title: Diagnosis, treatment and outcome of adrenocortical cancer.
abstract: BACKGROUND
  Adrenocortical cancer (ACC) is a rare disease with a dismal prognosis. The majority of patients are diagnosed with advanced disease and raise difficult management challenges.
  
  
  METHODS
  All references identified in PubMed, published between 2004 and 2014, using the keywords 'adrenocortical cancer' or 'adrenal surgery' or both, were uploaded into a database. The database was interrogated using keywords specific for each field studied.
  
  
  RESULTS
  In all, 2049 publications were identified. There is ongoing debate about the feasibility and oncological outcomes of laparoscopic adrenalectomy for small ACCs, and data derived from institutional case series have failed to provide an evidence level above expert opinion. The use of mitotane (1-(2-chlorophenyl)-1-(4-chlorophenyl)-2,2-dichloroethane) in combination with chemotherapy in the treatment of metastatic disease has been assessed in an international randomized trial (FIRM-ACT trial) involving patients with ACC. Based on this trial, mitotane plus etoposide, doxorubicin and cisplatin is now the established first-line cytotoxic therapy owing to a higher response rate and longer median progression-free survival than achieved with streptozocin-mitotane. For patients with tumours smaller than 5 cm and with no signs of lymph node or distant metastases, survival is favourable with a median exceeding 10 years. However, the overall 5-year survival rate for all patients with ACC is only 30 per cent.
  
  
  CONCLUSIONS
  Open and potentially laparoscopic adrenalectomy for selected patients is the main treatment for non-metastatic ACC, but the overall 5-year survival rate remains low.
authors.affiliation: Department of Endocrine Surgery, Oxford University Hospitals NHS Trust, Oxford, UK.
authors.forename: R
authors.initials: R
authors.lastname: Mihai
mesh_terms: D000306:Adrenal Cortex Neoplasms / Q000175:diagnosis / Q000401:mortality / Q000628:therapy*; D000315:Adrenalectomy / Q000706:statistics & numerical data; D018268:Adrenocortical Carcinoma / Q000175:diagnosis / Q000401:mortality / Q000628:therapy*; D000328:Adult; D018931:Antineoplastic Agents, Hormonal / Q000627:therapeutic use; D014408:Biomarkers, Tumor / Q000378:metabolism; D017024:Chemotherapy, Adjuvant; D002648:Child; D005260:Female; D006801:Humans; D010535:Laparoscopy / Q000706:statistics & numerical data; D008297:Male; D059146:Metastasectomy / Q000706:statistics & numerical data; D008939:Mitotane / Q000627:therapeutic use; D009154:Mutation; D009364:Neoplasm Recurrence, Local / Q000401:mortality / Q000628:therapy; D009367:Neoplasm Staging; D011379:Prognosis; D015996:Survival Rate
chemical_list: D018931:Antineoplastic Agents, Hormonal; D014408:Biomarkers, Tumor; D008939:Mitotane