World Journal of Surgery

, Volume 34, Issue 2, pp 249–255 | Cite as

Risk Factors and Causes of Death in MEN1 Disease. A GTE (Groupe d’Etude des Tumeurs Endocrines) Cohort Study Among 758 Patients

  • Pierre Goudet
  • Arnaud Murat
  • Christine Binquet
  • Christine Cardot-Bauters
  • Annie Costa
  • Philippe Ruszniewski
  • Patricia Niccoli
  • Fabrice Ménégaux
  • Georges Chabrier
  • Françoise Borson-Chazot
  • Antoine Tabarin
  • Philippe Bouchard
  • Brigitte Delemer
  • Alfred Beckers
  • Claire Bonithon-Kopp
Article

Abstract

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’étude 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.

Notes

Acknowledgments

We are grateful to Mr. Philip Bastable for help with translation of the manuscript into English. We also acknowledge the coauthors: C. Ajzenberg, J.J. Altman, F. Archambeaud, J.R. Attali, C. Badet, J. Barbier, M. Barthet, E. Baudin, B. Bauduceau, P. Bernades, X. Bertagna, J. Bertherat, P. Boissel, P. Bouchard, J.M. Boyaval, L. Bresler, J.F. Bretagne, J. Bringer, L. Brunaud, J. Burger, P. Carenco, P. Caron, B. Cathebras, M. Celerier, O. Chabre, G. Chabrier, D. Chadenas, P. Chanson, D. Charitanski, J.A. Chayvialle, C. Colmar Montiel, J.M. Comas, B. Conte Devolx, A. Cortot, E. Cosson, P. Cougard, P. Cubertafond, P. D’Anella, P. Darsy, T. Defechereux, F. Delecourt, J. Denis, C. Derrien, D. Dewailly, H. du Boullay-Choplin, A.S. Dramais, C. Droumaguet, C. Dubost, F. Duron, B. Emperauger-Beauvais, P. Emy, S. Gauthier, A.P. Gimenez Roqueplo, B. Goichot, D. Goldfain, M. Gosselin, I. Guilhem, P.J. Guillausseau, P. Hamon, J.F. Henry, P.J. Jaquet, V. Kerlan, J.M. Khun, B. Knebelmann, J.L. Kraimps, A. Krivtzky, J.D. Lalau, P. Lecomte, J.J. Legros, D. Levoir, B. Maizeray-Cailliau, D. Malet, M. Malinski, G. Mantion, C. Mathe, M. Mathonnet, D. Melliere, E.H. Metman, M. Meurisse, R. Modigliani, M. Monsaigeon, C. Naouri, C. Oliver, F. Olivier, J. Orgiazzi, M. Parneix, C. Partenski, J.L. Peix, A. Penfornis, A. Pradignac, C. Pouget, M. Pugeat, M.L. Raffin-Sanson, M. Rodier, P. Roger, V. Rohmer, P. Rougier, H. Rousset, J. Roy, J.L. Sadoul, E. Sarfati, J.L. Schlienger, M. Schlumberger, P. Seve, D. Simon, O. Soubrane, J.C. Soule, P. Thieblot, C. Thivolet, P. Thomopoulos, G. Turpin, P. Valensi, M.C. Vantighem, M.F. Verger, B. Verges, O. Verier-Mine, E. Verlet, B. Vialettes, R. Viard, S. Walter, A. Warnet, B. Wechsler, J.L. Wemeau, G. Weryha, B. Woehl-Kremer.

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Copyright information

© Société Internationale de Chirurgie 2009

Authors and Affiliations

  • Pierre Goudet
    • 1
    • 2
  • Arnaud Murat
    • 3
  • Christine Binquet
    • 4
  • Christine Cardot-Bauters
    • 5
  • Annie Costa
    • 4
  • Philippe Ruszniewski
    • 6
  • Patricia Niccoli
    • 7
  • Fabrice Ménégaux
    • 8
  • Georges Chabrier
    • 9
  • Françoise Borson-Chazot
    • 10
  • Antoine Tabarin
    • 11
  • Philippe Bouchard
    • 12
  • Brigitte Delemer
    • 13
  • Alfred Beckers
    • 14
  • Claire Bonithon-Kopp
    • 4
  1. 1.Service de Chirurgie EndocrinienneCentre Hospitalier UniversitaireDijonFrance
  2. 2.Faculté de médecine de DijonCentre d’Epidemiologie des Populations, Université de BourgogneDijonFrance
  3. 3.Clinique d’EndocrinologieCentre Hospitalier UniversitaireNantesFrance
  4. 4.INSERM CIE1, Centre d’Investigation clinique-Epidémiologie cliniqueCentre Hospitalier UniversitaireDijonFrance
  5. 5.Service de Médecine interne et Endocrinologie, Clinique Marc LinquetteCentre Hospitalier Régional et UniversitaireLilleFrance
  6. 6.Service de Gastro-entérologieGroupement Hospitalier Universitaire Nord, Hôpital BeaujonClichyFrance
  7. 7.Service d’Endocrinologie, Diabète et Maladies métaboliquesCentre Hospitalier Universitaire La TimoneMarseilleFrance
  8. 8.Service de Chirurgie Générale, Viscérale et EndocrinienneGroupement Hospitalier Universitaire Est, Hôpital de la PitiéParisFrance
  9. 9.Service de Médecine interne et NutritionCentre Hospitalier Universitaire, Hôpital de HautepierreStrasbourgFrance
  10. 10.Service d’EndocrinologieCentre Hospitalier Universitaire Est, Hôpital WertheimerLyonFrance
  11. 11.Service d’EndocrinologieCentre Hospitalier Universitaire, Hôpital du Haut LevêquePessacFrance
  12. 12.Service d’EndocrinologieGroupement Hospitalier Universitaire EstParisFrance
  13. 13.Service d’EndocrinologieCentre Hospitalier Universitaire, Hôpital Robert DebréReimsFrance
  14. 14.Service d’Endocrinologie CliniqueCentre Hospitalier Universitaire de LiègeLiègeBelgium

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