title: Improving Outcomes in Adrenocortical Cancer: An Australian Perspective.
abstract: BACKGROUND
  Adrenocortical carcinoma (ACC) is a rare malignancy that carries a poor prognosis. There has yet to be a large Australian series that documents the characteristics of ACC and there are a paucity of data on management and the long-term outcomes. We sought to provide a unique insight into the management of ACC in Australia as well as to identify factors associated with prognosis and survival.
  
  
  METHODS
  A multivariate analysis of a cohort of patients identified with ACC between 1998 and 2013 was undertaken. Recurrence-free survival (RFS) and overall survival (OS) were assessed as the main outcome measures and correlated with multiple clinical variables in order to identify prognostic markers.
  
  
  RESULTS
  Of the 104 patients identified, a total of 98 patients with complete clinical and outcome data were included in the study. Median OS was 56 months, with the 5-year survival being 48 % (95 % confidence interval 36-59). On multivariate analysis, age ≥50 years, metastases at presentation, and evidence of extra-adrenal invasion were found to be statistically associated with reduced OS. RFS was analyzed in patients without metastases. On multivariate analysis, extra-adrenal invasion and no preoperative endocrine investigations were found to be statistically significant poor prognostic factors, with a non-significant trend for higher individual surgeon volume to be associated with improved resection margins and RFS.
  
  
  CONCLUSIONS
  We present clinical outcomes and prognostic factors for patients with ACC in a landmark Australian series. We suggest that management in a specialized tertiary endocrine and/or surgical oncology unit is more likely to lead to improved outcomes.
authors.affiliation: Cancer Genetics, Kolling Institute of Medical Research, Royal North Shore Hospital and The University of Sydney, Sydney, NSW, Australia.
authors.forename: Julian C Y
authors.initials: JC
authors.lastname: Ip
authors.forename: Tony C Y
authors.initials: TC
authors.lastname: Pang
authors.forename: Anthony R
authors.initials: AR
authors.lastname: Glover
authors.forename: Patsy
authors.initials: P
authors.lastname: Soon
authors.forename: Stephen
authors.initials: S
authors.lastname: Clarke
authors.forename: Arthur
authors.initials: A
authors.lastname: Richardson
authors.forename: Peter
authors.initials: P
authors.lastname: Campbell
authors.forename: Bruce G
authors.initials: BG
authors.lastname: Robinson
authors.forename: Stan B
authors.initials: SB
authors.lastname: Sidhu
mesh_terms: D000306:Adrenal Cortex Neoplasms / Q000401:mortality* / Q000473:pathology / Q000628:therapy*; D001315:Australia; D015331:Cohort Studies; D003131:Combined Modality Therapy; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008207:Lymphatic Metastasis; D008297:Male; D008875:Middle Aged; D009361:Neoplasm Invasiveness; D009364:Neoplasm Recurrence, Local / Q000401:mortality* / Q000473:pathology / Q000628:therapy*; D009367:Neoplasm Staging; D011379:Prognosis; D015996:Survival Rate