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

• Type: Article in International Scientific Journal
• Date: 2019

Authors

Santos, AP (Author) Other; Vinagre, J (Author) Other; Soares, P (Author) FMUP; Claro, I (Author) Other; Sanches, AC (Author) Other; Gomes, L (Author) Other; Fernandes, I (Author) Other; Catarino, AL (Author) Other; John Preto (Author) Other; Pereira, BD (Author) Other; Marques, AP (Author) Other; Rodrigues, F (Author) Other; Amaral, C (Author) Other; Rocha, G (Author) Other; Mellidez, JC (Author) Other; Simoes, H (Author) Other; Lopes, JM (Author) FMUP; Bugalho, MJ (Author) Other

Journal

Title: International Journal of Endocrinology

Vol. 2019

Pages: 1-10

ISSN: 1687-8337

Publisher: Hindawi

Indexing

ISI Web of Knowledge - 3 Citations

Scopus - 3 Citations

Other information

Authenticus ID: P-00R-033

DOI: 10.1155/2019/4518742

Abstract (EN): 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.

Language: English

Type (Professor's evaluation): Scientific

No. of pages: 10