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Prognostic factors and survival in MEN1 patients with gastrinomas: Results from the DutchMEN study group (DMSG).
J Surg Oncol. 2019 Nov; 120(6):966-975.JS

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.

Authors+Show Affiliations

Department of Endocrine Surgical Oncology, University Medical Center Utrecht, Utrecht, The Netherlands.Department of Endocrine Surgical Oncology, University Medical Center Utrecht, Utrecht, The Netherlands.Department of Endocrine Oncology, University Medical Center Utrecht, Utrecht, The Netherlands.Department of Internal Medicine, Erasmus Medical Center, Rotterdam, The Netherlands.Department of Endocrinology, Radboud University Medical Center, Nijmegen, The Netherlands.Departments of Endocrinology and Metabolism and Clinical Epidemiology, Leiden University Medical Center, Leiden, The Netherlands.Department of Endocrinology, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.Department of Internal Medicine, Section of Endocrinology, Amsterdam UMC location, VU University Medical Center, Amsterdam, The Netherlands.Department of Endocrinology and Metabolism, Amsterdam UMC location Academic Medical Center, Amsterdam, The Netherlands.Department of Internal Medicine, Division of Endocrinology, Maastricht University Medical Center, Maastricht, The Netherlands.Department of Endocrine Surgical Oncology, University Medical Center Utrecht, Utrecht, The Netherlands.Department of Endocrine Surgical Oncology, University Medical Center Utrecht, Utrecht, The Netherlands.Department of Endocrine Oncology, University Medical Center Utrecht, Utrecht, The Netherlands.

Pub Type(s)

Journal Article

Language

eng

PubMed ID

31401809

Citation

van Beek, Dirk-Jan, et al. "Prognostic Factors and Survival in MEN1 Patients With Gastrinomas: Results From the DutchMEN Study Group (DMSG)." Journal of Surgical Oncology, vol. 120, no. 6, 2019, pp. 966-975.
van Beek DJ, Nell S, Pieterman CRC, et al. Prognostic factors and survival in MEN1 patients with gastrinomas: Results from the DutchMEN study group (DMSG). J Surg Oncol. 2019;120(6):966-975.
van Beek, D. J., Nell, S., Pieterman, C. R. C., de Herder, W. W., van de Ven, A. C., Dekkers, O. M., van der Horst-Schrivers, A. N., Drent, M. L., Bisschop, P. H., Havekes, B., Borel Rinkes, I. H. M., Vriens, M. R., & Valk, G. D. (2019). Prognostic factors and survival in MEN1 patients with gastrinomas: Results from the DutchMEN study group (DMSG). Journal of Surgical Oncology, 120(6), 966-975. https://doi.org/10.1002/jso.25667
van Beek DJ, et al. Prognostic Factors and Survival in MEN1 Patients With Gastrinomas: Results From the DutchMEN Study Group (DMSG). J Surg Oncol. 2019;120(6):966-975. PubMed PMID: 31401809.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Prognostic factors and survival in MEN1 patients with gastrinomas: Results from the DutchMEN study group (DMSG). AU - van Beek,Dirk-Jan, AU - Nell,Sjoerd, AU - Pieterman,Carolina R C, AU - de Herder,Wouter W, AU - van de Ven,Annenienke C, AU - Dekkers,Olaf M, AU - van der Horst-Schrivers,Anouk N, AU - Drent,Madeleine L, AU - Bisschop,Peter H, AU - Havekes,Bas, AU - Borel Rinkes,Inne H M, AU - Vriens,Menno R, AU - Valk,Gerlof D, Y1 - 2019/08/10/ PY - 2019/07/23/received PY - 2019/07/28/accepted PY - 2019/8/12/pubmed PY - 2019/10/24/medline PY - 2019/8/12/entrez KW - Zollinger-Ellison syndrome KW - multiple endocrine neoplasia type 1 KW - neuroendocrine tumor KW - oncology SP - 966 EP - 975 JF - Journal of surgical oncology JO - J Surg Oncol VL - 120 IS - 6 N2 - 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. SN - 1096-9098 UR - https://www.unboundmedicine.com/medline/citation/31401809/Prognostic_factors_and_survival_in_MEN1_patients_with_gastrinomas:_Results_from_the_DutchMEN_study_group_(DMSG) L2 - https://doi.org/10.1002/jso.25667 DB - PRIME DP - Unbound Medicine ER -