Risk factors and causes of death in MEN1 disease. A GTE (Groupe d'Etude des Tumeurs Endocrines) cohort study among 758 patients.
Fiche publication
Date publication
février 2010
Auteurs
Membres identifiés du Cancéropôle Est :
Pr BINQUET Christine
Tous les auteurs :
Goudet P, Murat A, Binquet C, Cardot-Bauters C, Costa A, Ruszniewski P, Niccoli P, Menegaux F, Chabrier G, Borson-Chazot F, Tabarin A, Bouchard P, Delemer B, Beckers A, Bonithon-Kopp C, Ajzenberg C, Altman JJ, Archambeaud F, Attali JR, Badet C, Barbier J, Barthet M, Baudin E, Bauduceau B, Bernades P, Bertagna X, Bertherat J, Boissel P, Bouchard P, Boyaval JM, Bresler L, Bretagne JF, Bringer J, Brunaud L, Burger J, Carenco P, Caron P, Cathebras B, Celerier M, Chabre O, Chabrier G, Chadenas D, Chanson P, Charitanski D, Chayvialle JA, Montiel CC, Comas JM, Devolx BC, Cortot A, Cosson E, Cougard P, Cubertafond P, D'Anella P, Darsy P, Defechereux T, Delecourt F, Denis J, Derrien C, Dewailly D, du Boullay-Choplin H, Dramais AS, Droumaguet C, Dubost C, Duron F, Emperauger-Beauvais B, Emy P, Gauthier S, Roqueplo AP, Goichot B, Goldfain D, Gosselin M, Guilhem I, Guillausseau PJ, Hamon P, Henry JF, Jaquet PJ, Kerlan V, Khun JM, Knebelmann B, Kraimps JL, Krivtzky A, Lalau JD, Lecomte P, Legros JJ, Levoir D, Maizeray-Cailliau B, Malet D, Malinski M, Mantion G, Mathe C, Mathonnet M, Melliere D, Metman EH, Meurisse M, Modigliani R, Monsaigeon M, Naouri C, Oliver C, Olivier F, Orgiazzi J, Parneix M, Partenski C, Peix JL, Penfornis A, Pradignac A, Pouget C, Pugeat M, Raffin-Sanson ML, Rodier M, Roger P, Rohmer V, Rougier P, Rousset H, Roy J, Sadoul JL, Sarfati E, Schlienger JL, Schlumberger M, Seve P, Simon D, Soubrane O, Soule JC, Thieblot P, Thivolet C, Thomopoulos P, Turpin G, Valensi P, Vantighem MC, Verger MF, Verges B, Verier-Mine O, Verlet E, Vialettes B, Viard R, Walter S, Warnet A, Wechsler B, Wemeau JL, Weryha G, Woehl-Kremer B
Lien Pubmed
Résumé
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.
Référence
World J Surg. 2010 Feb;34(2):249-55.