Prognostic factors and survival in MEN1 patients with gastrinomas: Results from the DutchMEN study group (DMSG).
Cohort Studies
Female
Follow-Up Studies
Gastrinoma
/ metabolism
Humans
Intestinal Neoplasms
/ metabolism
Liver Neoplasms
/ metabolism
Male
Middle Aged
Netherlands
Neuroendocrine Tumors
/ metabolism
Pancreatic Neoplasms
/ metabolism
Prognosis
Proto-Oncogene Proteins
/ metabolism
Stomach Neoplasms
/ metabolism
Survival Rate
Zollinger-Ellison syndrome
multiple endocrine neoplasia type 1
neuroendocrine tumor
oncology
Journal
Journal of surgical oncology
ISSN: 1096-9098
Titre abrégé: J Surg Oncol
Pays: United States
ID NLM: 0222643
Informations de publication
Date de publication:
Nov 2019
Nov 2019
Historique:
received:
23
07
2019
accepted:
28
07
2019
pubmed:
12
8
2019
medline:
24
10
2019
entrez:
12
8
2019
Statut:
ppublish
Résumé
Gastrinomas are the most prevalent functioning neuroendocrine tumors (NET) in multiple endocrine neoplasia type 1 (MEN1). Guidelines suggest medical therapy in most patients, but surgery may be considered in a subgroup. Currently, factors to guide management are necessary. This population-based cohort study assessed prognostic factors of survival in patients with MEN1-related gastrinomas. Patients with MEN1 having gastrinomas were identified in the Dutch MEN1 database from 1990 to 2014 based on fasting serum gastrin (FSG) levels and/or pathology. Predictors of overall survival were assessed using Cox regression. Sixty-three patients with gastrinoma (16% of the MEN1 population) were identified. Five- and 10-year overall survival rates were 83% and 65%, respectively. Prognostic factors associated with overall survival were initial FSG levels ≥20x upper limit of normal (ULN) (hazard ratio [HR], 6.2 [95% confidence interval, 1.7-23.0]), pancreatic NET ≥2 cm (HR 4.5; [1.5-13.1]), synchronous liver metastases (HR 8.9; [2.1-36.7]), gastroduodenoscopy suspicious for gastric NETs (HR 12.7; [1.4-115.6]), and multiple concurrent NETs (HR 5.9; [1.2-27.7]). Life expectancy of patients with MEN1 gastrinoma is reduced. FSG levels and pancreatic NETs ≥2 cm are prognostic factors. FSG levels might guide surveillance intensity, step-up to additional diagnostics, or provide arguments in selecting patients who might benefit from surgery.
Sections du résumé
BACKGROUND AND OBJECTIVES
OBJECTIVE
Gastrinomas are the most prevalent functioning neuroendocrine tumors (NET) in multiple endocrine neoplasia type 1 (MEN1). Guidelines suggest medical therapy in most patients, but surgery may be considered in a subgroup. Currently, factors to guide management are necessary. This population-based cohort study assessed prognostic factors of survival in patients with MEN1-related gastrinomas.
METHODS
METHODS
Patients with MEN1 having gastrinomas were identified in the Dutch MEN1 database from 1990 to 2014 based on fasting serum gastrin (FSG) levels and/or pathology. Predictors of overall survival were assessed using Cox regression.
RESULTS
RESULTS
Sixty-three patients with gastrinoma (16% of the MEN1 population) were identified. Five- and 10-year overall survival rates were 83% and 65%, respectively. Prognostic factors associated with overall survival were initial FSG levels ≥20x upper limit of normal (ULN) (hazard ratio [HR], 6.2 [95% confidence interval, 1.7-23.0]), pancreatic NET ≥2 cm (HR 4.5; [1.5-13.1]), synchronous liver metastases (HR 8.9; [2.1-36.7]), gastroduodenoscopy suspicious for gastric NETs (HR 12.7; [1.4-115.6]), and multiple concurrent NETs (HR 5.9; [1.2-27.7]).
CONCLUSION
CONCLUSIONS
Life expectancy of patients with MEN1 gastrinoma is reduced. FSG levels and pancreatic NETs ≥2 cm are prognostic factors. FSG levels might guide surveillance intensity, step-up to additional diagnostics, or provide arguments in selecting patients who might benefit from surgery.
Identifiants
pubmed: 31401809
doi: 10.1002/jso.25667
pmc: PMC6852496
doi:
Substances chimiques
MEN1 protein, human
0
Proto-Oncogene Proteins
0
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
966-975Subventions
Organisme : Ipsen Pharmaceutical
Informations de copyright
© 2019 The Authors. Journal of Surgical Oncology published by Wiley Periodicals, Inc.
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