Adrenocortical Carcinoma (ACC): Grading & Management
Pathological grading of ACC is critical for assessing tumor aggressiveness and guiding post-surgical care.
The Weiss Score & Beyond: While the Weiss Score remains a standard, multiple histology systems (modified Weiss, Lin-Weiss-Bisceglia, Reticulin criteria, etc) have been used to distinguish adrenocortical carcinoma from adenoma. Once the diagnosis of ACC is confirmed, the WHO 2022/2026 guidelines emphasize a two-tiered (low-grade vs high-grade) system based on a histologic evaluation of mitotic count threshold (20 per 10 mm²).
Prognostic Markers: High Ki-67 proliferation indices (typically >20%) are also an important pathological indicator of a poorer prognosis and have been incorporated into clinical trials.
Management Strategies: Clinical management relies on tumor staging. Standard care for localized ACC is complete surgical resection (R0), often followed by adjuvant Mitotane therapy to reduce the risk of recurrence when a high-grade ACC is confirmed by the pathologist.
