Severity and management of hypercortisolism in patients with adrenocortical carcinoma and overt Cushing syndrome
Authors' conclusion
Adrenal tumor size and the severity of cortisol excess are associated with a higher probability of metastatic disease at diagnosis. Our study suggests that the severity of cortisol excess appears to be associated with an increased risk of tumor recurrence/progression, suggesting that hypercortisolism intensity may serve as a marker of aggressive disease.
Study context
- Population
- Patients with Cushing´s syndrome (CS) due to adrenocortical carcinoma (ACC)
- Participants
- 101 patients
- Exposure
- Severity of hypercortisolism
- Outcome
- Prognostic role of baseline hypercortisolism on survival outcomes:
- PFS
- DFS
- OS
Methodological detailsinternational
- International
Main finding
As reported in the abstract
Recurrence/progression by severity of CS
P = 0.035
Observational data: associations, not effects.
Further findings
Survival (whole cohort)
- Median overall survival (OS) (range 1-176) months
- 19.4
- Median disease-free survival (DFS) (range 2.6-143) months
- 12.3
- Progression-free survival (PFS) (range 1.1-43.7) months
- 5.6
Baseline urinary free cortisol (UFC) and metastatic ACC · CS diagnosis
- Median UFC, times upper limit of normal (ULN) (IQR 2.9-14)
- 6.4
- Metastatic ACC
- 53.5% (n = 54)
- Max adrenal tumor size predicting metastatic ACC: OR
- 1.02 (95%CI 1.01-1.03)
- UFC levels predicting metastatic ACC
- 1.62 (95%CI 1.03-2.57)
- Independent predictors
Original publication
Severity and management of hypercortisolism in patients with adrenocortical carcinoma and overt Cushing syndrome. J Clin Endocrinol Metab. Published 10 September 2026. doi:10.
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