title: Gastric neuroendocrine tumors: prevalence in Europe, USA, and Japan, and rationale for treatment with a gastrin/CCK2 receptor antagonist.
abstract: OBJECTIVE
  Gastric carcinoids (neuroendocrine tumors) arise from enterochromaffin-like cells in the gastric mucosa. Most are caused by hypergastrinemia. The objectives were to determine if their prevalence in Europe, USA and Japan meets the criteria for an orphan disease and to justify treatment with a gastrin/CCK2 receptor antagonist.
  
  
  METHODS
  We obtained data from European and USA cancer registries, and searched PubMed.
  
  
  RESULTS
  Prevalence per 10,000 population obtained from cancer registries was: median 0.32 (range 0.09-0.92) for Europe; and 0.17 for the USA, equivalent to 4812 for the whole population. A PubMed search for gastric carcinoids yielded prevalence for Japan only, which was 0.05 per 10,000 population, equivalent to 665 for the entire population. A further search for gastric carcinoids in patients with pernicious anemia (PA) or autoimmune chronic atrophic gastritis (CAG), two presentations of about 80% of gastric carcinoids, produced prevalence rates of 5.2-11%. Prevalence of PA itself was 0.12-1.9%. Data on CAG epidemiology were sparse.
  
  
  CONCLUSIONS
  Prevalence of gastric carcinoids varied widely. All sources probably underestimate prevalence. However, prevalence was below the limits required for recognition by drug regulatory authorities as an orphan disease: 5 per 10,000 population of Europe; 200,000 for the whole population of the USA; and 50,000 for the whole population of Japan. Because gastric carcinoids are an orphan disease, and nonclinical and healthy volunteer studies support treatment with netazepide, a gastrin/CCK2 antagonist, netazepide has been designated an orphan medicinal product in Europe and the USA for development as targeted treatment for gastric carcinoids.
authors: Hammersmith Medicines Research, Central Middlesex Hospital , London NW10 7NS , England.
authors: Malcolm
authors: M
authors: Boyce
authors: Liv
authors: L
authors: Thomsen
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D000752:Anemia, Pernicious / Q000453:epidemiology; D001570:Benzodiazepinones / Q000627:therapeutic use*; D002276:Carcinoid Tumor / Q000188:drug therapy* / Q000453:epidemiology*; D019861:Enterochromaffin-like Cells / Q000378:metabolism; D005060:Europe / Q000453:epidemiology; D005260:Female; D005753:Gastric Mucosa / Q000378:metabolism; D005757:Gastritis, Atrophic / Q000453:epidemiology; D005770:Gastrointestinal Neoplasms / Q000188:drug therapy* / Q000453:epidemiology*; D006801:Humans; D007564:Japan / Q000453:epidemiology; D008297:Male; D010671:Phenylurea Compounds / Q000627:therapeutic use*; D015995:Prevalence; D035583:Rare Diseases; D043887:Receptor, Cholecystokinin B / Q000037:antagonists & inhibitors*; D017678:Sex Distribution; D014481:United States / Q000453:epidemiology
chemical_list: D001570:Benzodiazepinones; D010671:Phenylurea Compounds; D043887:Receptor, Cholecystokinin B; C104428:YF 476
keywords: autoimmune chronic atrophic gastritis; gastric carcinoids; gastrin/CCK2 receptor antagonist; hypergastrinemia; netazepide; pernicious anemia