PROGNOSTIC FACTORS FOR SURVIVAL OF MEN1 PATIENTS WITH DUODENOPANCREATIC TUMORS METASTATIC TO THE LIVER: RESULTS FROM THE DMSG

Elfi B. Conemans, Sjoerd Nell, Carolina R.C. Pieterman, Wouter W. de Herder, Olaf M. Dekkers, Ad R. Hermus, Anouk N. van der Horst-Schrivers, Peter H. Bisschop, Bas Havekes, Madeleine L. Drent, Menno R. Vriens, Gerlof D. Valk

Research output: Contribution to journalArticleAcademicpeer-review

Abstract

OBJECTIVE: Duodenopancreatic neuroendocrine tumors (DP-NETs) develop in a majority of patients with multiple endocrine neoplasia type 1 (MEN1) and are the leading cause of death. Overall survival (OS) and prognostic factors for patients with liver metastases from DP-NETs are not known.

METHODS: This was a cohort study using the Dutch National MEN1 database, which includes >90% of the Dutch MEN1 population treated between 1990 and 2014. OS was assessed with time to event analysis, and prognostic factors were evaluated.

RESULTS: A total of 56% of the MEN1 patients (n = 220) were diagnosed with a DP-NET, of who 34 (15%) developed DP-NET liver metastases. Median age at liver metastases diagnosis was 53 years (range 31-74). Of those patients, 16 patients (47%) had died after a median follow-up of 4 years (range 0.3-12.3). OS at 2, 5, and 10 years were 91%, 65%, and 50%, respectively. A trend towards worse survival was seen in males compared to females (5-year OS 58% versus 75%, P = .07) and also in patients with multiple liver metastases compared to patients with solitary liver metastasis (59 versus 83%, P = .09).

CONCLUSION: Despite the fairly indolent course of DP-NET liver metastases in MEN1 patients, half of the population was deceased after 10 years. Sex and tumor load at diagnosis of liver metastases are possible prognostic factors for worse survival.

ABBREVIATIONS: DMSG = DutchMEN1 Study Group; D-NET = duodenal neuroendocrine tumor; DP-NET = duodenopancreatic neuroendocrine tumor; HPF = high-power field; Ki67 LI = Ki67 labeling index; MEN1 = multiple endocrine neoplasia type 1; NET = neuroendocrine tumor; OS = overall survival; P-NET = pancreatic neuroendocrine tumor; PPI = proton pump inhibitor; ULN = upper limit of normal; WHO = World Health Organization.

Original languageEnglish
Pages (from-to)641-648
Number of pages8
JournalEndocrine practice : official journal of the American College of Endocrinology and the American Association of Clinical Endocrinologists
Volume23
Issue number6
DOIs
Publication statusPublished - 1 Jun 2017

