title: Adrenocortical carcinoma: a population-based study on incidence and survival in the Netherlands since 1993.
abstract: BACKGROUND
  The reported annual incidence of adrenocortical carcinoma (ACC) is 0.5-2.0 cases per million individuals. Updated population-based studies on incidence are lacking. The aim of this nationwide survey was to describe the incidence and survival rates of ACC in the Netherlands. Secondary objectives were to evaluate changes in both survival rates and the number of patients undergoing surgery.
  
  
  METHODS
  All ACC patients registered in the Netherlands Cancer Registry (NCR) between 1993 and 2010 were included. Data on demographics, stage of disease, primary treatment modality and survival were evaluated.
  
  
  RESULTS
  Included were 359 patients, 196 of whom were female (55%). Median age at diagnosis was 56 years (range 1-91). The 5-year age-standardised incidence rate decreased from 1.3 to 1.0 per one million person-years. Median survival for patients with stage I-II, stage III and stage IV disease was 159 months (95% confidence interval (CI) 93-225 months), 26 months (95% CI: 4-48 months) and 5 months (95% CI: 2-7 months), respectively (P<0.001). Improvement in survival was not observed, as reflected by the lack of association between survival and time of diagnosis. The percentage of patients receiving treatment within 6 months after diagnosis increased significantly from 76% in 1993-1998 to 88% in 2005-2010 (P=0.047), mainly due to an increase in surgery for stage III-IV patients.
  
  
  CONCLUSIONS
  These nationwide data provide an up-to-date survey of the epidemiology of ACC in the Netherlands. A trend towards a decreasing overall incidence rate was observed. Survival rates did not change during this period despite an increased number of surgical procedures.
authors.affiliation: Department of Internal Medicine, Máxima Medical Centre, Veldhoven, Eindhoven/Veldhoven, The Netherlands. Electronic address: T.Kerkhofs@mmc.nl.
authors.forename: Thomas M A
authors.initials: TM
authors.lastname: Kerkhofs
authors.affiliation: Department of Research, Eindhoven Cancer Registry, Comprehensive Cancer Centre South, Eindhoven, The Netherlands.
authors.forename: Rob H A
authors.initials: RH
authors.lastname: Verhoeven
authors.affiliation: Department of Registry and Research, Comprehensive Cancer Centre the Netherlands, Utrecht, The Netherlands.
authors.forename: Jan Maarten
authors.initials: JM
authors.lastname: Van der Zwan
authors.affiliation: MMC Academy, Máxima Medical Centre, Veldhoven, Eindhoven/Veldhoven, The Netherlands.
authors.forename: Jeanne
authors.initials: J
authors.lastname: Dieleman
authors.affiliation: Department of Endocrinology, University of Groningen, University Medical Center Groningen, The Netherlands.
authors.forename: Michiel N
authors.initials: MN
authors.lastname: Kerstens
authors.affiliation: Department of Endocrinology, University of Groningen, University Medical Center Groningen, The Netherlands.
authors.forename: Thera P
authors.initials: TP
authors.lastname: Links
authors.affiliation: Department of Research, Eindhoven Cancer Registry, Comprehensive Cancer Centre South, Eindhoven, The Netherlands; CoRPS - Center of Research on Psychology in Somatic diseases, Department of Medical Psychology and Neuropsychology, Tilburg University, The Netherlands.
authors.forename: Lonneke V
authors.initials: LV
authors.lastname: Van de Poll-Franse
authors.affiliation: Department of Internal Medicine, Máxima Medical Centre, Veldhoven, Eindhoven/Veldhoven, The Netherlands.
authors.forename: Harm R
authors.initials: HR
authors.lastname: Haak
mesh_terms: D000293:Adolescent; D018268:Adrenocortical Carcinoma / Q000453:epidemiology* / Q000401:mortality; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D015994:Incidence; D007223:Infant; D008297:Male; D008875:Middle Aged; D009426:Netherlands / Q000453:epidemiology; D012042:Registries; D015996:Survival Rate; D055815:Young Adult