outcomes related to MEN1 Neuro Endocrine Tumors (NETs) occurring in a group of 160 patients extracted from the "Groupe d'étude des Tumeurs Endocrines" MEN1 cohort.
  
  
  RESULTS
  The first symptoms were related to hyperparathyroidism in 122 cases (75%), pituitary adenoma in 55 cases (34%), nonsecreting pancreatic tumor (NSPT) in 14 cases (9%), insulinoma in 20 cases (12%), gastrinoma in three cases (2%), malignant adrenal tumors in 2 cases (1%), and malignant thymic-NET in one case (1%). Hyperparathyrodism was the first lesion in 90 cases (56%). The first symptoms occurred before 10 years old in 22 cases (14%) and before 5 years old in five cases (3%). Surgery was performed before age 21 in 66 patients (41%) with a total of 74 operations: pituitary adenoma (n = 9, 16%), hyperparathyroidism (n = 38, 31%), gastrinoma (n = 1, 33%), NSPT (n = 5, 36%), and all cases of insulinoma, adrenal tumors, and thymic-NET. One patient died before age 21 due to a thymic-NET. Overall, lesions were malignant in four cases.
  
  
  CONCLUSIONS
  Various MEN1 lesions occurred frequently before 21 years old, but mainly after 10 years of age. Rare, aggressive tumors may develop at any age. Hyperparathyroidism was the most frequently encountered lesion but was not always the first biological or clinical abnormality to appear during the course of MEN1.
authors.affiliation: Centre Hospitalier Universitaire de Dijon (P.G.), Endocrine Surgery, Dijon, France; INSERM U866, Dijon, France; University of Burgundy, Dijon, France; Service d'Endocrinologie et Maladies Métaboliques (A.D., N.L-B.), Centre Hospitalier Universitaire, Hôpital Robert Debré, Reims, France, Clinique d'Endocrinologie (M.L.), Centre Hospitalier Universitaire, Nantes, France; Service de Médecine Interne et Endocrinologie (C.C-B.), Clinique Marc Linquette, Centre Hospitalier Régional et Universitaire, Lille, France; Service d'Oncologie Médicale (P.N.), Institut Paoli-Calmettes, APHM, Université Aix-Marseille, Marseille, France; Service d'Endocrinologie (H.dB.), Centre Hospitalier de Chambéry, Chambéry, France; Département d'Endocrinologie (X.B.), Hôpital Cochin, Université Paris Descartes, Paris, France; Service de Gastroentérologie-Pancréatologie (P.R.), APHP, Hôpital Beaujon et Université Paris 7 Denis Diderot, Clichy, France; Fédération d'Endocrinologie (F.B-C.), Hospice Civils de Lyon et Université Lyon 1, Groupement Hospitalier Est. Lyon, France; Service d'Endocrinologie (B.V.), Diabète et Maladies Métaboliques, Centre Hospitalier Universitaire de Dijon, Hôpital du Bocage, Dijon, France; Département d'Endocrinologie (J.L.S.), Hopital de l'Archet, Nice, France; Service de Chirurgie Générale (F.M.), Viscérale et Endocrinienne, Groupement Hospitalier Universitaire Est, Hôpital de la Pitié, Paris, France; Service d'Endocrinologie (A.T.), Centre Hospitalier Universitaire, Hôpital du Haut Levêque, Pessac, France; Département d'Endocrinologie (J.M.K.), Hôpital Universitaire de Rouen. Rouen, France; Service d'Endocrinologie (P.dA.), Centre Hospitalier d'Avignon, Avignon, France; Service d'Endocrinologie (O.C.), Diabète et Maladies Métaboliques, Centre Hospitalier Universitaire de Grenoble, Hôpital Michalon, Grenoble, France; Service d'Endocrinologie (S.C-M.), Centre Hôpitalier Universitaire. Hôpital St-Antoine, Paris, France; Service d'Hép
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mesh_terms: D000236:Adenoma / Q000175:diagnosis / Q000453:epidemiology; D000293:Adolescent; D000310:Adrenal Gland Neoplasms / Q000175:diagnosis / Q000453:epidemiology; D000328:Adult; D017668:Age of Onset; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D005260:Female; D005602:France / Q000453:epidemiology; D006801:Humans; D007223:Infant; D007340:Insulinoma / Q000175:diagnosis / Q000453:epidemiology; D008297:Male; D018761:Multiple Endocrine Neoplasia Type 1 / Q000175:diagnosis / Q000453:epidemiology*; D018358:Neuroendocrine Tumors / Q000175:diagnosis / Q000453:epidemiology; D010190:Pancreatic Neoplasms / Q000175:diagnosis / Q000453:epidemiology; D010911:Pituitary Neoplasms / Q000175:diagnosis / Q000453:epidemiology; D055815:Young Adult