Advanced ACC with Widespread Metastases: What to Do After EDP-M Fails
Advanced adrenocortical carcinoma with widespread metastatic disease not amenable to local treatment — a stage requiring ongoing systemic management after first-line regimens are exhausted.
This protocol follows progression on EDP-M (etoposide, doxorubicin, cisplatin plus mitotane). The prior line is considered to have failed when it does not achieve a mitotane blood level within the therapeutic target range, or does not produce tumour response or stable disease.
After EDP-M failure, second-line systemic cytotoxic therapy is the primary strategy; gemcitabine-based combinations are among the regimens studied in this setting. Enrolment in clinical trials and evaluation of loco-regional therapies should also be considered. The complete regimen options, sequencing, and detailed clinical guidance are available in the full 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 EDP-M we suggest considering additional therapies including clinical trials on an individual basis (+OOO).
- Beyond cisplatin-based therapies, the two reasonably well-studied second-line cytotoxic regimens are gemcitabine + capecitabine (+/− mitotane) and streptozotocin + mitotane.