Cite this

Conemans, Elfi B. ; Nell, Sjoerd ; Pieterman, Carolina R.C. ; de Herder, Wouter W. ; Dekkers, Olaf M. ; Hermus, Ad R. ; van der Horst-Schrivers, Anouk N. ; Bisschop, Peter H. ; Havekes, Bas ; Drent, Madeleine L. ; Vriens, Menno R. ; Valk, Gerlof D. / PROGNOSTIC FACTORS FOR SURVIVAL OF MEN1 PATIENTS WITH DUODENOPANCREATIC TUMORS METASTATIC TO THE LIVER : RESULTS FROM THE DMSG. In: Endocrine practice : official journal of the American College of Endocrinology and the American Association of Clinical Endocrinologists. 2017 ; Vol. 23, No. 6. pp. 641-648.
@article{0bfea50b71ee4a4a8c8b3049c31d1393,
title = "PROGNOSTIC FACTORS FOR SURVIVAL OF MEN1 PATIENTS WITH DUODENOPANCREATIC TUMORS METASTATIC TO THE LIVER: RESULTS FROM THE DMSG",
abstract = "OBJECTIVE: Duodenopancreatic neuroendocrine tumors (DP-NETs) develop in a majority of patients with multiple endocrine neoplasia type 1 (MEN1) and are the leading cause of death. Overall survival (OS) and prognostic factors for patients with liver metastases from DP-NETs are not known.METHODS: This was a cohort study using the Dutch National MEN1 database, which includes >90{\%} of the Dutch MEN1 population treated between 1990 and 2014. OS was assessed with time to event analysis, and prognostic factors were evaluated.RESULTS: A total of 56{\%} of the MEN1 patients (n = 220) were diagnosed with a DP-NET, of who 34 (15{\%}) developed DP-NET liver metastases. Median age at liver metastases diagnosis was 53 years (range 31-74). Of those patients, 16 patients (47{\%}) had died after a median follow-up of 4 years (range 0.3-12.3). OS at 2, 5, and 10 years were 91{\%}, 65{\%}, and 50{\%}, respectively. A trend towards worse survival was seen in males compared to females (5-year OS 58{\%} versus 75{\%}, P = .07) and also in patients with multiple liver metastases compared to patients with solitary liver metastasis (59 versus 83{\%}, P = .09).CONCLUSION: Despite the fairly indolent course of DP-NET liver metastases in MEN1 patients, half of the population was deceased after 10 years. Sex and tumor load at diagnosis of liver metastases are possible prognostic factors for worse survival.ABBREVIATIONS: DMSG = DutchMEN1 Study Group; D-NET = duodenal neuroendocrine tumor; DP-NET = duodenopancreatic neuroendocrine tumor; HPF = high-power field; Ki67 LI = Ki67 labeling index; MEN1 = multiple endocrine neoplasia type 1; NET = neuroendocrine tumor; OS = overall survival; P-NET = pancreatic neuroendocrine tumor; PPI = proton pump inhibitor; ULN = upper limit of normal; WHO = World Health Organization.",
author = "Conemans, {Elfi B.} and Sjoerd Nell and Pieterman, {Carolina R.C.} and {de Herder}, {Wouter W.} and Dekkers, {Olaf M.} and Hermus, {Ad R.} and {van der Horst-Schrivers}, {Anouk N.} and Bisschop, {Peter H.} and Bas Havekes and Drent, {Madeleine L.} and Vriens, {Menno R.} and Valk, {Gerlof D.}",
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journal = "Endocrine Practice",
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}

PROGNOSTIC FACTORS FOR SURVIVAL OF MEN1 PATIENTS WITH DUODENOPANCREATIC TUMORS METASTATIC TO THE LIVER : RESULTS FROM THE DMSG. / Conemans, Elfi B.; Nell, Sjoerd; Pieterman, Carolina R.C.; de Herder, Wouter W.; Dekkers, Olaf M.; Hermus, Ad R.; van der Horst-Schrivers, Anouk N.; Bisschop, Peter H.; Havekes, Bas; Drent, Madeleine L.; Vriens, Menno R.; Valk, Gerlof D.

In: Endocrine practice : official journal of the American College of Endocrinology and the American Association of Clinical Endocrinologists, Vol. 23, No. 6, 01.06.2017, p. 641-648.

Research output: Contribution to journalArticleAcademicpeer-review

TY - JOUR

T1 - PROGNOSTIC FACTORS FOR SURVIVAL OF MEN1 PATIENTS WITH DUODENOPANCREATIC TUMORS METASTATIC TO THE LIVER

T2 - RESULTS FROM THE DMSG

AU - Conemans, Elfi B.

AU - Nell, Sjoerd

AU - Pieterman, Carolina R.C.

AU - de Herder, Wouter W.

AU - Dekkers, Olaf M.

AU - Hermus, Ad R.

AU - van der Horst-Schrivers, Anouk N.

AU - Bisschop, Peter H.

AU - Havekes, Bas

AU - Drent, Madeleine L.

AU - Vriens, Menno R.

AU - Valk, Gerlof D.

