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Ann Surg. 2018 Jul;268(1):158-164. doi: 10.1097/SLA.0000000000002191.

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.

Author information

1
Department of Thoracic and Endocrine Surgery, University Hospitals of Geneva and Faculty of Medicine of Geneva, Geneva, Switzerland.
2
Department of General and Endocrine Surgery, University Hospital of Lille, Lille, France.
3
Department of Endocrinology, University Hospital of Lille, Lille, France.
4
Department of Digestive and Endocrine Surgery, University Hospital of Nantes, Nantes, France.
5
Department of Endocrinology, University Hospital of Nantes, Nantes, France.
6
Department of Endocrine Surgery, La Conception University Hospital, Marseille, France.
7
Institut Paoli-Calmettes, Marseille, France.
8
Department of Digestive and Endocrine Surgery, University Hospital of Reims, Reims, France.
9
Department of Hepato-Gastroenterology and Digestive Oncology, University Hospital of Reims, Reims, France.
10
Department of Surgery, University Hospital of Lyon, Lyon, France.
11
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.
12
Department of Endocrinology, Hospital Louis Pradel, University Lyon I, Lyon, France.
13
Department of Thoracic, Vascular and Endocrine Surgery, University Hospital of Grenoble, Grenoble, France.
14
Department of Endocrinology and Diabetology, University Hospital of Grenoble, Grenoble, France.
15
Department of Digestive and Endocrine Surgery, University Hospital La Pitié Salpétrière, Paris, France.
16
Department of Nuclear Medicine and Endocrine Oncology, Gustave Roussy, University of Paris Sud, Villejuif, France.
17
Department of Gastroenterology and Pancreatology, Beaujon Hospital, University Paris 7, Clichy, France.
18
Department of Endocrinology, General Hospital of Chambéry, Chambéry, France.
19
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.

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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