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Long-term Follow-up of MEN1 Patients Who Do Not Have Initial Surgery for Small ≤2 cm Nonfunctioning Pancreatic Neuroendocrine Tumors, an AFCE and GTE Study

Frédéric Triponez 1 Samira Sadowski 1 Francois Pattou 2 Catherine Cardot-Bauters 3 Eric Mirallié 4 Maëlle Le Bras 5 Frédéric Sebag 6 Patricia Niccoli 7 Sophie Deguelte 8 Guillaume Cadiot 9 Gilles Poncet 10 Jean-Christophe Lifante 11, 10, 12 Françoise Borson-Chazot 13 Philippe Chaffanjon 14 Olivier Chabre 15 Fabrice Menegaux 16 Eric Baudin 17 Philippe Ruszniewski 18 Hélène Du Boullay 19 Pierre Goudet 20, 21, 22
17 Médecine nucléaire
Département d'imagerie médicale [Gustave Roussy]
Abstract : Objective: To report long-term follow-up of patients with multiple endocrine neoplasia type 1 (MEN1) and nonfunctioning pancreatic neuroendocrine tumors (NF-PET). Background: Pancreaticoduodenal tumors occur in almost all patients with MEN1 and are a major cause of death. The natural history and clinical outcome are poorly defined, and management is still controversial for small NF-PET. Methods: Clinical outcome and tumor progression were analyzed in 46 patients with MEN1 with 2 cm or smaller NF-PET who did not have surgery at the time of initial diagnosis. Survival data were analyzed using the Kaplan-Meier method. Results: Forty-six patients with MEN1 were followed prospectively for 10.7 ± 4.2 (mean ± standard deviation) years. One patient was lost to follow-up and 1 died from a cause unrelated to MEN1. Twenty-eight patients had stable disease and 16 showed significant progression of pancreaticoduodenal involvement, indicated by increase in size or number of tumors, development of a hypersecretion syndrome, need for surgery (7 patients), and death from metastatic NF-PET (1 patient). The mean event-free survival was 13.9 ± 1.1 years after NF-PET diagnosis. At last follow-up, none of the living patients who had undergone surgery or follow-up had evidence of metastases on imaging studies. Conclusions: Our study shows that conservative management for patients with MEN1 with NF-PET of 2 cm or smaller is associated with a low risk of disease-specific mortality. The decision to recommend surgery to prevent tumor spread should be balanced with operative mortality and morbidity, and patients should be informed about the risk-benefit ratio of conservative versus aggressive management when the NF-PET represents an intermediate risk.
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https://hal-univ-bourgogne.archives-ouvertes.fr/hal-01974901
Contributeur : Lnc - Université de Bourgogne <>
Soumis le : mercredi 9 janvier 2019 - 09:53:34
Dernière modification le : jeudi 27 août 2020 - 11:36:04

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Frédéric Triponez, Samira Sadowski, Francois Pattou, Catherine Cardot-Bauters, Eric Mirallié, et al.. Long-term Follow-up of MEN1 Patients Who Do Not Have Initial Surgery for Small ≤2 cm Nonfunctioning Pancreatic Neuroendocrine Tumors, an AFCE and GTE Study. Annals of Surgery, Lippincott, Williams & Wilkins, 2018, 268 (1), pp.158-164. ⟨10.1097/SLA.0000000000002191⟩. ⟨hal-01974901⟩

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