TOPLINE:

The incidence of adrenocortical carcinoma (ACC) remained stable in Netherlands from 1993 to 2020. The survival for stage I-III disease also remained stable over time, but the survival for stage IV disease increased; centralised care and treatment modalities such as adrenalectomy and mitotane therapy were linked to improved survival.

METHODOLOGY:

• Researchers conducted a nationwide study to evaluate time trends in the incidence, survival outcomes, and treatment modalities of patients with ACC.
• They included 685 adult patients diagnosed with ACC (median age at diagnosis, 58 years; 56.8% women) using data from the nationwide prospective registry (Netherlands Cancer Registry) between 1993 and 2020.
• Changes in the incidence and survival outcomes according to tumour stages (I-IV) and different treatment modalities (surgery, chemotherapy, radiotherapy, and immunotherapy) were assessed.
• The effect of the Dutch Adrenal Network on centralised care and patient survival was also evaluated.

TAKEAWAY:

• The median age-adjusted incidence rate of ACC was 1.62 per million person-years and remained stable over time. The proportion of stage III disease increased from 13% to 25%, whereas the proportion of stage IV disease remained stable at 40%.
• At 1 year, the survival remained stable at 90% for stage I-II disease and 67% for stage III disease; the survival at 5 years also remained stable (P for trend = .43). However, the 5-year survival for stage IV disease increased from 3% to 11% from 2017 to 2020 (P for trend = .01).
• Since the Dutch Adrenal Network was founded in 2004, referrals to expert centres increased (P < .001), with 85% of adrenalectomies and 95% of medical treatments performed in expert centres between 2014 and 2019. Treatment in expert centres was associated with improved survival (adjusted hazard ratio [aHR], 0.70; 95% CI, 0.57-0.85).
• Adrenalectomy (aHR, 0.53; 95% CI, 0.43-0.65) and adjuvant mitotane therapy (aHR, 0.73; 95% CI, 0.55-0.98) were associated with improved survival across all stages. In stage IV disease, adrenalectomy, additional and metastasis surgery, palliative mitotane therapy, and chemotherapy were all associated with improved survival.

IN PRACTICE:

"The finding that the five-year survival for stage IV disease did increase, while the one-year survival did not, might suggest that the survival benefit from the changes in chemotherapy and local therapies of the recent years only benefit patients with less aggressive stage IV disease who have survived the first year. This verifies the need for new or better tailored treatment options for ACC," the authors wrote.

SOURCE:

This study was led by Rebecca V. Steenaard, Department of Internal Medicine, Máxima MC, Eindhoven/Veldhoven, Netherlands. It was published online on February 28, 2026, in The Journal of Clinical Endocrinology & Metabolism.

LIMITATIONS:

The observational nature of the study may have introduced selection bias. Substantial missing data existed for ACC‑specific items and diagnostic characteristics recorded from 2014 onwards, suggesting poor documentation in hospital records.

DISCLOSURES:

This study did not disclose any specific funding. The authors declared having no conflicts of interest.

This article was created using several editorial tools, including AI, as part of the process. Human editors reviewed this content before publication.