Gastroenteropancreatic Neuroendocrine Neoplasia Characterization in Portugal: Results from the NETs Study Group of the Portuguese Society of Endocrinology, Diabetes and Metabolism

Background. The incidence of gastroenteropancreatic neuroendocrine neoplasms (GEP-NENs) has been increasing in the last five decades, but there is no large-scale data regarding these tumours in Portugal. We conducted a cross-sectional, multicentric study in main Portuguese centers to evaluate the cl...

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Main Authors: A. P. Santos, J. Vinagre, P. Soares, I. Claro, A. C. Sanches, L. Gomes, I. Fernandes, A. L. Catarino, J. Preto, B. D. Pereira, A. P. Marques, F. Rodrigues, C. Amaral, G. Rocha, J. C. Mellidez, H. Simões, J. M. Lopes, M. J. Bugalho, On behalf of the NETs Study Group of the Portuguese Society of Endocrinology, Diabetes and Metabolism
Format: Article
Language:English
Published: Wiley 2019-01-01
Series:International Journal of Endocrinology
Online Access:http://dx.doi.org/10.1155/2019/4518742
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author A. P. Santos
J. Vinagre
P. Soares
I. Claro
A. C. Sanches
L. Gomes
I. Fernandes
A. L. Catarino
J. Preto
B. D. Pereira
A. P. Marques
F. Rodrigues
C. Amaral
G. Rocha
J. C. Mellidez
H. Simões
J. M. Lopes
M. J. Bugalho
On behalf of the NETs Study Group of the Portuguese Society of Endocrinology, Diabetes and Metabolism
author_facet A. P. Santos
J. Vinagre
P. Soares
I. Claro
A. C. Sanches
L. Gomes
I. Fernandes
A. L. Catarino
J. Preto
B. D. Pereira
A. P. Marques
F. Rodrigues
C. Amaral
G. Rocha
J. C. Mellidez
H. Simões
J. M. Lopes
M. J. Bugalho
On behalf of the NETs Study Group of the Portuguese Society of Endocrinology, Diabetes and Metabolism
author_sort A. P. Santos
collection DOAJ
description Background. The incidence of gastroenteropancreatic neuroendocrine neoplasms (GEP-NENs) has been increasing in the last five decades, but there is no large-scale data regarding these tumours in Portugal. We conducted a cross-sectional, multicentric study in main Portuguese centers to evaluate the clinical, pathological, and therapeutic profile of GEP-NENs. Methods. From November, 2012, to July, 2014, data from 293 patients diagnosed with GEP-NENs from 15 centers in Portugal was collected and registered in an online electronic platform. Results. Median age at diagnosis was 56.5 (range: 15-87) years with a preponderance of females (54.6%). The most frequent primary sites were the pancreas (31.1%), jejunum-ileum (24.2%), stomach (13.7%), and rectum (8.5%). Data regarding hormonal status was not available in most patients (82.3%). Stratified by the tumour grade (WHO 2010 classification), we observed 64.0% of NET G1, 24.7% of NET G2, and 11.3% of NEC. Poorly differentiated tumours occurred mainly in older patients (p=0.017), were larger (p<0.001), and presented more vascular (p=0.004) and lymphatic (p=0.001) invasion. At the time of diagnosis, 44.4% of GEP-NENs presented metastatic disease. Surgery (79.6%) and somatostatin analogues (30.7%) were the most frequently used therapies of GEP-NENs with reported grading. Conclusion. In general, Portuguese patients with GEP-NENs presented similar characteristics to other populations described in the literature. This cross-sectional study represents the first step to establish a national database of GEP-NENs that may aid in understanding the clinical and epidemiological features of these tumours in Portugal.
