PMID- 35833229
OWN - NLM
STAT- MEDLINE
DCOM- 20220818
LR  - 20220901
IS  - 1365-2168 (Electronic)
IS  - 0007-1323 (Linking)
VI  - 109
IP  - 9
DP  - 2022 Aug 16
TI  - Life expectancy and likelihood of surgery in multiple endocrine neoplasia type 1: 
      AFCE and GTE cohort study.
PG  - 872-879
LID - 10.1093/bjs/znac006 [doi]
AB  - 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.
CI  - (c) The Author(s) 2022. Published by Oxford University Press on behalf of BJS 
      Society Ltd. All rights reserved. For permissions, please e-mail: 
      journals.permissions@oup.com.
FAU - Gaujoux, Sebastien
AU  - Gaujoux S
AUID- ORCID: 0000-0002-1072-7639
AD  - Department of General, Visceral, and Endocrine Surgery, Pitie Salpetriere 
      Hospital, AP-HP, Paris, France.
AD  - Department of Hepato-Biliary and Pancreatic Surgery and Liver Transplantation, 
      AP-HP Pitie-Salpetriere Hospital, Paris, France.
AD  - Sorbonne Universite, Paris, France.
FAU - Martin, Guillaume L
AU  - Martin GL
AD  - Departement d'Informatique Medicale, Biostatistiques et Sante Publique, Hopital 
      Europeen Georges Pompidou, and Assistance Publique-Hopitaux de Paris, Paris, 
      France.
AD  - Centre de Recherche des Cordeliers, INSERM UMRS1138, Universite de Paris, Paris, 
      France.
FAU - Mirallie, Eric
AU  - Mirallie E
AUID- ORCID: 0000-0002-0806-4514
AD  - Department of Digestive and Endocrine Surgery, Nantes University Hospital, 
      Nantes, France.
FAU - Regenet, Nicolas
AU  - Regenet N
AD  - Department of Digestive and Endocrine Surgery, Nantes University Hospital, 
      Nantes, France.
FAU - Le Bras, Maelle
AU  - Le Bras M
AD  - Department of Endocrinology, Nantes University Hospital, Nantes, France.
FAU - Pattou, Francois
AU  - Pattou F
AD  - Department of General and Endocrine Surgery, Lille University Hospital, Lille, 
      France.
FAU - Carnaille, Bruno
AU  - Carnaille B
AD  - Department of General and Endocrine Surgery, Lille University Hospital, Lille, 
      France.
FAU - Cardot-Bauters, Catherine
AU  - Cardot-Bauters C
AD  - Department of Endocrinology, Lille University Hospital, Lille, France.
FAU - Groussin, Lionel
AU  - Groussin L
AD  - Endocrinology, Cochin, Universite de Paris, Paris, France.
FAU - Faron, Matthieu
AU  - Faron M
AD  - Digestive and General Surgery, IGR, Universite de Paris, Paris, France.
FAU - Chanson, Philippe
AU  - Chanson P
AUID- ORCID: 0000-0001-5096-5722
AD  - Assistance Publique-Hopitaux de Paris, Hopital Bicetre, Service d'Endocrinologie 
      et des Maladies de la Reproduction, Centre de Reference des Maladies Rares de 
      l'Hypophyse, and Universite Paris-Saclay, Universite Paris-Sud, INSERM, 
      Signalisation Hormonale, Physiopathologie Endocrinienne et Metabolique, Le 
      Kremlin-Bicetre, France.
FAU - Najah, Haythem
AU  - Najah H
AD  - Department of Digestive and Endocrine Surgery, CHU Bordeaux, Bordeaux, France.
FAU - Tabarin, Antoine
AU  - Tabarin A
AUID- ORCID: 0000-0003-1231-3306
AD  - Endocrinology, Bordeaux University, Bordeaux, France.
FAU - Sauvanet, Alain
AU  - Sauvanet A
AD  - Department of Hepato-Pancreato-Biliary Surgery, Beaujon Hospital, Assistance 
      Publique-Hopitaux de Paris, Universite de Paris, Paris, France.
FAU - Ruszniewski, Philippe
AU  - Ruszniewski P
AD  - Department of Pancreatology, Beaujon Hospital, Assistance Publique-Hopitaux de 
      Paris, Universite de Paris, Paris, France.
FAU - Lifante, Jean Christophe
AU  - Lifante JC
AD  - Digestive and Endocrine Surgery, Lyon Sud Hospital, Claude Bernard Lyon 1 
      University, Lyon, France.
FAU - Walter, Thomas
AU  - Walter T
AD  - Department of Oncology, University Claude Bernard Lyon 1, Lyon, France.
FAU - Carrere, Nicolas
AU  - Carrere N
AD  - Department of Digestive Surgery, Toulouse University, Toulouse, France.
FAU - Caron, Philippe
AU  - Caron P
AD  - Department of Endocrinology, Hopital Larrey, CHU Toulouse, Toulouse, France.
FAU - Deguelte, Sophie
AU  - Deguelte S
AD  - Department of General and Digestive Surgery, Robert-Debre Hospital, 
      Reims-Champagne-Ardennes University, Reims, France.
FAU - Delemer, Brigitte
AU  - Delemer B
AUID- ORCID: 0000-0002-4320-2601
AD  - Department of Endocrinology, Robert-Debre Hospital, Reims-Champagne-Ardennes 
      University, Reims, France.
FAU - Binquet, Christine
AU  - Binquet C
AD  - INSERM, CIC1432, Clinical Epidemiology Unit, Dijon, France.
AD  - Dijon-Bourgogne University Hospital, Clinical Investigation Centre, Clinical 
      Epidemiology/Clinical Trials Unit, Dijon, France.
FAU - Jannot, Anne Sophie
AU  - Jannot AS
AD  - Departement d'Informatique Medicale, Biostatistiques et Sante Publique, Hopital 
      Europeen Georges Pompidou, and Assistance Publique-Hopitaux de Paris, Paris, 
      France.
AD  - Centre de Recherche des Cordeliers, INSERM UMRS1138, Universite de Paris, Paris, 
      France.
FAU - Goudet, Pierre
AU  - Goudet P
AD  - INSERM, U1231, Epidemiology and Clinical Research in Digestive Cancers Team, 
      Dijon, France.
AD  - Burgundy-Franche-Comte University, UMR1231, Epidemiology and Clinical Research in 
      Digestive Oncology Team, Dijon, France.
AD  - Department of Digestive and Endocrine Surgery, Dijon University Hospital, Dijon, 
      France.
LA  - eng
PT  - Journal Article
PL  - England
TA  - Br J Surg
JT  - The British journal of surgery
JID - 0372553
SB  - IM
MH  - Cohort Studies
MH  - Humans
MH  - Life Expectancy
MH  - *Multiple Endocrine Neoplasia Type 1/genetics/surgery
MH  - Mutation
MH  - *Pancreatic Neoplasms/surgery
MH  - Probability
EDAT- 2022/07/15 06:00
MHDA- 2022/08/19 06:00
CRDT- 2022/07/14 02:51
PHST- 2021/08/06 00:00 [received]
PHST- 2021/11/21 00:00 [revised]
PHST- 2021/12/26 00:00 [accepted]
PHST- 2022/07/15 06:00 [pubmed]
PHST- 2022/08/19 06:00 [medline]
PHST- 2022/07/14 02:51 [entrez]
AID - 6643541 [pii]
AID - 10.1093/bjs/znac006 [doi]
PST - ppublish
SO  - Br J Surg. 2022 Aug 16;109(9):872-879. doi: 10.1093/bjs/znac006.