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

Dirk-Jan van Beek, Sjoerd Nell, Carolina R. C. Pieterman, Wouter W. de Herder, Annenienke C. van de Ven, Olaf M. Dekkers, Anouk N. van der Horst-Schrivers, Madeleine L. Drent, Peter H. Bisschop, Bas Havekes, Inne H. M. Borel Rinkes, Menno R. Vriens, Gerlof D. Valk

Research output: Contribution to journalArticleAcademicpeer-review

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.
Original languageEnglish
JournalJournal of Surgical Oncology
DOIs
Publication statusPublished - 2019

Cite this

van Beek, D-J., Nell, S., Pieterman, C. R. C., de Herder, W. W., van de Ven, A. C., Dekkers, O. M., ... Valk, G. D. (2019). Prognostic factors and survival in MEN1 patients with gastrinomas: Results from the DutchMEN study group (DMSG). Journal of Surgical Oncology. https://doi.org/10.1002/jso.25667
van Beek, Dirk-Jan ; Nell, Sjoerd ; Pieterman, Carolina R. C. ; de Herder, Wouter W. ; van de Ven, Annenienke C. ; Dekkers, Olaf M. ; van der Horst-Schrivers, Anouk N. ; Drent, Madeleine L. ; Bisschop, Peter H. ; Havekes, Bas ; Borel Rinkes, Inne H. M. ; Vriens, Menno R. ; Valk, Gerlof D. / Prognostic factors and survival in MEN1 patients with gastrinomas: Results from the DutchMEN study group (DMSG). In: Journal of Surgical Oncology. 2019.
@article{ca002943120741d6a28903d15c743d57,
title = "Prognostic factors and survival in MEN1 patients with gastrinomas: Results from the DutchMEN study group (DMSG)",
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.",
author = "{van Beek}, Dirk-Jan and Sjoerd Nell and Pieterman, {Carolina R. C.} and {de Herder}, {Wouter W.} and {van de Ven}, {Annenienke C.} and Dekkers, {Olaf M.} and {van der Horst-Schrivers}, {Anouk N.} and Drent, {Madeleine L.} and Bisschop, {Peter H.} and Bas Havekes and {Borel Rinkes}, {Inne H. M.} and Vriens, {Menno R.} and Valk, {Gerlof D.}",
year = "2019",
doi = "10.1002/jso.25667",
language = "English",
journal = "Journal of Surgical Oncology",
issn = "0022-4790",
publisher = "Wiley-Liss Inc.",

}

van Beek, D-J, Nell, S, Pieterman, CRC, de Herder, WW, van de Ven, AC, Dekkers, OM, van der Horst-Schrivers, AN, Drent, ML, Bisschop, PH, Havekes, B, Borel Rinkes, IHM, Vriens, MR & Valk, GD 2019, 'Prognostic factors and survival in MEN1 patients with gastrinomas: Results from the DutchMEN study group (DMSG)' Journal of Surgical Oncology. https://doi.org/10.1002/jso.25667

Prognostic factors and survival in MEN1 patients with gastrinomas: Results from the DutchMEN study group (DMSG). / van Beek, Dirk-Jan; Nell, Sjoerd; Pieterman, Carolina R. C.; de Herder, Wouter W.; van de Ven, Annenienke C.; Dekkers, Olaf M.; van der Horst-Schrivers, Anouk N.; Drent, Madeleine L.; Bisschop, Peter H.; Havekes, Bas; Borel Rinkes, Inne H. M.; Vriens, Menno R.; Valk, Gerlof D.

In: Journal of Surgical Oncology, 2019.

Research output: Contribution to journalArticleAcademicpeer-review

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.

PY - 2019

Y1 - 2019

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.

AB - 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.

UR - https://www.scopus.com/inward/record.uri?partnerID=HzOxMe3b&scp=85070355954&origin=inward

UR - https://www.ncbi.nlm.nih.gov/pubmed/31401809

U2 - 10.1002/jso.25667

DO - 10.1002/jso.25667

M3 - Article

JO - Journal of Surgical Oncology

JF - Journal of Surgical Oncology

SN - 0022-4790

ER -