• Sandrine Vinault, Anne-Sophie Mariet, Maëlle Le Bras, Eric Mirallié, Catherine Cardot-Bauters, François Pattou, Philippe Ruszniewski, Alain Sauvanet, Philippe Chanson, Eric Baudin, Dominique Elias, Fabrice Menegaux, Sébastien Gaujoux, Françoise Borson-Chazot, Jean-Christophe Lifante, Philippe Caron, Nicolas Carrère, Antoine Tabarin, Christophe Laurent, Marc Klein, Laurent Brunaud, Patricia Niccoli, Frédéric Sebag, Guillaume Cadiot, Reza Kianmanesh, Maxime Luu, Christine Binquet, and Pierre Goudet.
• INSERM, CIC1432, Clinical Epidemiology Unit, Dijon, France.
• Ann. Surg. 2020 Dec 1; 272 (6): 1094-1101.

ObjectiveTo assess the distant metastatic potential of duodeno-pancreatic neuroendocrine tumors (DP-NETs) in patients with MEN1, according to functional status and size.Summary Background DataDP-NETs, with their numerous lesions and endocrine secretion-related symptoms, continue to be a medical challenge; unfortunately they can become aggressive tumors associated with distant metastasis, shortening survival. The survival of patients with large nonfunctional DP-NETs is known to be poor, but the overall contribution of DP-NETs to metastatic spread is poorly known.MethodsThe study population included patients with DP-NETs diagnosed after 1990 and followed in the MEN1 cohort of the Groupe d'étude des Tumeurs Endocrines (GTE). A multistate Markov piecewise constant intensities model was applied to separate the effects of prognostic factors on 1) metastasis, and 2) metastasis-free death or 3) death after appearance of metastases.ResultsAmong the 603 patients included, 39 had metastasis at diagnosis of DP-NET, 50 developed metastases during follow-up, and 69 died. The Markov model showed that Zollinger-Ellison-related tumors (regardless of tumor size and thymic tumor pejorative impact), large tumors over 2 cm, and age over 40 years were independently associated with an increased risk of metastases. Men, patients over 40 years old and patients with tumors larger than 2 cm, also had an increased risk of death once metastasis appeared.ConclusionsDP-NETs of 2 cm in size or more, regardless of the associated secretion, should be removed to prevent metastasis and increase survival. Surgery for gastrinoma remains debatable.

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