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

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.

Clinicopathologic Characteristics of Gastroenteropancreatic Neuroendocrine Tumors: Experience of a National Referral Hospital in Indonesia

“…Of the NETs, G2 was the most frequent followed by G1 and G3. These results are different from other studies reported, which reported a higher frequency of low-grade tumors than high-grade tumors [7], [10], [12]. However, we are limited in sample size and study population, and thus, a nationwide epidemiology study might not be comparable to ours.…”

“…The most frequent primary site in our study was the rectum, followed by the pancreas. Santos et al reported a study from Portugal that showed that GEP-NET predominantly arises from the pancreas followed by the jejunum-ileum, stomach, and rectum [10]. Similar results were reported in a multicenter study by Cong et al that the most common primary site was the pancreas followed by the rectum [11].…”

“…The most common primary site from which the tumor disseminated to other organs was the small intestine, followed by the colon, rectum, and pancreas [6]. The previous study also reported that the most common tumor stage was disseminated, followed by localized and locoregional extension, with the liver as the most common metastatic site [10]. Most tumors in our study were localized.…”

“…In our study, the organ with the largest proportion of negative chromogranin A expression was the large intestine. Chromogranin A, which can be negative, especially in the rectum, may become focally positive in G3 NETs and NECs [10]. Kimura reported that stage, grade, metastasis, and Ki67 index were significantly related to survival.…”

Gastroenteropancreatic Neuroendocrine Tumors (GEPNET) Registry 2009-2018: A Large Multi-country Registry with Over 1000 GEPNET Patients

“…In the Asia-Paci c and Middle East regions as well as in Turkey and South Africa, there is a paucity of published information on the epidemiology and treatment of GEP-NETs [11][12][13]. In addition, there is a lack of consensus on the diagnosis and treatment of GEP-NETs in these areas which may contribute to the late diagnosis, inadequate treatment, underestimation of GEP-NET prevalence and inability to prioritize treatment from the available options.…”