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01-02-2010

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

Authors: 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

Published in: World Journal of Surgery | Issue 2/2010

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.

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Title: Risk Factors and Causes of Death in MEN1 Disease. A GTE (Groupe d’Etude des Tumeurs Endocrines) Cohort Study Among 758 Patients
Authors: 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
Publication date: 01-02-2010
Publisher: Springer-Verlag
Published in: World Journal of Surgery / Issue 2/2010
Print ISSN: 0364-2313
Electronic ISSN: 1432-2323
DOI: https://doi.org/10.1007/s00268-009-0290-1

Keynote webinar | Spotlight on medication adherence

Springer Medicine

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

Abstract

Background

Methods

Results

Conclusions

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Abstract

Background

Methods

Results

Conclusions

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