Long-term outcomes in patients with multiple endocrine neoplasia type 1 and pancreaticoduodenal neuroendocrine tumours

Summary Background In patients with multiple endocrine neoplasia type 1 (MEN-1), pancreaticoduodenal (PD) neuroendocrine tumours (NETs) are associated with early mortality, yet the best treatment strategy remains uncertain. Aim To assess patient important outcomes (mortality and metastasis) of PD-N...

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Main Authors: Donegan, D., Singh Ospina, Naykky, Rodríguez Gutiérrez, René, Al Hilli, Zahraa, Thompson, G. B., Clarke, B. L., Young, W. F.
Format: Article
Language:English
Published: Blackwell Scientific Publications 2016
Subjects:
Online Access:http://eprints.uanl.mx/18149/1/447.pdf
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author Donegan, D.
Singh Ospina, Naykky
Rodríguez Gutiérrez, René
Al Hilli, Zahraa
Thompson, G. B.
Clarke, B. L.
Young, W. F.
author_facet Donegan, D.
Singh Ospina, Naykky
Rodríguez Gutiérrez, René
Al Hilli, Zahraa
Thompson, G. B.
Clarke, B. L.
Young, W. F.
author_sort Donegan, D.
collection Repositorio Institucional
description Summary Background In patients with multiple endocrine neoplasia type 1 (MEN-1), pancreaticoduodenal (PD) neuroendocrine tumours (NETs) are associated with early mortality, yet the best treatment strategy remains uncertain. Aim To assess patient important outcomes (mortality and metastasis) of PD-NETs and predictors of outcomes in patients with MEN-1. Methods Retrospective cohort of patients with MEN-1 who attended the Mayo Clinic, Rochester, MN from 1997 to 2014. Results We identified 287 patients with MEN-1; 199 (69%) patients had 217 PD-NETs. Among those with a PD-NETs, 129 (65%) had surgery of which 90 (70%) had their primary surgery performed at Mayo Clinic. The median postoperative follow-up was 8 years during which 13 (14%) patients died. The mean (±standard deviation) age of death was 51 (±9) years. Tumour size, metastasis at surgery or tumour type were not predictive of mortality, but for every year older at surgery, the odds of metastasis increased by 6%. Surgery was not performed in 70 (35%) patients. Among those who were observed/medically managed without known metastatic disease, mean tumour growth was 0·02 cm/year (range, -0·13–0-4 cm/year). Four patients (7%) died at a median age of 77 (range, 51–89) years. Conclusion PD-NETs are common in patients with MEN-1 and are associated with early mortality even after surgical intervention. Active surveillance is a viable option in nonaggressive PD-NETs, although definitive factors identifying such patients are lacking. Therefore, counselling regarding risks and benefits of current treatment options remains integral to the care of patients with MEN-1.
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spelling eprints-181492020-09-09T13:44:38Z http://eprints.uanl.mx/18149/ Long-term outcomes in patients with multiple endocrine neoplasia type 1 and pancreaticoduodenal neuroendocrine tumours Donegan, D. Singh Ospina, Naykky Rodríguez Gutiérrez, René Al Hilli, Zahraa Thompson, G. B. Clarke, B. L. Young, W. F. R Medicina en General Summary Background In patients with multiple endocrine neoplasia type 1 (MEN-1), pancreaticoduodenal (PD) neuroendocrine tumours (NETs) are associated with early mortality, yet the best treatment strategy remains uncertain. Aim To assess patient important outcomes (mortality and metastasis) of PD-NETs and predictors of outcomes in patients with MEN-1. Methods Retrospective cohort of patients with MEN-1 who attended the Mayo Clinic, Rochester, MN from 1997 to 2014. Results We identified 287 patients with MEN-1; 199 (69%) patients had 217 PD-NETs. Among those with a PD-NETs, 129 (65%) had surgery of which 90 (70%) had their primary surgery performed at Mayo Clinic. The median postoperative follow-up was 8 years during which 13 (14%) patients died. The mean (±standard deviation) age of death was 51 (±9) years. Tumour size, metastasis at surgery or tumour type were not predictive of mortality, but for every year older at surgery, the odds of metastasis increased by 6%. Surgery was not performed in 70 (35%) patients. Among those who were observed/medically managed without known metastatic disease, mean tumour growth was 0·02 cm/year (range, -0·13–0-4 cm/year). Four patients (7%) died at a median age of 77 (range, 51–89) years. Conclusion PD-NETs are common in patients with MEN-1 and are associated with early mortality even after surgical intervention. Active surveillance is a viable option in nonaggressive PD-NETs, although definitive factors identifying such patients are lacking. Therefore, counselling regarding risks and benefits of current treatment options remains integral to the care of patients with MEN-1. Blackwell Scientific Publications 2016-10-19 Article PeerReviewed text en cc_by_nc_nd http://eprints.uanl.mx/18149/1/447.pdf http://eprints.uanl.mx/18149/1.haspreviewThumbnailVersion/447.pdf Donegan, D. y Singh Ospina, Naykky y Rodríguez Gutiérrez, René y Al Hilli, Zahraa y Thompson, G. B. y Clarke, B. L. y Young, W. F. (2016) Long-term outcomes in patients with multiple endocrine neoplasia type 1 and pancreaticoduodenal neuroendocrine tumours. Clinical endocrinology, 86 (2). pp. 199-206. ISSN 03000664 http://doi.org/10.1111/cen.13264 doi:10.1111/cen.13264
spellingShingle R Medicina en General
Donegan, D.
Singh Ospina, Naykky
Rodríguez Gutiérrez, René
Al Hilli, Zahraa
Thompson, G. B.
Clarke, B. L.
Young, W. F.
Long-term outcomes in patients with multiple endocrine neoplasia type 1 and pancreaticoduodenal neuroendocrine tumours
thumbnail https://rediab.uanl.mx/themes/sandal5/images/online.png
title Long-term outcomes in patients with multiple endocrine neoplasia type 1 and pancreaticoduodenal neuroendocrine tumours
title_full Long-term outcomes in patients with multiple endocrine neoplasia type 1 and pancreaticoduodenal neuroendocrine tumours
title_fullStr Long-term outcomes in patients with multiple endocrine neoplasia type 1 and pancreaticoduodenal neuroendocrine tumours
title_full_unstemmed Long-term outcomes in patients with multiple endocrine neoplasia type 1 and pancreaticoduodenal neuroendocrine tumours
title_short Long-term outcomes in patients with multiple endocrine neoplasia type 1 and pancreaticoduodenal neuroendocrine tumours
title_sort long term outcomes in patients with multiple endocrine neoplasia type 1 and pancreaticoduodenal neuroendocrine tumours
topic R Medicina en General
url http://eprints.uanl.mx/18149/1/447.pdf
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