Clinical predictors of outcome in advanced adrenocortical carcinoma: a multicenter international ENSAT study
Authors' conclusion
The ENSAT Risk Score for Advanced ACC is a practical, prognostic tool for patients with advanced ACC receiving systemic therapy. Based on accessible clinical and biochemical markers, it can support treatment decisions and facilitate informed discussions in routine care.
Study context
- Population
- Patients with advanced adrenocortical carcinoma (ACC) undergoing systemic therapy, 11 centers; 61.5% women, median age 52 years
- Participants
- 418 patients11 centers
- Exposure
- Clinical predictors and ENSAT Risk Score >2 vs ≤2
- Mitotane monotherapyn = 161
- Etoposide + doxorubicin + cisplatin ± mitotanen = 178
- Second-line regimensn = 79
- Outcome
- OS
Methodological details11 centers, ENSAT Risk Score: tumor burden (0-2), cortisol excess (0/1), ECOG-PS (0-2), neutrophil-to-lymphocyte ratio (NLR) (0/1)
- 11 centers
- ENSAT Risk Score: tumor burden (0-2), cortisol excess (0/1), ECOG-PS (0-2), NLR (0/1)
Main finding
As reported in the abstract
Tumor burden, cortisol excess, ECOG-PS and NLR ≥5 independently predicted shorter overall survival (OS) (HR)
1.55-2.68
ENSAT Risk Score >2 (poor-risk) associated with worse OS across all treatment groups (HRs)
3.05-3.96
Observational data: associations, not effects.
Further findings
Time to progression, ENSAT Risk Score >2
- Score >2 associated with worse time to progression (TTP) across all treatment groups (HRs)
- 2.53-3.08
Objective response, ENSAT Risk Score >2
- Poorer response to mitotane
- P < 0.01
- Poorer response to second-line therapies
- P = 0.04
Original publication
Clinical predictors of outcome in advanced adrenocortical carcinoma: a multicenter international ENSAT study. Eur J Endocrinol. Published 6 March 2026. doi:10.
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