Adrenocortical Carcinoma Management and Prognosis

Adrenocortical carcinoma (ACC) is a rare, aggressive malignancy arising from the adrenal cortex, with heterogeneous clinical presentations ranging from hormone excess to mass effect. Surgical resection remains the cornerstone of curative intent therapy for localised disease, while mitotane—an adrenolytic agent—serves as the primary adjuvant and palliative systemic treatment. Risk stratification incorporates stage (based on tumour size, local invasion and distant spread), resection status and proliferative indices such as Ki-67, with histopathological scoring systems (e.g. the Weiss score) guiding diagnostic certainty and prognostic estimation. Despite radical surgery and adjuvant therapy, recurrence rates remain high, particularly in stage III–IV disease. Recent advances in immunohistochemistry and molecular profiling have refined prognostic models, enabling more precise patient selection for adjuvant mitotane and combination chemotherapy regimens. In metastatic disease, cytotoxic combinations (for example etoposide, doxorubicin and cisplatin) added to mitotane represent standard first-line therapy, though therapeutic outcomes remain modest. Emerging targeted approaches and immunotherapies are under investigation, prompted by an improved understanding of ACC genomics and tumour microenvironment. Overall survival varies widely according to stage and molecular subtype, underscoring the need for personalised management strategies and international collaborative trials to improve long-term outcomes.

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Adrenocortical Carcinoma Management and Prognosis publication trend

The graph below shows the total number of articles in adrenocortical carcinoma management and prognosis across all publications each year (not limited to Nature Index journals).

Technical terms

Mitotane: Adrenolytic agent used as adjuvant and palliative therapy in ACC.

Weiss score: Histopathological classification system employing nine criteria to distinguish benign from malignant adrenocortical tumours.

Recurrence-free survival: Time interval after primary treatment during which the patient remains free of detectable tumour recurrence.

Tumour necrosis: Microscopic areas of cell death within a tumour, serving as an adverse prognostic marker in ACC.

Pan-genomic analysis: Comprehensive examination of genome-wide alterations, including mutations, copy-number changes and DNA methylation patterns.

References

1. Adjuvant mitotane versus surveillance in low-grade, localised adrenocortical carcinoma (ADIUVO): an international, multicentre, open-label, randomised, phase 3 trial and observational study. The Lancet Diabetes & Endocrinology (2023).
2. Prognostic Value of Microscopic Tumor Necrosis in Adrenal Cortical Carcinoma. Endocrine Pathology (2023).
3. Comprehensive Pan-Genomic Characterization of Adrenocortical Carcinoma. Cancer Cell (2016).