title: Adrenocortical carcinoma.
abstract: OBJECTIVE
  Adrenocortical carcinoma is a rare malignancy, accounting for 0.02% of all annual cancers reported. Given the generally advanced stage at diagnosis, the overall 5-year survival remains poor, varying between 20 and 45%. While older studies purported an improved outcome for functional tumors in adult patients, this has not been borne out in more recent studies. In the pediatric population, though, virilizing tumors carry a better survival than non-functional or cortisol-secreting tumors.
  
  
  RESULTS
  Recent studies focusing on the tumorigenesis of adrenocortical carcinoma have focused on onco-developmental genes present in the fetal adrenal cortex, as well as local adrenal paracrine and autocrine effects of cellular peptides.
  
  
  CONCLUSIONS
  Pre-operative diagnostic advances in positron emission scanning are emerging as promising modalities for confirmation of malignancy of indeterminate adrenal masses. No significant advances in the treatment of adrenocortical carcinoma have been developed. Surgery remains the mainstay for primary and recurrent disease, including select patients with isolated liver metastases. Mitotane has remained the preferred adjuvant treatment agent, showing modest effect in patients with unresectable, residual or metastatic disease. Multi-institutional registries and trials need to be established, with multidisciplinary efforts focused on the development of new therapeutic strategies.
authors.affiliation: Yale University School of Medicine, New Haven, Connecticut 06520, USA. sanziana.roman@yale.edu
authors.forename: Sanziana
authors.initials: S
authors.lastname: Roman
mesh_terms: D028341:Activins / Q000378:metabolism; D000306:Adrenal Cortex Neoplasms / Q000378:metabolism / Q000473:pathology / Q000628:therapy; D000315:Adrenalectomy; D018268:Adrenocortical Carcinoma / Q000378:metabolism / Q000473:pathology / Q000628:therapy; D018931:Antineoplastic Agents, Hormonal / Q000627:therapeutic use; D017024:Chemotherapy, Adjuvant; D006801:Humans; D007265:Inhibins / Q000378:metabolism; D008939:Mitotane / Q000627:therapeutic use; D049268:Positron-Emission Tomography / Q000379:methods; D042461:Vascular Endothelial Growth Factor A / Q000378:metabolism
chemical_list: D018931:Antineoplastic Agents, Hormonal; D042461:Vascular Endothelial Growth Factor A; D028341:Activins; D007265:Inhibins; D008939:Mitotane