PMID- 22084155
OWN - NLM
STAT- MEDLINE
DCOM- 20120320
LR  - 20220318
IS  - 1479-683X (Electronic)
IS  - 0804-4643 (Linking)
VI  - 166
IP  - 2
DP  - 2012 Feb
TI  - Adrenal involvement in MEN1. Analysis of 715 cases from the Groupe d'etude des 
      Tumeurs Endocrines database.
PG  - 269-79
LID - 10.1530/EJE-11-0679 [doi]
AB  - OBJECTIVE: Limited data regarding adrenal involvement in multiple endocrine 
      neoplasia type 1 (MEN1) is available. We describe the characteristics of 
      MEN1-associated adrenal lesions in a large cohort to provide a rationale for 
      their management. METHODS: Analysis of records from 715 MEN1 patients from a 
      multicentre database between 1956 and 2008. Adrenal lesions were compared with 
      those from a multicentre cohort of 144 patients with adrenal sporadic 
      incidentalomas. RESULTS: Adrenal enlargement was reported in 20.4% (146/715) of 
      patients. Adrenal tumours (>10 mm in size) accounted for 58.1% of these cases 
      (10.1% of the whole patient cohort). Tumours were bilateral and >40 mm in size in 
      12.5 and 19.4% of cases respectively. Hormonal hypersecretion was restricted to 
      patients with tumours and occurred in 15.3% of them. Compared with 
      incidentalomas, MEN1-related tumours exhibited more cases of primary 
      hyperaldosteronism, fewer pheochromocytomas and more adrenocortical carcinomas 
      (ACCs; 13.8 vs 1.3%). Ten ACCs occurred in eight patients. Interestingly, ACCs 
      occurred after several years of follow-up of small adrenal tumours in two of the 
      eight affected patients. Nine of the ten ACCs were classified as stage I or II 
      according to the European Network for the Study of Adrenal Tumors. No evident 
      genotype/phenotype correlation was found for the occurrence of adrenal lesions, 
      endocrine hypersecretion or ACC. CONCLUSIONS: Adrenal pathology in MEN1 differs 
      from that observed in sporadic incidentalomas. In the absence of relevant 
      symptoms, endocrine biology can be restricted to patients with adrenal tumours 
      and should focus on steroid secretion including the aldosterone-renin system. 
      MEN1 is a high-risk condition for the occurrence of ACCs. It should be considered 
      regardless of the size of the tumour.
FAU - Gatta-Cherifi, B
AU  - Gatta-Cherifi B
AD  - Service d'Endocrinologie, Diabetologie et Maladies Metaboliques, Hopital Haut 
      Leveque, Centre Hospitalier Universitaire de Bordeaux, Avenue de Magellan, 33600 
      Pessac, France.
FAU - Chabre, O
AU  - Chabre O
FAU - Murat, A
AU  - Murat A
FAU - Niccoli, P
AU  - Niccoli P
FAU - Cardot-Bauters, C
AU  - Cardot-Bauters C
FAU - Rohmer, V
AU  - Rohmer V
FAU - Young, J
AU  - Young J
FAU - Delemer, B
AU  - Delemer B
FAU - Du Boullay, H
AU  - Du Boullay H
FAU - Verger, M F
AU  - Verger MF
FAU - Kuhn, J M
AU  - Kuhn JM
FAU - Sadoul, J L
AU  - Sadoul JL
FAU - Ruszniewski, Ph
AU  - Ruszniewski P
FAU - Beckers, A
AU  - Beckers A
FAU - Monsaingeon, M
AU  - Monsaingeon M
FAU - Baudin, E
AU  - Baudin E
FAU - Goudet, P
AU  - Goudet P
FAU - Tabarin, A
AU  - Tabarin A
LA  - eng
PT  - Journal Article
DEP - 20111114
PL  - England
TA  - Eur J Endocrinol
JT  - European journal of endocrinology
JID - 9423848
RN  - 0 (MEN1 protein, human)
RN  - 0 (Proto-Oncogene Proteins)
RN  - Adrenal incidentaloma
SB  - IM
MH  - Adolescent
MH  - Adrenal Gland Neoplasms/*epidemiology/genetics/pathology
MH  - Adult
MH  - Aged
MH  - Belgium/epidemiology
MH  - Case-Control Studies
MH  - Child
MH  - Child, Preschool
MH  - Cohort Studies
MH  - DNA Mutational Analysis
MH  - Databases as Topic/*statistics & numerical data
MH  - Female
MH  - France/epidemiology
MH  - Humans
MH  - Male
MH  - Middle Aged
MH  - *Multicenter Studies as Topic/statistics & numerical data
MH  - Multiple Endocrine Neoplasia Type 1/*epidemiology/genetics/pathology
MH  - Pheochromocytoma/*epidemiology/genetics/pathology
MH  - Proto-Oncogene Proteins/genetics
MH  - Tumor Burden
MH  - Young Adult
EDAT- 2011/11/16 06:00
MHDA- 2012/03/21 06:00
CRDT- 2011/11/16 06:00
PHST- 2011/11/16 06:00 [entrez]
PHST- 2011/11/16 06:00 [pubmed]
PHST- 2012/03/21 06:00 [medline]
AID - EJE-11-0679 [pii]
AID - 10.1530/EJE-11-0679 [doi]
PST - ppublish
SO  - Eur J Endocrinol. 2012 Feb;166(2):269-79. doi: 10.1530/EJE-11-0679. Epub 2011 Nov 
      14.