title: Surgery for adrenocortical carcinoma in The Netherlands: analysis of the national cancer registry data.
abstract: OBJECTIVE
  Adrenocortical carcinoma (ACC) is a rare disease with an estimated incidence of one to two cases per 1 million inhabitants. The Dutch Adrenal Network (DAN) was initiated with the aim to improve patient care and to stimulate scientific research on ACC. Currently, not all patients with ACC are treated in specialized DAN hospitals. The objective of the current investigation was to determine whether there are differences in survival between patients operated on in DAN hospitals and those operated on in non-DAN hospitals.
  
  
  METHODS
  The study was set up as a retrospective and population-based survival analysis.
  
  
  METHODS
  Data on all adult ACC patients diagnosed between 1999 and 2009 were obtained from The Netherlands Cancer Registry (NCR). Overall survival was calculated and a comparison was made between DAN and non-DAN hospitals.
  
  
  RESULTS
  The NCR contained data of 189 patients. The median survival of patients with European Network for the Study of Adrenal Tumors stages I-III disease was significantly longer for patients operated on in a DAN hospital (n=46) than for those operated on in a non-DAN hospital (n=37, 5-year survival 63 vs 42%). Survival remained significantly different after correction for sex, age, year of diagnosis, and stage of disease in the multivariate analysis (hazard ratio 1.96 (95% CI 1.01-3.81), P=0.047).
  
  
  CONCLUSIONS
  The results associate surgery in a DAN center with a survival benefit for patients with local or locally advanced ACC. We hypothesize that a multidisciplinary approach for these patients explains the observed survival benefit. These findings should be carefully considered in view of the aim for further centralization of ACC treatment.
authors: Department of Internal Medicine, Máxima Medical Center, Ds. Theodor Fliednerstraat 1, 5631 BM Eindhoven, Veldhoven, The Netherlands. t.kerkhofs@mmc.nl
authors: T M A
authors: TM
authors: Kerkhofs
authors: R H A
authors: RH
authors: Verhoeven
authors: H J
authors: HJ
authors: Bonjer
authors: E J Nieveen
authors: EJ
authors: van Dijkum
authors: M R
authors: MR
authors: Vriens
authors: J
authors: J
authors: De Vries
authors: C H
authors: CH
authors: Van Eijck
authors: B A
authors: BA
authors: Bonsing
authors: L V
authors: LV
authors: Van de Poll-Franse
authors: H R
authors: HR
authors: Haak
mesh_terms: D000306:Adrenal Cortex Neoplasms / Q000401:mortality* / Q000473:pathology / Q000601:surgery*; D018268:Adrenocortical Carcinoma / Q000401:mortality* / Q000473:pathology / Q000601:surgery*; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009367:Neoplasm Staging; D009426:Netherlands / Q000453:epidemiology; D016016:Proportional Hazards Models; D012042:Registries; D012189:Retrospective Studies; D016019:Survival Analysis; D016896:Treatment Outcome