# OnCo record advanced-adrenocortical-carcinoma (cancer). Data CC BY-NC 4.0, attribute "Data from OnCo (onco.cc)". Whole corpus: https://onco.cc/api/v1/onco.nt
@prefix schema: <https://schema.org/> .
@prefix onco: <https://onco.cc/ns#> .
@prefix xsd: <http://www.w3.org/2001/XMLSchema#> .

<https://onco.cc/cancers/advanced-adrenocortical-carcinoma/>
  a schema:MedicalCondition ;
  onco:kind "cancer" ;
  schema:identifier "advanced-adrenocortical-carcinoma" ;
  schema:name "Advanced and metastatic adrenocortical carcinoma (ENSAT stage IV or unresectable)"@en ;
  schema:alternateName "Metastatic ACC"@en, "Stage IV adrenocortical carcinoma"@en, "Unresectable adrenal cortical carcinoma"@en, "Recurrent adrenocortical carcinoma"@en ;
  schema:description "Advanced adrenocortical carcinoma is adrenal cortex cancer that has spread to distant organs or cannot be removed, one of the hardest endocrine cancers to treat. The standard is mitotane with etoposide, doxorubicin and cisplatin, established by the FIRM-ACT trial; limited spread is treated locally, hormone excess with steroid-blocking drugs, and immunotherapy helps a minority."@en ;
  schema:url <https://onco.cc/cancers/advanced-adrenocortical-carcinoma/> ;
  schema:dateModified "2026-09-18"^^xsd:date ;
  schema:sameAs <https://en.wikipedia.org/wiki/Adrenocortical_carcinoma> ;
  schema:citation <https://doi.org/10.1056/NEJMoa1200966>, <https://doi.org/10.1530/EJE-18-0608>, <https://en.wikipedia.org/wiki/Adrenocortical_carcinoma> ;
  onco:tag "subtype-page", "endocrine" ;
  onco:related <https://onco.cc/cancers/localised-adrenocortical-carcinoma/>, <https://onco.cc/cancers/adrenocortical/>, <https://onco.cc/cancers/metastatic-ppgl/> ;
  onco:technologies <https://onco.cc/technologies/cytotoxic-chemotherapy/>, <https://onco.cc/technologies/checkpoint-inhibitor/>, <https://onco.cc/technologies/kinase-inhibitors/>, <https://onco.cc/technologies/thermal-ablation/>, <https://onco.cc/technologies/sbrt/> ;
  onco:targets <https://onco.cc/targets/tp53/>, <https://onco.cc/targets/pd1/>, <https://onco.cc/targets/vegf/> ;
  onco:drugs <https://onco.cc/drugs/mitotane/>, <https://onco.cc/drugs/etoposide/>, <https://onco.cc/drugs/doxorubicin/>, <https://onco.cc/drugs/cisplatin/>, <https://onco.cc/drugs/gemcitabine/>, <https://onco.cc/drugs/capecitabine/>, <https://onco.cc/drugs/cabozantinib/>, <https://onco.cc/drugs/pembrolizumab/>, <https://onco.cc/drugs/relacorilant/> ;
  onco:terms <https://onco.cc/terms/adrenalectomy/>, <https://onco.cc/terms/germline-vs-somatic/>, <https://onco.cc/terms/msi/>, <https://onco.cc/terms/tmb/> ;
  onco:keyPapers <https://onco.cc/key-papers/paper-firm-act-edp-mitotane-adrenocortical-carcinoma-nejm-2012/>, <https://onco.cc/key-papers/paper-ese-ensat-adrenocortical-carcinoma-guideline-eur-j-endocrinol-2018/> ;
  onco:pipeline <https://onco.cc/drugs/cabozantinib/>, <https://onco.cc/drugs/pembrolizumab/>, <https://onco.cc/drugs/relacorilant/>, <https://onco.cc/drugs/mitotane/> ;
  onco:standardOfCare <https://onco.cc/drugs/mitotane/>, <https://onco.cc/drugs/mitotane/>, <https://onco.cc/technologies/thermal-ablation/>, <https://onco.cc/technologies/sbrt/>, <https://onco.cc/terms/adrenalectomy/>, <https://onco.cc/drugs/etoposide/>, <https://onco.cc/drugs/doxorubicin/>, <https://onco.cc/drugs/cisplatin/>, <https://onco.cc/drugs/mitotane/>, <https://onco.cc/technologies/cytotoxic-chemotherapy/>, <https://onco.cc/drugs/gemcitabine/>, <https://onco.cc/drugs/capecitabine/>, <https://onco.cc/drugs/cabozantinib/>, <https://onco.cc/drugs/pembrolizumab/>, <https://onco.cc/technologies/checkpoint-inhibitor/>, <https://onco.cc/technologies/kinase-inhibitors/>, <https://onco.cc/drugs/relacorilant/>, <https://onco.cc/drugs/cabozantinib/>, <https://onco.cc/drugs/pembrolizumab/> .