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, 120 (6), 966-975

Prognostic Factors and Survival in MEN1 Patients With Gastrinomas: Results From the DutchMEN Study Group (DMSG)

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Prognostic Factors and Survival in MEN1 Patients With Gastrinomas: Results From the DutchMEN Study Group (DMSG)

Dirk-Jan van Beek et al. J Surg Oncol.

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.

Conflict of interest statement

There is no conflict of interest that could be perceived as prejudicing the impartiality of the research reported.

Figures

Figure 1
Figure 1
Overall survival (OS) of MEN1 gastrinoma patients (A). OS of MEN1 patients with and without gastrinomas (B). OS of MEN1 patients with and without gastrinomas (age and gender matched) (C). MEN1, multiple endocrine neoplasia type 1
Figure 2
Figure 2
Overall survival of patients with MEN1 according to initial fasting serum gastrin levels. MEN1, multiple endocrine neoplasia type 1

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References

    1. Chandrasekharappa SC. Positional cloning of the gene for multiple endocrine neoplasia‐type 1. Science. 1997;276:404‐407. - PubMed
    1. de Laat JM, van der Luijt RB, Pieterman CRC, et al. MEN1 redefined, a clinical comparison of mutation‐positive and mutation‐negative patients. BMC Med. 2016;14:182 10.1186/s12916-016-0708-1 - DOI - PMC - PubMed
    1. Pieterman CRC, Conemans EB, Dreijerink KMA, et al. Thoracic and duodenopancreatic neuroendocrine tumors in multiple endocrine neoplasia type 1: natural history and function of menin in tumorigenesis. Endocr Relat Cancer. 2014;21:R121‐R142. 10.1530/ERC-13-0482 - DOI - PubMed
    1. Zollinger RM, Ellison EH. Primary peptic ulcerations of the jejunum associated with islet cell tumors of the pancreas. Ann Surg. 1955;142:709‐728. 724–8. - PMC - PubMed
    1. Pipeleers‐Marichal M, Somers G, Willems G, et al. Gastrinomas in the duodenums of patients with multiple endocrine neoplasia type 1 and the Zollinger‐Ellison syndrome. N Engl J Med. 1990;322:723‐727. 10.1056/NEJM199003153221103 - DOI - PubMed

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