Life expectancy and likelihood of surgery in multiple endocrine neoplasia type 1: AFCE and GTE cohort study
Sébastien Gaujoux
(1)
,
Guillaume Martin
(2)
,
Eric Mirallié
(3)
,
Nicolas Regenet
(3)
,
Maëlle Le Bras
(3)
,
François Pattou
(4)
,
Bruno Carnaille
(4)
,
Catherine Cardot-Bauters
(4)
,
Lionel Groussin
(5)
,
Matthieu Faron
(6, 7)
,
Philippe Chanson
(8)
,
Haythem Najah
(9)
,
Antoine Tabarin
(10)
,
Alain Sauvanet
(11)
,
Philippe Ruszniewski
(11)
,
Jean Christophe Lifante
(12)
,
Thomas Walter
(13)
,
Nicolas Carrère
(14)
,
Philippe Caron
(15)
,
Sophie Deguelte
(16)
,
Brigitte Delemer
(16)
,
Christine Binquet
(17)
,
Anne Sophie Jannot
(2, 18, 19, 20)
,
Pierre Goudet
(21)
1
CHU Pitié-Salpêtrière [AP-HP]
2 HEGP - Hôpital Européen Georges Pompidou [APHP]
3 CHU Nantes - Centre Hospitalier Universitaire de Nantes = Nantes University Hospital
4 CHRU Lille - Centre Hospitalier Régional Universitaire [CHU Lille]
5 Hôpital Cochin [AP-HP]
6 IGR - Institut Gustave Roussy
7 Département de chirurgie viscérale [Gustave Roussy]
8 Hôpital Bicêtre [AP-HP, Le Kremlin-Bicêtre]
9 CHU Bordeaux - Centre Hospitalier Universitaire de Bordeaux
10 UB - Université de Bordeaux
11 Hôpital Beaujon [AP-HP]
12 CHLS - Centre Hospitalier Lyon Sud [CHU - HCL]
13 UCBL - Université Claude Bernard Lyon 1
14 UTPS - Université Toulouse III Paul Sabatier - Faculté de médecine Purpan
15 CHU Toulouse - Centre Hospitalier Universitaire de Toulouse
16 Hôpital Robert Debré
17 CIC-EC - Centre d'Investigation Clinique 1432 (Dijon) - Epidemiologie Clinique/Essais Cliniques
18 UPCité - Université Paris Cité
19 CRC (UMR_S_1138 / U1138) - Centre de Recherche des Cordeliers
20 HeKA - Health data- and model- driven Knowledge Acquisition
21 Service d'Hépato-Gastro-Entérologie (CHU de Dijon)
2 HEGP - Hôpital Européen Georges Pompidou [APHP]
3 CHU Nantes - Centre Hospitalier Universitaire de Nantes = Nantes University Hospital
4 CHRU Lille - Centre Hospitalier Régional Universitaire [CHU Lille]
5 Hôpital Cochin [AP-HP]
6 IGR - Institut Gustave Roussy
7 Département de chirurgie viscérale [Gustave Roussy]
8 Hôpital Bicêtre [AP-HP, Le Kremlin-Bicêtre]
9 CHU Bordeaux - Centre Hospitalier Universitaire de Bordeaux
10 UB - Université de Bordeaux
11 Hôpital Beaujon [AP-HP]
12 CHLS - Centre Hospitalier Lyon Sud [CHU - HCL]
13 UCBL - Université Claude Bernard Lyon 1
14 UTPS - Université Toulouse III Paul Sabatier - Faculté de médecine Purpan
15 CHU Toulouse - Centre Hospitalier Universitaire de Toulouse
16 Hôpital Robert Debré
17 CIC-EC - Centre d'Investigation Clinique 1432 (Dijon) - Epidemiologie Clinique/Essais Cliniques
18 UPCité - Université Paris Cité
19 CRC (UMR_S_1138 / U1138) - Centre de Recherche des Cordeliers
20 HeKA - Health data- and model- driven Knowledge Acquisition
21 Service d'Hépato-Gastro-Entérologie (CHU de Dijon)
Sébastien Gaujoux
- Fonction : Auteur
- PersonId : 1198046
- ORCID : 0000-0002-1072-7639
Eric Mirallié
- Fonction : Auteur
- PersonId : 1198047
- ORCID : 0000-0002-0806-4514
François Pattou
- Fonction : Auteur
- PersonId : 758667
- ORCID : 0000-0001-8388-3766
- IdRef : 074750364
Philippe Chanson
- Fonction : Auteur
- PersonId : 1329835
- IdHAL : philippe-chanson
- ORCID : 0000-0001-5096-5722
- IdRef : 029294452
Antoine Tabarin
- Fonction : Auteur
- PersonId : 1198048
- ORCID : 0000-0003-1231-3306
Thomas Walter
- Fonction : Auteur
- PersonId : 766421
- IdHAL : thomas-walter01
- ORCID : 0000-0002-4199-4561
Philippe Caron
- Fonction : Auteur
- PersonId : 1321942
- ORCID : 0000-0001-5391-7582
- IdRef : 056739737
Brigitte Delemer
- Fonction : Auteur
- PersonId : 1171436
- ORCID : 0000-0002-4320-2601
Résumé
Abstract Background
The overall natural history, risk of death and surgical burden of patients with multiple endocrine neoplasia type 1 (MEN1) is not well known. Methods Patients with MEN1 from a nationwide cohort were included. The survival of patients with MEN1 was compared with that of the general population using simulated controls. The cumulative probabilities of MEN1-specific operations and postoperative mortality were assessed, and surgical sequences were analysed using sunburst charts and Venn diagrams.
Results A total of 1386 patients with MEN1 were included. Life expectancy was significantly reduced in patients with MEN1 compared with simulated controls from the general population, with a lifetime difference of 15 years. Mutations affecting the JunD interaction domain had a significant negative impact on survival. Survival for patients with MEN1 compared with the general population improved over time. The probability of experiencing at least one specific MEN1 operation was above 95 per cent after 75 years, and most patients had surgery at least twice during their lifetime. Time to a 50 per cent risk of MEN1 surgery was 30.5 years for patients born after 1960, compared with 47.9 years for those born before 1960. Sex and mutations affecting the JunD interacting domain had no impact on time to first surgery. There was considerable heterogeneity in surgical sequences, with no specific clinical pathway.
Conclusion Life expectancy was significantly lower among patients with MEN1 compared with the general population, and further decreased in patients with mutations affecting the JunD interacting domain. Almost all patients underwent at least one MEN1-specific operation during their lifetime, but there was no standardized sequence of surgery.