Gastroenteropancreatic neuroendocrine tumours: an overview

摘要

The incidence of GEP-NET has more recently been reported to be 5.25/100000 (Vinik et al, 2014).Therefore, in the UK some 3000 newly diagnosed patients each year can be expected. Prolonged survival further contributes to a raising prevalence. Median survival in metastatic NET of pancreatic origin has been reported to be between 4.7 and 7.4 years (Jann et al, 201 l;Ter-Minassian et al, 2013). In well-differentiated midgut NET, even with metastatic liver disease, survival appears to be even better, in some of the studies over 10 years (Jann et al, 2011;Ter-Minassian et al, 2013). Prime age for developing a GEP-NET is the sixth and seventh decade, with the exception of often benign-behaving, well-differentiated NET of the appendix, most frequently incidentally diagnosed following appendectomy (3-5/1000 appendectomies) (Pape et al, 2016) (a condition that often presents also in young patients). In paediatric patients with appendiceal NET, long-term outcomes are excellent, even in higher stages, with the reasons for better prognosis not being completely understood (Pape et al, 2016). Apart from tumours of rectum origin, GEP-NET appear to be less common in South Asian and Afro-Caribbean populations (Vinik et al, 2010). The frequency of occurrence of GEP-NET according to the location of the primary tumour is shown in Table 1.