• Sébastien Gaujoux, Guillaume L Martin, Eric Mirallié, Nicolas Regenet, Maëlle Le Bras, François Pattou, Bruno Carnaille, Catherine Cardot-Bauters, Lionel Groussin, Matthieu Faron, Philippe Chanson, Haythem Najah, Antoine Tabarin, Alain Sauvanet, Philippe Ruszniewski, Jean Christophe Lifante, Thomas Walter, Nicolas Carrère, Philippe Caron, Sophie Deguelte, Brigitte Delemer, Christine Binquet, Anne Sophie Jannot, and Pierre Goudet.
• Department of General, Visceral, and Endocrine Surgery, Pitié Salpêtrière Hospital, AP-HP, Paris, France.
• Br J Surg. 2022 Aug 16; 109 (9): 872-879.

BackgroundThe overall natural history, risk of death and surgical burden of patients with multiple endocrine neoplasia type 1 (MEN1) is not well known.MethodsPatients 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.ResultsA 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.ConclusionLife 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.© 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.

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