title: Incidence and management of malignant digestive endocrine tumours in a well defined French population.
abstract: OBJECTIVE
  Little is known about the epidemiology of malignant digestive endocrine tumours. The aim of this study was to report on their incidence and management in a well defined population.
  
  
  METHODS
  Data were obtained from the population based Digestive Cancer Registry of Burgundy (France) over a 24 year period. Incidence rates were calculated by sex, age groups, and period of diagnosis. Treatment and stage at diagnosis were also investigated. Prognosis was determined using crude and relative survival rates. A multivariate relative survival analysis was performed.
  
  
  RESULTS
  Between 1976 and 1999, 229 cases were recorded. Age standardised incidence rates were 0.76/100,000 for men and 0.50/100,000 for women. They increased over time in both sexes. The resectability rate was 74.1%. Among recorded cases, 26.6% did not extend beyond the organ, 20% had lymph node metastases, and 53.3% had visceral metastases or were unresectable. There was no improvement in the resection rate or in the stage at diagnosis over the study period. The overall relative survival rate was 66.9% at one year, 50.4% at five years, and 40.6% at 10 years. Stage at diagnosis, age at diagnosis, and subsite were independent significant prognostic factors.
  
  
  CONCLUSIONS
  Although their incidence is increasing, malignant digestive endocrine tumours remain a rare cancer, representing 1% of digestive cancers. Stage at diagnosis and prognosis at a population level are worse than those reported in hospital series. In the short term, new therapeutic possibilities represent the best way to improve their prognosis.
authors.affiliation: Faculté de Médecine, Registre Bourguignon des Cancers Digestifs, INSERM EPI 0106, Dijon, France. jean.faivre@u-bourgogne.fr
authors.forename: C
authors.initials: C
authors.lastname: Lepage
authors.forename: A M
authors.initials: AM
authors.lastname: Bouvier
authors.forename: J M
authors.initials: JM
authors.lastname: Phelip
authors.forename: C
authors.initials: C
authors.lastname: Hatem
authors.forename: C
authors.initials: C
authors.lastname: Vernet
authors.forename: J
authors.initials: J
authors.lastname: Faivre
mesh_terms: D000293:Adolescent; D000328:Adult; D017677:Age Distribution; D000368:Aged; D000369:Aged, 80 and over; D004067:Digestive System Neoplasms / Q000453:epidemiology* / Q000473:pathology / Q000628:therapy; D005260:Female; D005602:France / Q000453:epidemiology; D006801:Humans; D015994:Incidence; D008297:Male; D008875:Middle Aged; D018358:Neuroendocrine Tumors / Q000453:epidemiology* / Q000473:pathology / Q000628:therapy; D011379:Prognosis; D012042:Registries; D017678:Sex Distribution; D016019:Survival Analysis; D016896:Treatment Outcome