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J Surg Oncol. 2019 Aug 10. doi: 10.1002/jso.25667. [Epub ahead of print]

Prognostic factors and survival in MEN1 patients with gastrinomas: Results from the DutchMEN study group (DMSG).

Author information

1
Department of Endocrine Surgical Oncology, University Medical Center Utrecht, Utrecht, The Netherlands.
2
Department of Endocrine Oncology, University Medical Center Utrecht, Utrecht, The Netherlands.
3
Department of Internal Medicine, Erasmus Medical Center, Rotterdam, The Netherlands.
4
Department of Endocrinology, Radboud University Medical Center, Nijmegen, The Netherlands.
5
Departments of Endocrinology and Metabolism and Clinical Epidemiology, Leiden University Medical Center, Leiden, The Netherlands.
6
Department of Endocrinology, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.
7
Department of Internal Medicine, Section of Endocrinology, Amsterdam UMC location, VU University Medical Center, Amsterdam, The Netherlands.
8
Department of Endocrinology and Metabolism, Amsterdam UMC location Academic Medical Center, Amsterdam, The Netherlands.
9
Department of Internal Medicine, Division of Endocrinology, Maastricht University Medical Center, Maastricht, The Netherlands.

Abstract

BACKGROUND AND OBJECTIVES:

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:

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:

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:

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.

KEYWORDS:

Zollinger-Ellison syndrome; multiple endocrine neoplasia type 1; neuroendocrine tumor; oncology

PMID:
31401809
DOI:
10.1002/jso.25667

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