Pathology · Endocrine Pathology (Thyroid, Adrenal, Pituitary)

In the WHO 2022 classification, all adrenocortical carcinomas (ACC) are now graded using the Helsinki score. A 50-year-old patient has a 12 cm adrenal mass. On resection: 32 mitoses/50 HPF, diffuse architecture, vascular invasion, and Ki67 of 30%. According to the Weiss criteria (still applied alongside Helsinki score), which constellation meets criteria for malignancy?

  • A A single Weiss criterion (≥5 mitoses/50HPF) is sufficient to diagnose adrenocortical carcinoma
  • B The Weiss score requires presence of all nine criteria to be positive for a definitive diagnosis of ACC; partial criteria indicate uncertain malignant potential
  • C Only size (>6 cm) and Ki67 >10% are required for the WHO 2022 diagnosis of adrenocortical carcinoma, replacing the Weiss criteria
  • D Three or more of the nine Weiss criteria (nuclear grade III/IV, high mitotic rate ≥5/50HPF, atypical mitoses, clear cells ≤25% of tumor, diffuse architecture >33%, tumor necrosis, venous invasion, sinusoidal invasion, capsular invasion) diagnose ACC; this case meets at least 5-6 criteria, confirming malignancy
Correct answer: D. Three or more of the nine Weiss criteria (nuclear grade III/IV, high mitotic rate ≥5/50HPF, atypical mitoses, clear cells ≤25% of tumor, diffuse architecture >33%, tumor necrosis, venous invasion, sinusoidal invasion, capsular invasion) diagnose ACC; this case meets at least 5-6 criteria, confirming malignancy

Explanation

The Weiss criteria for adrenocortical carcinoma (ACC) are a nine-parameter scoring system: nuclear grade III-IV, mitotic rate ≥5/50HPF, atypical mitoses, clear (lipid-rich) cells ≤25%, diffuse architecture >33% of tumor, coagulative necrosis, venous invasion, sinusoidal invasion, and capsular invasion. A score of ≥3 of 9 criteria indicates malignancy (ACC). This tumor with 32 mitoses/50HPF (criterion met), diffuse architecture (criterion met), vascular invasion (criterion met), and likely other features meets well above 3 criteria, confirming ACC. WHO 2022 has introduced the Helsinki score (mitotic count and Ki67 for grading ACC into grade 1-3) but has not replaced Weiss criteria for the diagnostic threshold. Size alone is not a Weiss criterion, though large size is suspicious.

Reference: Robbins & Cotran Pathologic Basis of Disease, 10th ed.

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