PY - 2017/6/1

Y1 - 2017/6/1

N2 - OBJECTIVE: Duodenopancreatic neuroendocrine tumors (DP-NETs) develop in a majority of patients with multiple endocrine neoplasia type 1 (MEN1) and are the leading cause of death. Overall survival (OS) and prognostic factors for patients with liver metastases from DP-NETs are not known.METHODS: This was a cohort study using the Dutch National MEN1 database, which includes >90% of the Dutch MEN1 population treated between 1990 and 2014. OS was assessed with time to event analysis, and prognostic factors were evaluated.RESULTS: A total of 56% of the MEN1 patients (n = 220) were diagnosed with a DP-NET, of who 34 (15%) developed DP-NET liver metastases. Median age at liver metastases diagnosis was 53 years (range 31-74). Of those patients, 16 patients (47%) had died after a median follow-up of 4 years (range 0.3-12.3). OS at 2, 5, and 10 years were 91%, 65%, and 50%, respectively. A trend towards worse survival was seen in males compared to females (5-year OS 58% versus 75%, P = .07) and also in patients with multiple liver metastases compared to patients with solitary liver metastasis (59 versus 83%, P = .09).CONCLUSION: Despite the fairly indolent course of DP-NET liver metastases in MEN1 patients, half of the population was deceased after 10 years. Sex and tumor load at diagnosis of liver metastases are possible prognostic factors for worse survival.ABBREVIATIONS: DMSG = DutchMEN1 Study Group; D-NET = duodenal neuroendocrine tumor; DP-NET = duodenopancreatic neuroendocrine tumor; HPF = high-power field; Ki67 LI = Ki67 labeling index; MEN1 = multiple endocrine neoplasia type 1; NET = neuroendocrine tumor; OS = overall survival; P-NET = pancreatic neuroendocrine tumor; PPI = proton pump inhibitor; ULN = upper limit of normal; WHO = World Health Organization.

AB - OBJECTIVE: Duodenopancreatic neuroendocrine tumors (DP-NETs) develop in a majority of patients with multiple endocrine neoplasia type 1 (MEN1) and are the leading cause of death. Overall survival (OS) and prognostic factors for patients with liver metastases from DP-NETs are not known.METHODS: This was a cohort study using the Dutch National MEN1 database, which includes >90% of the Dutch MEN1 population treated between 1990 and 2014. OS was assessed with time to event analysis, and prognostic factors were evaluated.RESULTS: A total of 56% of the MEN1 patients (n = 220) were diagnosed with a DP-NET, of who 34 (15%) developed DP-NET liver metastases. Median age at liver metastases diagnosis was 53 years (range 31-74). Of those patients, 16 patients (47%) had died after a median follow-up of 4 years (range 0.3-12.3). OS at 2, 5, and 10 years were 91%, 65%, and 50%, respectively. A trend towards worse survival was seen in males compared to females (5-year OS 58% versus 75%, P = .07) and also in patients with multiple liver metastases compared to patients with solitary liver metastasis (59 versus 83%, P = .09).CONCLUSION: Despite the fairly indolent course of DP-NET liver metastases in MEN1 patients, half of the population was deceased after 10 years. Sex and tumor load at diagnosis of liver metastases are possible prognostic factors for worse survival.ABBREVIATIONS: DMSG = DutchMEN1 Study Group; D-NET = duodenal neuroendocrine tumor; DP-NET = duodenopancreatic neuroendocrine tumor; HPF = high-power field; Ki67 LI = Ki67 labeling index; MEN1 = multiple endocrine neoplasia type 1; NET = neuroendocrine tumor; OS = overall survival; P-NET = pancreatic neuroendocrine tumor; PPI = proton pump inhibitor; ULN = upper limit of normal; WHO = World Health Organization.

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U2 - 10.4158/EP161639.OR

DO - 10.4158/EP161639.OR

M3 - Article

VL - 23

SP - 641

EP - 648

JO - Endocrine Practice

JF - Endocrine Practice

SN - 1530-891X

IS - 6

ER -