Adrenocortical carcinoma (ACC)

Features that raise suspicion

Imaging and clinical features that push the diagnosis toward cancer rather than a benign adenoma include:

• Size greater than about 4 cm, especially larger than 6 cm.³
• Heterogeneous, high-density or irregular appearance on CT or MRI.
• Rapid growth on serial imaging.
• Hormone over-production, particularly combined glucocorticoid and androgen excess (rapid onset of virilisation or Cushing's features).
• Local invasion or distant spread on staging.

Workup

• Full hormone screen (cortisol, androgens, aldosterone, metanephrines) to characterise the tumour and plan peri-operative care.
• Cross-sectional imaging with dedicated adrenal CT and, often, chest CT for staging.
• FDG-PET is sometimes used to help differentiate malignant from benign adrenal lesions and to look for distant disease.
• Multidisciplinary team review is recommended for any suspected ACC.

Principles of surgery

For disease that can be completely removed, surgery aims for an R0 (complete) resection with an intact tumour capsule. Rupture must be avoided because it markedly worsens outcome. For larger or invasive tumours, an open approach with en-bloc resection of involved structures may be required rather than a minimally invasive approach.⁸

When the tumour is smaller and clearly confined to the gland, some centres perform robotic or laparoscopic resection provided the same oncological principles can be met. The choice is made by a surgeon experienced in both approaches.

After surgery

Management after ACC surgery is multidisciplinary. Depending on stage, grade and margins, adjuvant mitotane, targeted radiotherapy or other systemic therapy may be considered. Surveillance imaging and biochemical monitoring are regular and long-term.