Metastatic Potential and Survival of Duodenal and Pancreatic Tumors in Multiple Endocrine Neoplasia Type 1

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Vinault, Sandrine | Mariet, Anne-Sophie | Le Bras, Maëlle | Mirallié, Eric | Cardot-Bauters, Catherine | Pattou, Francois | Ruszniewski, Philippe | Sauvanet, Alain | Chanson, Philippe | Baudin, Eric | Elias, Dominique | Menegaux, Fabrice | Gaujoux, Sebastien | Borson-Chazot, François | Lifante, Jean-Christophe | Caron, Philippe | Carrere, Nicolas | Tabarin, Antoine | Laurent, Christophe | Klein, Marc | Brunaud, Laurent | Niccoli, Patricia | Sebag, Frédéric | Cadiot, Guillaume | Kianmanesh, Reza | Luu, Maxime | Binquet, Christine | Goudet, Pierre

Edité par CCSD ; Lippincott, Williams & Wilkins -

IF 9.203. International audience. Objective: To assess the distant metastatic potential of duodeno-pancreatic neuroendocrine tumors (DP-NETs) in patients with MEN1, according to functional status and size.Summary Background Data: DP-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.Methods: The 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.Results: Among 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.Conclusions: DP-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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