PMID- 19949948
OWN - NLM
STAT- MEDLINE
DCOM- 20100518
LR  - 20220409
IS  - 1432-2323 (Electronic)
IS  - 0364-2313 (Linking)
VI  - 34
IP  - 2
DP  - 2010 Feb
TI  - Risk factors and causes of death in MEN1 disease. A GTE (Groupe d'Etude des 
      Tumeurs Endocrines) cohort study among 758 patients.
PG  - 249-55
LID - 10.1007/s00268-009-0290-1 [doi]
AB  - BACKGROUND: The natural history of multiple endocrine neoplasia type 1 (MEN1) is 
      known through single-institution or single-family studies. We aimed to analyze 
      the risk factors and causes of death in a large cohort of MEN1 patients. METHODS: 
      Overall, 758 symptomatic MEN1 patients were identified through the GTE network 
      (Groupe d'etude des Tumeurs Endocrines), which involves French and Belgian 
      genetics laboratories responsible for MEN1 diagnosis and 80 clinical reference 
      centers. The causes of death were analyzed. A frailty model, including 
      time-dependent variables, was used to assess the impact of each clinical lesion, 
      except for hyperparathyroidism, on survival. RESULTS: The median follow-up was 
      6.3 years. Female gender, family history of MEN1, and recent diagnosis were 
      associated with a lower risk of death. Compared with nonaffected patients, those 
      with thymic tumors (hazard ratio [HR] = 4.64, 95% CI = 1.73-12.41), 
      glucagonomas-vipomas-somatostatinomas (HR = 4.29, 95% CI = 1.54-11.93), 
      nonfunctioning pancreatic tumors (HR = 3.43, 95% CI = 1.71-6.88), and gastrinoma 
      (HR = 1.89, 95% CI = 1.09-3.25) had a higher risk of death after adjustment for 
      age, gender, and diagnosis period. The increased risk of death among patients 
      with adrenal tumors was not significant, but three patients died from aggressive 
      adrenal tumors. Pituitary tumors, insulinomas, and bronchial tumors did not 
      increase the risk of death. The proportion of MEN1-related deaths decreased from 
      76.8 to 71.4% after 1990. CONCLUSIONS: The prognosis of MEN1 disease has improved 
      since 1980. Thymic tumors and duodenopancreatic tumors, including nonsecreting 
      pancreatic tumors, increased the risk of death. Rare but aggressive adrenal 
      tumors may also cause death. Most deaths were related to MEN1. New 
      recommendations on abdominal and thoracic imaging are required.
FAU - Goudet, Pierre
AU  - Goudet P
AD  - Service de Chirurgie Endocrinienne, Centre Hospitalier Universitaire, Dijon, 
      France. pierre.goudet@chu-dijon.fr
FAU - Murat, Arnaud
AU  - Murat A
FAU - Binquet, Christine
AU  - Binquet C
FAU - Cardot-Bauters, Christine
AU  - Cardot-Bauters C
FAU - Costa, Annie
AU  - Costa A
FAU - Ruszniewski, Philippe
AU  - Ruszniewski P
FAU - Niccoli, Patricia
AU  - Niccoli P
FAU - Menegaux, Fabrice
AU  - Menegaux F
FAU - Chabrier, Georges
AU  - Chabrier G
FAU - Borson-Chazot, Francoise
AU  - Borson-Chazot F
FAU - Tabarin, Antoine
AU  - Tabarin A
FAU - Bouchard, Philippe
AU  - Bouchard P
FAU - Delemer, Brigitte
AU  - Delemer B
FAU - Beckers, Alfred
AU  - Beckers A
FAU - Bonithon-Kopp, Claire
AU  - Bonithon-Kopp C
LA  - eng
PT  - Journal Article
PT  - Multicenter Study
PL  - United States
TA  - World J Surg
JT  - World journal of surgery
JID - 7704052
SB  - IM
MH  - Adult
MH  - Belgium/epidemiology
MH  - Cause of Death
MH  - Chi-Square Distribution
MH  - Diagnostic Imaging
MH  - Female
MH  - Follow-Up Studies
MH  - France/epidemiology
MH  - Humans
MH  - Male
MH  - Middle Aged
MH  - Multiple Endocrine Neoplasia Type 1/diagnosis/*mortality
MH  - Prognosis
MH  - Proportional Hazards Models
MH  - Risk Factors
MH  - Survival Analysis
EDAT- 2009/12/02 06:00
MHDA- 2010/05/19 06:00
CRDT- 2009/12/02 06:00
PHST- 2009/12/02 06:00 [entrez]
PHST- 2009/12/02 06:00 [pubmed]
PHST- 2010/05/19 06:00 [medline]
AID - 10.1007/s00268-009-0290-1 [doi]
PST - ppublish
SO  - World J Surg. 2010 Feb;34(2):249-55. doi: 10.1007/s00268-009-0290-1.