Gastroenteropancreatic Neuroendocrine Neoplasia Characterization in Portugal: Results from the NETs Study Group of the Portuguese Society of Endocrinology, Diabetes and Metabolism. (1st August 2019)

• Record Type: Journal Article
• Authors: Santos, A. P.; Vinagre, J.; Soares, P.; Claro, I.; Sanches, A. C.; Gomes, L.; Fernandes, I.; Catarino, A. L.; Preto, J.; Pereira, B. D.; Marques, A. P.; Rodrigues, F.; Amaral, C.; Rocha, G.; Mellidez, J. C.; Simões, H.; Lopes, J. M.; Bugalho, M. J.
• Other Names: Reimondo Giuseppe Academic Editor.
• Is Part Of: International journal of endocrinology. Volume 2019(2019)
• Publication Date: 2019-08-01
• Subjects: Endocrinology -- Periodicals; Endocrinology; Endocrinology -- Periodicals; Endocrine System Diseases -- Periodicals; Periodicals; 616.4
• DOI: 10.1155/2019/4518742
• Languages: English
• ISSNs: 1687-8337

Abstract

Abstract : 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.