Treatment of Advanced Adrenocortical Carcinoma with Widespread Metastatic Disease Not Amenable to Local Treatment
Clinical Scenario
This protocol applies to patients presenting with advanced adrenocortical carcinoma (ACC) who have widespread metastatic disease at diagnosis that is not amenable to local or surgical intervention. In this setting, local measures — including adrenal surgery — are not indicated as first management.
Treatment Approach
Systemic medical therapy is the cornerstone of management. The choice of regimen — whether a single-agent oral approach or a more intensive combination — is guided by individual prognostic parameters. A specific blood-level target for the primary agent is central to monitoring treatment adequacy.
The full regimen, combination criteria, and monitoring thresholds are detailed in the structured protocol →
Treatment Goals
The primary aims are achievement of tumour response or durable stable disease. In patients who respond to medical therapy, re-evaluation for local measures aimed at long-term tumour control may subsequently be considered.
References
DOI: 10.1530/EJE-18-0608
- We suggest against the routine use of adrenal surgery in case of widespread metastatic disease at the time of first diagnosis (+OOO).
- In patients with advanced ACC at the time of diagnosis not qualifying for local treatment, we recommend either mitotane monotherapy or mitotane + EDP depending on prognostic parameters (+++O).
- If more aggressive therapy is indicated, then the combination of EDP in addition to mitotane (EDP-M) is the most validated regimen.
- In patients who respond to medical therapy (including achievement of long-term stable disease), we suggest re-considering local measures aiming at long-term tumor control.
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