title: Prognostic parameters of metastatic adrenocortical carcinoma.
abstract: BACKGROUND
  Prognostic parameters of metastatic adrenocortical carcinoma (ACC) are poorly characterized.
  
  
  OBJECTIVE
  The objective of the study was to describe the clinical presentation of metastatic ACC and determine prognostic factors for survival.
  
  
  METHODS
  This was a retrospective cohort study (1988-2004).
  
  
  METHODS
  The study was conducted in an institutional practice.
  
  
  METHODS
  Participants included 124 consecutive patients with metastatic ACC, 70 from Gustave-Roussy Institute (main cohort) and 54 patients from the Cochin Hospital (validation cohort). Clinical data concerning all patients, histopathologic slides of primary tumors (44 in the main cohort and 40 in the validation cohort), and molecular biology data on 15 primary tumors (main cohort) were analyzed.
  
  
  METHODS
  There was no intervention.
  
  
  RESULTS
  The main outcome was the specific survival after discovery of the first metastasis (Kaplan-Meier method). This included univariate analysis on the main cohort, confirmed on the validation cohort and then analyzed in a multivariate analysis.
  
  
  RESULTS
  In the main cohort, overall median survival was 20 months. In univariate analysis, the presence of hepatic and bone metastases, the number of metastatic lesions and the number of tumoral organs at the time of the first metastasis, a high mitotic rate (>20 per 50 high-power field), and atypical mitoses in the primary tumor predicted survival (P = 0.05, 0.003, 0.046, 0.001, 0.01, and < 0.001, respectively). The number of tumoral organs and a high mitotic rate were confirmed on the validation cohort (P = 0.009 and 0.03, respectively). These two parameters were confirmed in multivariate analysis (P = 0.0058 and 0.049).
  
  
  CONCLUSIONS
  Metastatic ACC is a heterogeneous disease with poor outcome. The combination of the number of tumoral organs at the time of the first metastasis and the mitotic rate can predict different outcomes.
authors.affiliation: Service de Médecine Nucléaire et de Cancérologie Endocrinienne, Institut Gustave-Roussy, Université Paris XI, 94800 Villejuif, France.
authors.forename: Guillaume
authors.initials: G
authors.lastname: Assié
authors.forename: Guillemette
authors.initials: G
authors.lastname: Antoni
authors.forename: Frédérique
authors.initials: F
authors.lastname: Tissier
authors.forename: Bernard
authors.initials: B
authors.lastname: Caillou
authors.forename: Gwenaelle
authors.initials: G
authors.lastname: Abiven
authors.forename: Christine
authors.initials: C
authors.lastname: Gicquel
authors.forename: Sophie
authors.initials: S
authors.lastname: Leboulleux
authors.forename: Jean-Paul
authors.initials: JP
authors.lastname: Travagli
authors.forename: Clarisse
authors.initials: C
authors.lastname: Dromain
authors.forename: Xavier
authors.initials: X
authors.lastname: Bertagna
authors.forename: Jérôme
authors.initials: J
authors.lastname: Bertherat
authors.forename: Martin
authors.initials: M
authors.lastname: Schlumberger
authors.forename: Eric
authors.initials: E
authors.lastname: Baudin
mesh_terms: D000293:Adolescent; D000306:Adrenal Cortex Neoplasms / Q000235:genetics / Q000401:mortality* / Q000473:pathology; D000328:Adult; D000368:Aged; D001859:Bone Neoplasms / Q000556:secondary; D015331:Cohort Studies; D005260:Female; D006801:Humans; D008113:Liver Neoplasms / Q000556:secondary; D019656:Loss of Heterozygosity; D008297:Male; D008875:Middle Aged; D015999:Multivariate Analysis; D011379:Prognosis; D012189:Retrospective Studies; D015996:Survival Rate