title: Surgery is associated with improved survival for adrenocortical cancer, even in metastatic disease.
abstract: BACKGROUND
  Adrenocortical carcinoma (ACC) is a rare but lethal tumor. Predictors of survival include earlier stage at presentation and complete operative resection. We assessed effect of treatment and demographic variables on survival.
  
  
  METHODS
  ACC cases were abstracted from the California Cancer Registry and Office of Statewide Health Planning and Development (1999-2008). Predictors included patient demographics, comorbidities, tumor size, stage, and treatment (none, surgery, chemotherapy and/or radiation [CRT], and surgery plus CRT).
  
  
  RESULTS
  We studied 367 patients with median tumor size of 10 cm. At presentation, 37% had localized, 17% had regional, and 46% had metastatic disease. Median survival was 1.7 years (7.4 years local, 2.6 years regional, and 0.3 years metastatic, P < .0001). One-year and 5-year survival was: 92%/62% (local); 73%/39% (regional); and 24%/7% (metastatic). Increased age (hazard ratio [HR] 1.16) and Cushing's syndrome (HR 1.66) worsened survival (P < .05). Low socioeconomic status worsened survival in local and regional disease (P < .05). In multivariable regression, both surgery (regional HR 0.13; metastatic HR 0.52) and surgery plus CRT (regional HR 0.15; metastatic HR 0.31) improved survival compared with no treatment (P < .02).
  
  
  CONCLUSIONS
  In ACC, surgery is associated with improved survival, even in metastatic disease. Surgery should be considered for select patients as part of multimodality treatment.
authors.affiliation: Section of Endocrine Surgery, UCLA David Geffen School of Medicine, Los Angeles, CA.
authors.forename: Masha
authors.initials: M
authors.lastname: Livhits
authors.affiliation: Department of Biomathematics, University of California, Los Angeles, Los Angeles, CA.
authors.forename: Ning
authors.initials: N
authors.lastname: Li
authors.affiliation: Section of Endocrine Surgery, UCLA David Geffen School of Medicine, Los Angeles, CA.
authors.forename: Michael W
authors.initials: MW
authors.lastname: Yeh
authors.affiliation: Section of Endocrine Surgery, UCLA David Geffen School of Medicine, Los Angeles, CA. Electronic address: aharari@mednet.ucla.edu.
authors.forename: Avital
authors.initials: A
authors.lastname: Harari
mesh_terms: D000306:Adrenal Cortex Neoplasms / Q000401:mortality* / Q000473:pathology / Q000601:surgery*; D000315:Adrenalectomy / Q000379:methods*; D018268:Adrenocortical Carcinoma / Q000401:mortality* / Q000556:secondary / Q000601:surgery*; D000328:Adult; D000368:Aged; D002140:California; D015331:Cohort Studies; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D053208:Kaplan-Meier Estimate; D016015:Logistic Models; D008297:Male; D008875:Middle Aged; D015999:Multivariate Analysis; D009361:Neoplasm Invasiveness / Q000473:pathology; D009362:Neoplasm Metastasis; D009367:Neoplasm Staging; D011379:Prognosis; D016016:Proportional Hazards Models; D012042:Registries; D012189:Retrospective Studies; D018570:Risk Assessment; D016019:Survival Analysis; D016896:Treatment Outcome