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 - Université de Bourgogne Accéder directement au contenu
Article Dans Une Revue Annals of Surgery Année : 2018

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

1 Service de chirurgie thoracique et endocrinienne (hôpitaux universitaires de Genève)
2 Département de chirurgie endocrinienne [Lille]
3 Service d’endocrinologie, diabétologie et maladies métaboliques [CHRU LIlle]
4 Service de Chirurgie Digestive et Endocrinienne [Nantes]
5 Service d'Endocrinologie [Nantes]
6 Endocrinologie, diabète, maladies métaboliques (CHU Marseille, AP-HM)
7 Institut Paoli-Calmettes
8 Service de chirurgie digestive et endocrinienne, et Laboratoire d’anatomie et cytologie pathologique, Hôpital Robert Debré, CHU de Reims
9 Service d'hépato-gastro-entérologie et cancérologie digestive [CHU de Reims]
10 Service de Chirurgie Générale et Endocrinienne [Lyon]
11 CHLS - Centre Hospitalier Lyon Sud [CHU - HCL]
12 HESPER - Health Service and Performance Research
13 Service d'endocrinologie, de diabétologie et des maladies métaboliques A (Hôpital Louis Pradel)
14 Département de chirurgie thoracique, vasculaire et endocrinienne
15 Service d'endocrinologie-diabétologie-nutrition [CHU Grenoble-Alpes]
16 Service de Chirurgie Digestive et Endocrinienne [CHU Pitié-Salpêtrière]
17 Médecine nucléaire
18 Service de Gastroentérologie [Hôpital Beaujon]
19 Service d'encrinologie (hôpital de Chambéry)
20 Service de Chirurgie Digestive, Cancérologique, Générale, Endocrinienne et Urgences (CHU de Dijon)
21 LNC - Lipides - Nutrition - Cancer [Dijon - U1231]
22 CIC-EC - Centre d'Investigation Clinique 1432 (Dijon) - Epidemiologie Clinique/Essais Cliniques
Eric Baudin

Résumé

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.

Dates et versions

hal-01974901 , version 1 (09-01-2019)

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Citer

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, 2018, 268 (1), pp.158-164. ⟨10.1097/SLA.0000000000002191⟩. ⟨hal-01974901⟩
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