Advanced and metastatic adrenocortical carcinoma (ENSAT stage IV or unresectable): lines of therapy
5 standard-of-care settings across 3 lines and 1 biomarker subgroup. Rows come from the cancer page's standard of care; the grid places each on its line and subgroup.
Early / localised
| Subgroup | Setting | Approach | Products and trials | Guideline | Evidence |
|---|---|---|---|---|---|
| All comers | Low-burden, indolent disease | Mitotane alone, with resection, thermal ablation or stereotactic radiotherapy of limited metastases. | ESE/ENSAT guideline on adrenocortical carcinoma 2018 | 43 |
Second line
| Subgroup | Setting | Approach | Products and trials | Guideline | Evidence |
|---|---|---|---|---|---|
| All comers | High-burden or progressive disease, first line | Etoposide, doxorubicin and cisplatin with mitotane (EDP-M, FIRM-ACT). | ESE/ENSAT guideline on adrenocortical carcinoma 2018 | 94 |
Other settings
| Subgroup | Setting | Approach | Products and trials | Guideline | Evidence |
|---|---|---|---|---|---|
| All comers | All patients | Mitotane from diagnosis with glucocorticoid replacement; adrenal enzyme inhibitors (metyrapone, osilodrostat, ketoconazole) for cortisol excess; thromboprophylaxis and infection vigilance. | ESE/ENSAT guideline on adrenocortical carcinoma 2018 | 43 | |
| All comers | After EDP-M | Gemcitabine with capecitabine; cabozantinib; pembrolizumab, especially for mismatch-repair-deficient tumours; streptozocin-mitotane; clinical trials. | ESE/ENSAT guideline on adrenocortical carcinoma 2018 | 99 | |
| All comers | Trials | Relacorilant with checkpoint inhibition for cortisol-secreting tumours; cabozantinib with immunotherapy; radiolabelled CYP11B tracers. | ESE/ENSAT guideline on adrenocortical carcinoma 2018 | 98 |
Lines and subgroups are parsed from the setting text of each standard-of-care row and can misclassify an unusual phrasing; the row’s own setting is always shown. Guideline chips reflect the NCCN category and ESMO-MCBS grade recorded on the cancer page, checked on its stated date. Not medical advice.