PMID- 28263205
OWN - NLM
STAT- MEDLINE
DCOM- 20190903
LR  - 20190903
IS  - 1528-1140 (Electronic)
IS  - 0003-4932 (Print)
IS  - 0003-4932 (Linking)
VI  - 268
IP  - 1
DP  - 2018 Jul
TI  - 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.
PG  - 158-164
LID - 10.1097/SLA.0000000000002191 [doi]
AB  - 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.
FAU - Triponez, Frederic
AU  - Triponez F
AD  - Department of Thoracic and Endocrine Surgery, University Hospitals of Geneva and 
      Faculty of Medicine of Geneva, Geneva, Switzerland.
FAU - Sadowski, Samira M
AU  - Sadowski SM
AD  - Department of Thoracic and Endocrine Surgery, University Hospitals of Geneva and 
      Faculty of Medicine of Geneva, Geneva, Switzerland.
FAU - Pattou, Francois
AU  - Pattou F
AD  - Department of General and Endocrine Surgery, University Hospital of Lille, Lille, 
      France.
FAU - Cardot-Bauters, Catherine
AU  - Cardot-Bauters C
AD  - Department of Endocrinology, University Hospital of Lille, Lille, France.
FAU - Mirallie, Eric
AU  - Mirallie E
AD  - Department of Digestive and Endocrine Surgery, University Hospital of Nantes, 
      Nantes, France.
FAU - Le Bras, Maelle
AU  - Le Bras M
AD  - Department of Endocrinology, University Hospital of Nantes, Nantes, France.
FAU - Sebag, Frederic
AU  - Sebag F
AD  - Department of Endocrine Surgery, La Conception University Hospital, Marseille, 
      France.
FAU - Niccoli, Patricia
AU  - Niccoli P
AD  - Institut Paoli-Calmettes, Marseille, France.
FAU - Deguelte, Sophie
AU  - Deguelte S
AD  - Department of Digestive and Endocrine Surgery, University Hospital of Reims, 
      Reims, France.
FAU - Cadiot, Guillaume
AU  - Cadiot G
AD  - Department of Hepato-Gastroenterology and Digestive Oncology, University Hospital 
      of Reims, Reims, France.
FAU - Poncet, Gilles
AU  - Poncet G
AD  - Department of Surgery, University Hospital of Lyon, Lyon, France.
FAU - Lifante, Jean-Christophe
AU  - Lifante JC
AD  - 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.
FAU - Borson-Chazot, Francoise
AU  - Borson-Chazot F
AD  - Department of Endocrinology, Hospital Louis Pradel, University Lyon I, Lyon, 
      France.
FAU - Chaffanjon, Philippe
AU  - Chaffanjon P
AD  - Department of Thoracic, Vascular and Endocrine Surgery, University Hospital of 
      Grenoble, Grenoble, France.
FAU - Chabre, Olivier
AU  - Chabre O
AD  - Department of Endocrinology and Diabetology, University Hospital of Grenoble, 
      Grenoble, France.
FAU - Menegaux, Fabrice
AU  - Menegaux F
AD  - Department of Digestive and Endocrine Surgery, University Hospital La Pitie 
      Salpetriere, Paris, France.
FAU - Baudin, Eric
AU  - Baudin E
AD  - Department of Nuclear Medicine and Endocrine Oncology, Gustave Roussy, University 
      of Paris Sud, Villejuif, France.
FAU - Ruszniewski, Philippe
AU  - Ruszniewski P
AD  - Department of Gastroenterology and Pancreatology, Beaujon Hospital, University 
      Paris 7, Clichy, France.
FAU - Du Boullay, Helene
AU  - Du Boullay H
AD  - Department of Endocrinology, General Hospital of Chambery, Chambery, France.
FAU - Goudet, Pierre
AU  - Goudet P
AD  - 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.
LA  - eng
PT  - Clinical Trial
PT  - Journal Article
PT  - Multicenter Study
PL  - United States
TA  - Ann Surg
JT  - Annals of surgery
JID - 0372354
RN  - Non functioning pancreatic endocrine tumor
SB  - IM
MH  - Adult
MH  - Clinical Decision-Making
MH  - *Conservative Treatment
MH  - Female
MH  - Follow-Up Studies
MH  - Humans
MH  - Kaplan-Meier Estimate
MH  - Male
MH  - Middle Aged
MH  - Multiple Endocrine Neoplasia Type 1/mortality/*therapy
MH  - Pancreatic Neoplasms/mortality/*therapy
MH  - Prospective Studies
MH  - Treatment Outcome
PMC - PMC6012055
COIS- The authors report no conflicts of interest.
EDAT- 2017/03/07 06:00
MHDA- 2019/09/04 06:00
PMCR- 2018/06/21
CRDT- 2017/03/07 06:00
PHST- 2017/03/07 06:00 [pubmed]
PHST- 2019/09/04 06:00 [medline]
PHST- 2017/03/07 06:00 [entrez]
PHST- 2018/06/21 00:00 [pmc-release]
AID - ANNSURG-D-16-01708 [pii]
AID - 10.1097/SLA.0000000000002191 [doi]
PST - ppublish
SO  - Ann Surg. 2018 Jul;268(1):158-164. doi: 10.1097/SLA.0000000000002191.