title: Trends in incidence and management of cancer of the ampulla of Vater.
abstract: OBJECTIVE
  To provide trends in incidence, management and survival of cancer of the ampulla of Vater in a well-defined French population.
  
  
  METHODS
  Data were obtained from the population-based digestive cancer registry of Burgundy over a 34-year period. Age-standardized incidence rates were computed using the world standard population. Average annual variations in incidence rates were estimated using a poisson regression. A univariate and multivariate relative survival analysis was performed.
  
  
  RESULTS
  Age-standardized incidence rates were 0.46 and 0.30 per 100000 inhabitants for men and women, respectively. Incidence rate increased from 0.26 (1976-1984) to 0.58 (2003-2009) for men and remained stable for women. Resection for cure was performed in 48.3% of cases. This proportion was stable over the study period. Among cases with curative resection, pancreatico-duodenectomy was performed in 94.0% of cases and ampullectomy in 6.0% of cases. A total of 50.8% of cancers of the ampulla of Vater were diagnosed at an advanced stage. Their proportion remained stable throughout the study period. The overall 1- and 5-year relative survival rates were 60.2% and 27.7%, respectively. Relative survival did not vary over time. Treatment and stage at diagnosis were the most important determinants of survival. The 5-year relative survival rate was 41.5% after resection for cure, 9.5% after palliative surgery and 6.7% after symptomatic treatment. In multivariate analysis, only stage at diagnosis significantly influenced the risk of death.
  
  
  CONCLUSIONS
  Cancer of the ampulla of Vater is still uncommon, but its incidence increased for men in Burgundy. Diagnosis is often made at an advanced stage, dramatically worsening the prognosis.
authors.affiliation: Florian Rostain, Samia Hamza, Antoine Drouillard, Jean Faivre, Anne-Marie Bouvier, Côme Lepage, Registre Bourguignon des Cancers Digestifs, INSERM U866, CHU de Dijon, Faculté de Médecine, 21079 Dijon Cedex, France.
authors.forename: Florian
authors.initials: F
authors.lastname: Rostain
authors.affiliation: Florian Rostain, Samia Hamza, Antoine Drouillard, Jean Faivre, Anne-Marie Bouvier, Côme Lepage, Registre Bourguignon des Cancers Digestifs, INSERM U866, CHU de Dijon, Faculté de Médecine, 21079 Dijon Cedex, France.
authors.forename: Samia
authors.initials: S
authors.lastname: Hamza
authors.affiliation: Florian Rostain, Samia Hamza, Antoine Drouillard, Jean Faivre, Anne-Marie Bouvier, Côme Lepage, Registre Bourguignon des Cancers Digestifs, INSERM U866, CHU de Dijon, Faculté de Médecine, 21079 Dijon Cedex, France.
authors.forename: Antoine
authors.initials: A
authors.lastname: Drouillard
authors.affiliation: Florian Rostain, Samia Hamza, Antoine Drouillard, Jean Faivre, Anne-Marie Bouvier, Côme Lepage, Registre Bourguignon des Cancers Digestifs, INSERM U866, CHU de Dijon, Faculté de Médecine, 21079 Dijon Cedex, France.
authors.forename: Jean
authors.initials: J
authors.lastname: Faivre
authors.affiliation: Florian Rostain, Samia Hamza, Antoine Drouillard, Jean Faivre, Anne-Marie Bouvier, Côme Lepage, Registre Bourguignon des Cancers Digestifs, INSERM U866, CHU de Dijon, Faculté de Médecine, 21079 Dijon Cedex, France.
authors.forename: Anne-Marie
authors.initials: AM
authors.lastname: Bouvier
authors.affiliation: Florian Rostain, Samia Hamza, Antoine Drouillard, Jean Faivre, Anne-Marie Bouvier, Côme Lepage, Registre Bourguignon des Cancers Digestifs, INSERM U866, CHU de Dijon, Faculté de Médecine, 21079 Dijon Cedex, France.
authors.forename: Côme
authors.initials: C
authors.lastname: Lepage
mesh_terms: D000293:Adolescent; D000328:Adult; D017677:Age Distribution; D000367:Age Factors; D000368:Aged; D000369:Aged, 80 and over; D014670:Ampulla of Vater / Q000473:pathology / Q000601:surgery*; D001662:Biliary Tract Surgical Procedures / Q000009:adverse effects / Q000401:mortality / Q000639:trends*; D016009:Chi-Square Distribution; D002648:Child; D002675:Child, Preschool; D003138:Common Bile Duct Neoplasms / Q000175:diagnosis / Q000453:epidemiology* / Q000401:mortality / Q000628:therapy*; D005260:Female; D005602:France / Q000453:epidemiology; D006801:Humans; D015994:Incidence; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D008875:Middle Aged; D015999:Multivariate Analysis; D009367:Neoplasm Staging; D010166:Palliative Care / Q000639:trends*; D016577:Pancreaticoduodenectomy / Q000009:adverse effects / Q000401:mortality / Q000639:trends*; D016016:Proportional Hazards Models; D011446:Prospective Studies; D012042:Registries; D012307:Risk Factors; D017678:Sex Distribution; D012737:Sex Factors; D013997:Time Factors; D016896:Treatment Outcome; D055815:Young Adult
keywords: Cancer of the ampulla of Vater; Epidemiology; Incidence; Survival; Treatment
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