title: 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: Association Francophone de Chirurgie Endocrinienne & Groupe d'Etude des Tumeurs Endocrines.
abstract: To report long-term follow-up of patients with multiple endocrine neoplasia type 1 (MEN1) and nonfunctioning pancreatic neuroendocrine tumors (NF-PET).
  
  
  
  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.
  
  
  
  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.
  
  
  
  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.
  
  
  
  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.
authors.affiliation: Department of Thoracic and Endocrine Surgery, University Hospitals of Geneva and Faculty of Medicine of Geneva, Geneva, Switzerland.
authors.forename: Frederic
authors.initials: F
authors.lastname: Triponez
authors.affiliation: Department of Thoracic and Endocrine Surgery, University Hospitals of Geneva and Faculty of Medicine of Geneva, Geneva, Switzerland.
authors.forename: Samira M
authors.initials: SM
authors.lastname: Sadowski
authors.affiliation: Department of General and Endocrine Surgery, University Hospital of Lille, Lille, France.
authors.forename: François
authors.initials: F
authors.lastname: Pattou
authors.affiliation: Department of Endocrinology, University Hospital of Lille, Lille, France.
authors.forename: Catherine
authors.initials: C
authors.lastname: Cardot-Bauters
authors.affiliation: Department of Digestive and Endocrine Surgery, University Hospital of Nantes, Nantes, France.
authors.forename: Eric
authors.initials: E
authors.lastname: Mirallié
authors.affiliation: Department of Endocrinology, University Hospital of Nantes, Nantes, France.
authors.forename: Maëlle
authors.initials: M
authors.lastname: Le Bras
authors.affiliation: Department of Endocrine Surgery, La Conception University Hospital, Marseille, France.
authors.forename: Frédéric
authors.initials: F
authors.lastname: Sebag
authors.affiliation: Institut Paoli-Calmettes, Marseille, France.
authors.forename: Patricia
authors.initials: P
authors.lastname: Niccoli
authors.affiliation: Department of Digestive and Endocrine Surgery, University Hospital of Reims, Reims, France.
authors.forename: Sophie
authors.initials: S
authors.lastname: Deguelte
authors.affiliation: Department of Hepato-Gastroenterology and Digestive Oncology, University Hospital of Reims, Reims, France.
authors.forename: Guillaume
authors.initials: G
authors.lastname: Cadiot
authors.affiliation: Department of Surgery, University Hospital of Lyon, Lyon, France.
authors.forename: Gilles
authors.initials: G
authors.lastname: Poncet
authors.affiliation: Department of Digestive and Endocrine Surgery, University Hospital of Lyon Sud and 2/EA 7425 HESPER, Health Services and Performance Research, University Claude Bernard Lyon 1, Lyon, France.
authors.forename: Jean-Christophe
authors.initials: JC
authors.lastname: Lifante
authors.affiliation: Department of Endocrinology, Hospital Louis Pradel, University Lyon I, Lyon, France.
authors.forename: Françoise
authors.initials: F
authors.lastname: Borson-Chazot
authors.affiliation: Department of Thoracic, Vascular and Endocrine Surgery, University Hospital of Grenoble, Grenoble, France.
authors.forename: Philippe
authors.initials: P
authors.lastname: Chaffanjon
authors.affiliation: Department of Endocrinology and Diabetology, University Hospital of Grenoble, Grenoble, France.
authors.forename: Olivier
authors.initials: O
authors.lastname: Chabre
authors.affiliation: Department of Digestive and Endocrine Surgery, University Hospital La Pitié Salpétrière, Paris, France.
authors.forename: Fabrice
authors.initials: F
authors.lastname: Menegaux
authors.affiliation: Department of Nuclear Medicine and Endocrine Oncology, Gustave Roussy, University of Paris Sud, Villejuif, France.
authors.forename: Eric
authors.initials: E
authors.lastname: Baudin
authors.affiliation: Department of Gastroenterology and Pancreatology, Beaujon Hospital, University Paris 7, Clichy, France.
authors.forename: Philippe
authors.initials: P
authors.lastname: Ruszniewski
authors.affiliation: Department of Endocrinology, General Hospital of Chambéry, Chambéry, France.
authors.forename: Hélène
authors.initials: H
authors.lastname: Du Boullay
authors.affiliation: Department of Endocrine Surgery, University Hospital of Dijon, and INSERM, U866, Epidemiology and Clinical Research in Digestive Oncology Team, and INSERM, CIC1432, Clinical Epidemiology Unit, University Hospital of Dijon, Clinical Investigation Center, Clinical Epidemiology/Clinical Trials Unit, Dijon, France.
authors.forename: Pierre
authors.initials: P
authors.lastname: Goudet
mesh_terms: D000328:Adult; D000066491:Clinical Decision-Making; D000072700:Conservative Treatment; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D053208:Kaplan-Meier Estimate; D008297:Male; D008875:Middle Aged; D018761:Multiple Endocrine Neoplasia Type 1 / Q000401:mortality / Q000628:therapy*; D010190:Pancreatic Neoplasms / Q000401:mortality / Q000628:therapy*; D011446:Prospective Studies; D016896:Treatment Outcome