Abstract
BackgroundThe worldwide prevalence and incidence of neuroendocrine neoplasms (NEN) have been increasing recently, although few studies have analyzed data on the current situation of NENs in Japan. Here, the Japan Neuroendocrine Tumor Society (JNETS) planned to investigate the recent incidence and distribution of these tumors using data from the national cancer registry started in 2016. This study examined the incidence and distribution of primary sites as well as rate of advanced disease from this population-based registry.MethodsA retrospective, population-based study using data from the national cancer registry in Japan (NCR) was conducted to evaluate patients with gastro-entero-pancreatic NEN (GEP-NEN) in 2016. Associated population data were used to determine annual age-adjusted incidences.ResultsA total of 6735 individuals were diagnosed with GEP-NEN in Japan in 2016. Annual onset incidence was 0.70/100,000 for pancreatic NEN and 2.84/100,000 for gastrointestinal NEN. NEN in the ileum accounted for only 1% of total GEP-NENs in Japan. Most NENs in the esophagus or lungs were neuroendocrine carcinomas (NECs), while the majority of those in the duodenum, ileum, appendix and rectum were grade 1 neuroendocrine tumors (NETs). Median age at initial diagnosis was in between 60 to 65. Tumors in the duodenum, appendix and rectum were mostly limited to local, while those in the esophagus, stomach and colon tended to show distant metastasis. In Japan, initial treatment for GEP-NENs was resection even if the tumor was NEC.ConclusionsThis is the first report of a national registry-based incidence and distribution of GEP-NEN in Japan. These data will serve as an important first step to determining the exact etiology and trends for this pathology in Japan.
Highlights
The worldwide prevalence and incidence of neuroendocrine neoplasms (NEN) have been increasing recently, few studies have analyzed data on the current situation of NENs in Japan
Epidemiology of gastro-entero-pancreatic NEN (GEP-NEN) in Japan in 2016 Based on data derived from the NCR2016, the total number of patients treated for GEP-NENs in 2016 was recorded as 6735 and the age-adjusted overall incidence was 3.53 per 100,000 population
For tumor location of pancreatic NEN (PanNEN), 36.0% of all PanNEN patients had the lesion in the pancreatic head, 29.7% in the pancreatic body, and 28.7% in the pancreatic tail
Summary
The worldwide prevalence and incidence of neuroendocrine neoplasms (NEN) have been increasing recently, few studies have analyzed data on the current situation of NENs in Japan. The Japan Neuroendocrine Tumor Society (JNETS) planned to investigate the recent incidence and distribution of these tumors using data from the national cancer registry started in 2016. The same group conducted a second survey in 2010 and reported increased incidences of 1.25 per 100,000 for PanNEN and 4.35 per 10, 000 for GI-NEN [5]. These analyses provided numerous important new insights, such as the low incidence of ileal NEN in Japan, the data were based on questionnaire surveys. A more precise analysis of NEN patients in Japan has been necessary to clarify the status quo of NEN diagnosis/treatment in Japan
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