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spelling doaj-art-b06279028dfd4a7d9ad9e7f12debeb9b2025-02-03T06:11:22ZengWileyInternational Journal of Endocrinology1687-83371687-83452019-01-01201910.1155/2019/45187424518742Gastroenteropancreatic Neuroendocrine Neoplasia Characterization in Portugal: Results from the NETs Study Group of the Portuguese Society of Endocrinology, Diabetes and MetabolismA. P. Santos0J. Vinagre1P. Soares2I. Claro3A. C. Sanches4L. Gomes5I. Fernandes6A. L. Catarino7J. Preto8B. D. Pereira9A. P. Marques10F. Rodrigues11C. Amaral12G. Rocha13J. C. Mellidez14H. Simões15J. M. Lopes16M. J. Bugalho17On behalf of the NETs Study Group of the Portuguese Society of Endocrinology, Diabetes and Metabolism18Instituto Português de Oncologia do Porto, Francisco Gentil (IPOPFG), 4200-162 Porto, PortugalInstituto de Investigação e Inovação em Saúde (i3S), 4200-135 Porto, PortugalInstituto de Investigação e Inovação em Saúde (i3S), 4200-135 Porto, PortugalCentro Hospitalar de Lisboa Ocidental (CHLO), 1349-019 Lisboa, PortugalInstituto Português de Oncologia do Porto, Francisco Gentil (IPOPFG), 4200-162 Porto, PortugalCentro Hospitalar e Universitário de Coimbra (CHUC), 3000-075 Coimbra, PortugalCentro Hospitalar Lisboa Norte, EPE (CHLN), 1649-035 Lisboa, PortugalHospital da Luz, 1500-650 Lisboa, PortugalFaculdade de Medicina da Universidade do Porto (FMUP), 4200-319 Porto, PortugalHospital Garcia de Orta, EPE, 2801-951 Almada, PortugalUnidade Local de Saúde de Matosinhos, 4464-513 Senhora da Hora, PortugalInstituto Português de Oncologia de Coimbra, Francisco Gentil (IPOCFG), 3000-075 Coimbra, PortugalCentro Hospitalar do Porto-Hospital Santo António, 4099-001 Porto, PortugalCentro Hospitalar Gaia/Espinho (CHGE), 4434-502 Vila Nova de Gaia, PortugalCentro Hospitalar do Baixo Vouga (CHBV), 3810-501 Aveiro, PortugalCentro Hospitalar de Lisboa Ocidental (CHLO), 1349-019 Lisboa, PortugalInstituto de Investigação e Inovação em Saúde (i3S), 4200-135 Porto, PortugalCentro Hospitalar Lisboa Norte, EPE (CHLN), 1649-035 Lisboa, PortugalPortuguese Society of Endocrinology, Diabetes and Metabolism, Rua Fernando Vicente Mendes, 1B1600-892 Lisboa, PortugalBackground. The incidence of gastroenteropancreatic neuroendocrine neoplasms (GEP-NENs) has been increasing in the last five decades, but there is no large-scale data regarding these tumours in Portugal. We conducted a cross-sectional, multicentric study in main Portuguese centers to evaluate the clinical, pathological, and therapeutic profile of GEP-NENs. Methods. From November, 2012, to July, 2014, data from 293 patients diagnosed with GEP-NENs from 15 centers in Portugal was collected and registered in an online electronic platform. Results. Median age at diagnosis was 56.5 (range: 15-87) years with a preponderance of females (54.6%). The most frequent primary sites were the pancreas (31.1%), jejunum-ileum (24.2%), stomach (13.7%), and rectum (8.5%). Data regarding hormonal status was not available in most patients (82.3%). Stratified by the tumour grade (WHO 2010 classification), we observed 64.0% of NET G1, 24.7% of NET G2, and 11.3% of NEC. Poorly differentiated tumours occurred mainly in older patients (p=0.017), were larger (p<0.001), and presented more vascular (p=0.004) and lymphatic (p=0.001) invasion. At the time of diagnosis, 44.4% of GEP-NENs presented metastatic disease. Surgery (79.6%) and somatostatin analogues (30.7%) were the most frequently used therapies of GEP-NENs with reported grading. Conclusion. In general, Portuguese patients with GEP-NENs presented similar characteristics to other populations described in the literature. This cross-sectional study represents the first step to establish a national database of GEP-NENs that may aid in understanding the clinical and epidemiological features of these tumours in Portugal.http://dx.doi.org/10.1155/2019/4518742
spellingShingle A. P. Santos
J. Vinagre
P. Soares
I. Claro
A. C. Sanches
L. Gomes
I. Fernandes
A. L. Catarino
J. Preto
B. D. Pereira
A. P. Marques
F. Rodrigues
C. Amaral
G. Rocha
J. C. Mellidez
H. Simões
J. M. Lopes
M. J. Bugalho
On behalf of the NETs Study Group of the Portuguese Society of Endocrinology, Diabetes and Metabolism
Gastroenteropancreatic Neuroendocrine Neoplasia Characterization in Portugal: Results from the NETs Study Group of the Portuguese Society of Endocrinology, Diabetes and Metabolism
International Journal of Endocrinology
title Gastroenteropancreatic Neuroendocrine Neoplasia Characterization in Portugal: Results from the NETs Study Group of the Portuguese Society of Endocrinology, Diabetes and Metabolism
title_full Gastroenteropancreatic Neuroendocrine Neoplasia Characterization in Portugal: Results from the NETs Study Group of the Portuguese Society of Endocrinology, Diabetes and Metabolism
title_fullStr Gastroenteropancreatic Neuroendocrine Neoplasia Characterization in Portugal: Results from the NETs Study Group of the Portuguese Society of Endocrinology, Diabetes and Metabolism
title_full_unstemmed Gastroenteropancreatic Neuroendocrine Neoplasia Characterization in Portugal: Results from the NETs Study Group of the Portuguese Society of Endocrinology, Diabetes and Metabolism
title_short Gastroenteropancreatic Neuroendocrine Neoplasia Characterization in Portugal: Results from the NETs Study Group of the Portuguese Society of Endocrinology, Diabetes and Metabolism
title_sort gastroenteropancreatic neuroendocrine neoplasia characterization in portugal results from the nets study group of the portuguese society of endocrinology diabetes and metabolism
url http://dx.doi.org/10.1155/2019/4518742
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