PMID- 25594862
OWN - NLM
STAT- MEDLINE
DCOM- 20150806
LR  - 20220331
IS  - 1945-7197 (Electronic)
IS  - 0021-972X (Linking)
VI  - 100
IP  - 4
DP  - 2015 Apr
TI  - MEN1 disease occurring before 21 years old: a 160-patient cohort study from the 
      Groupe d'etude des Tumeurs Endocrines.
PG  - 1568-77
LID - 10.1210/jc.2014-3659 [doi]
AB  - CONTEXT: 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'etude 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.
FAU - Goudet, P
AU  - Goudet P
AD  - 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 Metaboliques (A.D., N.L-B.), Centre 
      Hospitalier Universitaire, Hopital Robert Debre, Reims, France, Clinique 
      d'Endocrinologie (M.L.), Centre Hospitalier Universitaire, Nantes, France; 
      Service de Medecine Interne et Endocrinologie (C.C-B.), Clinique Marc Linquette, 
      Centre Hospitalier Regional et Universitaire, Lille, France; Service d'Oncologie 
      Medicale (P.N.), Institut Paoli-Calmettes, APHM, Universite Aix-Marseille, 
      Marseille, France; Service d'Endocrinologie (H.dB.), Centre Hospitalier de 
      Chambery, Chambery, France; Departement d'Endocrinologie (X.B.), Hopital Cochin, 
      Universite Paris Descartes, Paris, France; Service de 
      Gastroenterologie-Pancreatologie (P.R.), APHP, Hopital Beaujon et Universite 
      Paris 7 Denis Diderot, Clichy, France; Federation d'Endocrinologie (F.B-C.), 
      Hospice Civils de Lyon et Universite Lyon 1, Groupement Hospitalier Est. Lyon, 
      France; Service d'Endocrinologie (B.V.), Diabete et Maladies Metaboliques, Centre 
      Hospitalier Universitaire de Dijon, Hopital du Bocage, Dijon, France; Departement 
      d'Endocrinologie (J.L.S.), Hopital de l'Archet, Nice, France; Service de 
      Chirurgie Generale (F.M.), Viscerale et Endocrinienne, Groupement Hospitalier 
      Universitaire Est, Hopital de la Pitie, Paris, France; Service d'Endocrinologie 
      (A.T.), Centre Hospitalier Universitaire, Hopital du Haut Leveque, Pessac, 
      France; Departement d'Endocrinologie (J.M.K.), Hopital Universitaire de Rouen. 
      Rouen, France; Service d'Endocrinologie (P.dA.), Centre Hospitalier d'Avignon, 
      Avignon, France; Service d'Endocrinologie (O.C.), Diabete et Maladies 
      Metaboliques, Centre Hospitalier Universitaire de Grenoble, Hopital Michalon, 
      Grenoble, France; Service d'Endocrinologie (S.C-M.), Centre Hopitalier 
      Universitaire. Hopital St-Antoine, Paris, France; Service d'Hep
FAU - Dalac, A
AU  - Dalac A
FAU - Le Bras, M
AU  - Le Bras M
FAU - Cardot-Bauters, C
AU  - Cardot-Bauters C
FAU - Niccoli, P
AU  - Niccoli P
FAU - Levy-Bohbot, N
AU  - Levy-Bohbot N
FAU - du Boullay, H
AU  - du Boullay H
FAU - Bertagna, X
AU  - Bertagna X
FAU - Ruszniewski, P
AU  - Ruszniewski P
FAU - Borson-Chazot, F
AU  - Borson-Chazot F
FAU - Verges, B
AU  - Verges B
FAU - Sadoul, J L
AU  - Sadoul JL
FAU - Menegaux, F
AU  - Menegaux F
FAU - Tabarin, A
AU  - Tabarin A
FAU - Kuhn, J M
AU  - Kuhn JM
FAU - d'Anella, P
AU  - d'Anella P
FAU - Chabre, O
AU  - Chabre O
FAU - Christin-Maitre, S
AU  - Christin-Maitre S
FAU - Cadiot, G
AU  - Cadiot G
FAU - Binquet, C
AU  - Binquet C
FAU - Delemer, B
AU  - Delemer B
LA  - eng
PT  - Journal Article
DEP - 20150116
PL  - United States
TA  - J Clin Endocrinol Metab
JT  - The Journal of clinical endocrinology and metabolism
JID - 0375362
SB  - IM
MH  - Adenoma/diagnosis/epidemiology
MH  - Adolescent
MH  - Adrenal Gland Neoplasms/diagnosis/epidemiology
MH  - Adult
MH  - Age of Onset
MH  - Child
MH  - Child, Preschool
MH  - Cohort Studies
MH  - Female
MH  - France/epidemiology
MH  - Humans
MH  - Infant
MH  - Insulinoma/diagnosis/epidemiology
MH  - Male
MH  - Multiple Endocrine Neoplasia Type 1/diagnosis/*epidemiology
MH  - Neuroendocrine Tumors/diagnosis/epidemiology
MH  - Pancreatic Neoplasms/diagnosis/epidemiology
MH  - Pituitary Neoplasms/diagnosis/epidemiology
MH  - Young Adult
EDAT- 2015/01/17 06:00
MHDA- 2015/08/08 06:00
CRDT- 2015/01/17 06:00
PHST- 2015/01/17 06:00 [entrez]
PHST- 2015/01/17 06:00 [pubmed]
PHST- 2015/08/08 06:00 [medline]
AID - 10.1210/jc.2014-3659 [doi]
PST - ppublish
SO  - J Clin Endocrinol Metab. 2015 Apr;100(4):1568-77. doi: 10.1210/jc.2014-3659. Epub 
      2015 Jan 16.