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

doi:10.4158/EP161639.OR

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^*

^*Corresponding author for this work

Research output: Contribution to journal › Article › Academic › peer-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.

Original language	English
Pages (from-to)	641-648
Number of pages	8
Journal	Endocrine Practice
Volume	23
Issue number	6
DOIs	https://doi.org/10.4158/EP161639.OR
Publication status	Published - Jun 2017

Keywords

ENDOCRINE NEOPLASIA TYPE-1
DES-TUMEURS-ENDOCRINES
NEUROENDOCRINE TUMORS
GUIDELINES
MANAGEMENT
GTE

Access to Document

10.4158/EP161639.OR

Cite this

Conemans, E. B., Nell, S., Pieterman, C. R. C., de Herder, W. W., Dekkers, O. M., Hermus, A. R., van der Horst-Schrivers, A. N., Bisschop, P. H., Havekes, B., Drent, M. L., Vriens, M. R., & Valk, G. D. (2017). PROGNOSTIC FACTORS FOR SURVIVAL OF MEN1 PATIENTS WITH DUODENOPANCREATIC TUMORS METASTATIC TO THE LIVER: RESULTS FROM THE DMSG. Endocrine Practice, 23(6), 641-648. https://doi.org/10.4158/EP161639.OR

@article{38500d1051a54002a180aa169b614fbe,

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.",

keywords = "ENDOCRINE NEOPLASIA TYPE-1, DES-TUMEURS-ENDOCRINES, NEUROENDOCRINE TUMORS, GUIDELINES, MANAGEMENT, GTE",

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.}",

year = "2017",

month = jun,

doi = "10.4158/EP161639.OR",

language = "English",

volume = "23",

pages = "641--648",

journal = "Endocrine Practice",

issn = "1530-891X",

publisher = "American Association of Clinical Endocrinologists",

number = "6",

}

Conemans, EB, Nell, S, Pieterman, CRC, de Herder, WW, Dekkers, OM, Hermus, AR, van der Horst-Schrivers, AN, Bisschop, PH, Havekes, B, Drent, ML, Vriens, MR & Valk, GD 2017, 'PROGNOSTIC FACTORS FOR SURVIVAL OF MEN1 PATIENTS WITH DUODENOPANCREATIC TUMORS METASTATIC TO THE LIVER: RESULTS FROM THE DMSG', Endocrine Practice, vol. 23, no. 6, pp. 641-648. https://doi.org/10.4158/EP161639.OR

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

Y1 - 2017/6

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.

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.

KW - ENDOCRINE NEOPLASIA TYPE-1

KW - DES-TUMEURS-ENDOCRINES

KW - NEUROENDOCRINE TUMORS

KW - GUIDELINES

KW - MANAGEMENT

KW - GTE

U2 - 10.4158/EP161639.OR

DO - 10.4158/EP161639.OR

M3 - Article

C2 - 28225311

SN - 1530-891X

VL - 23

SP - 641

EP - 648

JO - Endocrine Practice

JF - Endocrine Practice

IS - 6

ER -