Advanced ACC with Widespread Metastases — Treatment When Mitotane Monotherapy Does Not Work
This protocol applies to advanced adrenocortical carcinoma (ACC) with widespread metastatic disease not amenable to local treatment, in patients who have progressed under first-line mitotane monotherapy and have not achieved the expected tumour response or stable disease.
Clinical scenario
Advanced adrenocortical carcinoma with widespread metastatic disease not amenable to local treatment. Routine adrenal surgery is not recommended in the setting of widespread metastasis at initial diagnosis.
Previous line — failure condition
The initial approach involved mitotane monotherapy (or mitotane plus EDP depending on prognostic parameters), targeting a therapeutic mitotane blood level and achievement of tumour response or long-term stable disease. Progression under mitotane monotherapy — failure to reach these goals — triggers escalation to this next-line protocol.
Next-line approach (partial overview)
For patients progressing under mitotane monotherapy, the recommended escalation involves adding a multi-agent chemotherapy combination to ongoing mitotane. The therapeutic goal remains achieving tumour response or stable disease, with ongoing monitoring of mitotane blood levels. The complete regimen and monitoring targets are in the structured protocol.
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).
- In patients who progress under mitotane monotherapy, we recommend to add EDP (+++O).