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

Autor: Santos AP; Instituto Português de Oncologia do Porto, Francisco Gentil (IPOPFG), 4200-162 Porto, Portugal., Vinagre J; Instituto de Investigação e Inovação em Saúde (i3S), 4200-135 Porto, Portugal.; Instituto de Patologia e Imunologia Molecular da Universidade do Porto (IPATIMUP), 4200-465 Porto, Portugal.; Faculdade de Medicina da Universidade do Porto (FMUP), 4200-319 Porto, Portugal., Soares P; Instituto de Investigação e Inovação em Saúde (i3S), 4200-135 Porto, Portugal.; Instituto de Patologia e Imunologia Molecular da Universidade do Porto (IPATIMUP), 4200-465 Porto, Portugal.; Centro Hospitalar de São João (CHSJ), 4200-319 Porto, Portugal., Claro I; Centro Hospitalar de Lisboa Ocidental (CHLO), 1349-019 Lisboa, Portugal., Sanches AC; Instituto Português de Oncologia do Porto, Francisco Gentil (IPOPFG), 4200-162 Porto, Portugal., Gomes L; Centro Hospitalar e Universitário de Coimbra (CHUC), 3000-075 Coimbra, Portugal., Fernandes I; Centro Hospitalar Lisboa Norte, EPE (CHLN), 1649-035 Lisboa, Portugal.; Centro Académico de Medicina de Lisboa (CAML), 1649-035 Lisboa, Portugal., Catarino AL; Hospital da Luz, 1500-650 Lisboa, Portugal., Preto J; Faculdade de Medicina da Universidade do Porto (FMUP), 4200-319 Porto, Portugal.; Centro Hospitalar de São João (CHSJ), 4200-319 Porto, Portugal., Pereira BD; Hospital Garcia de Orta, EPE, 2801-951 Almada, Portugal., Marques AP; Unidade Local de Saúde de Matosinhos, 4464-513 Senhora da Hora, Portugal., Rodrigues F; Instituto Português de Oncologia de Coimbra, Francisco Gentil (IPOCFG), 3000-075 Coimbra, Portugal., Amaral C; Centro Hospitalar do Porto-Hospital Santo António, 4099-001 Porto, Portugal., Rocha G; Centro Hospitalar Gaia/Espinho (CHGE), 4434-502 Vila Nova de Gaia, Portugal., Mellidez JC; Centro Hospitalar do Baixo Vouga (CHBV), 3810-501 Aveiro, Portugal., Simões H; Centro Hospitalar de Lisboa Ocidental (CHLO), 1349-019 Lisboa, Portugal., Lopes JM; Instituto de Investigação e Inovação em Saúde (i3S), 4200-135 Porto, Portugal.; Instituto de Patologia e Imunologia Molecular da Universidade do Porto (IPATIMUP), 4200-465 Porto, Portugal.; Faculdade de Medicina da Universidade do Porto (FMUP), 4200-319 Porto, Portugal.; Centro Hospitalar de São João (CHSJ), 4200-319 Porto, Portugal., Bugalho MJ; Centro Hospitalar Lisboa Norte, EPE (CHLN), 1649-035 Lisboa, Portugal.; Centro Académico de Medicina de Lisboa (CAML), 1649-035 Lisboa, Portugal.
Jazyk: angličtina
Zdroj: International journal of endocrinology [Int J Endocrinol] 2019 Aug 01; Vol. 2019, pp. 4518742. Date of Electronic Publication: 2019 Aug 01 (Print Publication: 2019).
DOI: 10.1155/2019/4518742
Abstrakt: 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.
Competing Interests: The authors declare no conflict of interest.
Databáze: MEDLINE
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