Synopsis
Recognise an adrenal mass suspicious for adrenocortical carcinoma, complete safe hormonal and metastatic staging, and protect oncological resection through early specialist cancer-centre management.
- Adrenocortical carcinoma is rare and aggressive; presentation may be an incidental mass, abdominal or back discomfort, mass effect, constitutional decline or rapid steroid-hormone excess.
- Rapid virilisation, combined androgen and cortisol secretion, severe new Cushing syndrome or feminisation is particularly concerning for cortical malignancy.
- Dedicated adrenal imaging should define size, heterogeneity, necrosis, local invasion, venous extension, nodes and metastases, with staging of chest, abdomen and pelvis before definitive surgery.
Key red flags
A suspected resectable ACC should not undergo local biopsy or non-specialist partial removal because capsule breach can convert a potentially curable field into disseminated disease.
Investigation priorities
Define primary tumour anatomy, resectability, venous involvement and metastatic stage.
Management branches
Imaging, growth or steroid phenotype makes adrenocortical carcinoma a realistic diagnosis.
- Contact a specialist adrenal cancer MDT before biopsy or surgery and transfer all original imaging for endocrine-radiology review.
- Complete chest-abdomen-pelvis staging and a cortisol, androgen, precursor and metanephrine assessment without delaying treatment of dangerous hormone excess.
Key medicines
MitotaneInitiate and escalate only under an experienced ACC protocol, using serial plasma levels and the current UK SmPC rather than a fixed empirical target dose.
Hydrocortisone replacement during mitotaneUse an endocrinologist-adjusted divided regimen that may exceed standard replacement because mitotane increases binding proteins and steroid clearance, with stress doses for illness.