Time trends in incidence, treatment, and survival of patients with adrenocortical carcinoma, a nationwide study
Authors' conclusion
The incidence of adrenocortical carcinoma is stable over time. The 5-year survival for stage I-III remained stable, whereas the survival for stage IV increased. Factors associated with increased survival are centralization of care, adrenalectomy, surgical control of disease, chemotherapy, and mitotane therapy.
Study context
- Population
- Adults with ACC diagnosed 1993–2020 in the Netherlands, Netherlands Cancer Registry
- Participants
- 685 adults
- Outcome
- Time trends in incidence, treatment and survival of ACC
Methodological detailsprospective registry, multivariate Cox regression
- Prospective registry
- Multivariate Cox regression
Main finding
As reported in the abstract
Median age-adjusted incidence rate (European Standard Population) · 1993–2020
- Median age-adjusted incidence rate, stable over time
- 1.62 per million person-years [0.83-2.11]
- Stage III at diagnosis, gradual increase
- 13%-25%
- Stage IV at diagnosis, stable proportion
- 40%
- 5-year survival stage I-II, stable
- 65%
- 5-year survival stage III, stable
- 35%
- 5-year survival stage IV, 1993 through 1996
- 3%
- 5-year survival stage IV, 2017 through 2020
- 11%
Observational data: associations, not effects.
Further findings
Referral to expert center since Dutch Adrenal Network (2004)
- More patients referred to an expert center
- P < 0.001
- Expert center referral and survival, adjusted HR
- 0.70; 95% CI, 0.57-0.85
Survival by treatment, all stages (multivariate Cox regression)
- Adrenalectomy, adjusted HR
- 0.53; 95% CI, 0.43-0.65
- Mitotane therapy, adjusted HR
- 0.73; 95% CI, 0.55-0.98
Survival in stage IV disease
- Adrenalectomy, surgical control of disease, chemotherapy and mitotane therapy associated with increased survival
Mitotane use
- Mitotane users reaching therapeutic drug level
- 58%
- Discontinued mitotane prematurely
- 59.5%
Original publication
Time trends in incidence, treatment, and survival of patients with adrenocortical carcinoma, a nationwide study. J Clin Endocrinol Metab. Published 28 February 2026. doi:10.
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