Recurrent Adrenocortical Carcinoma: Next-Line Systemic Treatment After Surgery or Ablation Plus Mitotane

This protocol covers recurrent adrenocortical carcinoma in patients who have had a disease-free interval of at least 12 months and whose recurrence was amenable to complete resection or ablation — and where escalation to more aggressive systemic therapy is now indicated.

Clinical Scenario

Patients with recurrent ACC who experienced a disease-free interval of at least 12 months and whose recurrence was considered amenable to complete resection or ablation. In this population, surgery or local ablative therapy followed by initiation of mitotane is the recommended first approach.

Previous Line: What Was Tried — and Why Escalation Is Needed

The prior approach — complete surgical resection, or local ablative therapies such as radiofrequency ablation, radiation therapy, cryoablation, microwave ablation, or chemoembolization, followed by mitotane — aimed to achieve and maintain a mitotane blood level of 14–20 mg/L. When that goal is not reached and more aggressive treatment becomes necessary, this protocol defines the next step.

Treatment Approach (Partial)

When more aggressive systemic therapy is indicated, the EDP-M combination regimen — chemotherapy plus mitotane — is the most validated approach in this setting; full dosing, scheduling, and monitoring details are available in the complete protocol.

Instant Access to Structured Evidence-Based Regimens

References

In patients with recurrent disease and a disease-free interval of at least 12 months, in whom a complete resection/ablation seems feasible, we recommend surgery or alternatively other local therapies (+OOO).
If more aggressive therapy is indicated, then the combination of EDP in addition to mitotane (EDP-M) is the most validated regimen (30).
Plus oral mitotane aiming at a blood level between 14 and 20 mg/L

DOI: 10.1530/EJE-18-0608

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