Epidemiology and classification of gastroenteropancreatic neuroendocrine neoplasms using current coding criteria

Abstract

BackgroundThe lack of uniform criteria for coding of gastroenteropancreatic neuroendocrine neoplasia (GEP‐NEN) has hampered previous epidemiological studies. The epidemiology of GEP‐NEN was investigated in this study using currently available criteria.MethodsAll patients diagnosed with GEP‐NEN between January 2003 and December 2013 in a well defined Norwegian population of approximately 350 000 people were included. Age‐ and sex‐adjusted incidence rates were calculated. The current 2010 World Health Organization criteria, European Neuroendocrine Tumour Society classification and International Union Against Cancer (UICC) classification were used.ResultsA total of 204 patients (114 male, 55·9 per cent) were identified. The median age at diagnosis was 61 (range 10–94) years. The annual overall crude incidence was 5·83 per 100 000 inhabitants, with an increasing trend (P = 0·033). The most frequent location was small intestine (60 patients, 29·4 per cent) followed by appendix (48 patients, 23·5 per cent) and pancreas (33 patients, 16·2 per cent). Grade 1 tumours were more common in gastrointestinal (100 patients, 58·5 per cent) than in pancreatic (9 patients, 27 per cent) NEN. According to the UICC classification, 77 patients (37·7 per cent) had stage I, 17 patients (8·3 per cent) stage II, 37 patients (18·1 per cent) stage III and 70 patients (34·3 per cent) had stage IV disease. No patient with stage I disease had grade 3 tumours; advanced tumour grade increased with stage.ConclusionA high crude incidence of GEP‐NEN, at 5·83 per 100 000 inhabitants, was noted together with a significant increasing trend over time.

Nomogram predicting the risk of recurrence after curative‐intent resection of primary non‐metastatic gastrointestinal neuroendocrine tumors: An analysis of the U.S. Neuroendocrine Tumor Study Group

“…Gastro‐entero‐pancreatic neuroendocrine tumors (GEP‐NET) are a heterogeneous group of tumors that originate from the diffuse neuroendocrine cell system of the gastrointestinal tract and pancreas . Even though GEP‐NET have classically been considered rare neoplasia, recent data have suggested an exponential increase in the incidence of GEP‐NET over the last several decades .…”

Patterns of Recurrence after Resection for Pancreatic Neuroendocrine Tumors: Who, When, and Where?

“…Although these tumors account for only 1–5% of all pancreatic tumors [1-4], pan-NENs are among the main indications for pancreatic resection after pancreatic ductal adenocarcinoma (PDAC) and pancreatic cystic neoplasms [5-10]. Over the past 2 decades, the increased diagnoses of pan-NENs [11] have generated great interest, leading to the development of various classifications and grading and staging systems [12-15] as well as definition of international guidelines for management of the disease [16-18]. Unlike PDAC, pan-NENs encompass a wide spectrum of disease entities, ranging from indolent and asymptomatic neoplasms to severely symptomatic or biologically aggressive cancers.…”

Risk factors associated with gastroenteropancreatic neuroendocrine tumors in a cohort of 2.3 million Israeli adolescents

“…Neuroendocrine gastroenteropancreatic tumors (GEP‐NETs) are neoplasms that form in either well‐differentiated neuroendocrine cells or from their precursors (stem cells) . Data from the SEER (Surveillance, Epidemiology and End Results Program, USA) as well as from other countries suggest that the incidence and prevalence of NETs have increased substantially over the past three decades . This is largely due to greater awareness of the disease among physicians, including pathologists and improved diagnostic techniques, but the incidence may be also be rising .…”