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.
- Complete endocrine assessment before biopsy or operation, including metanephrines to exclude phaeochromocytoma and a broad cortical steroid profile when ACC is suspected.
- Do not biopsy a potentially resectable primary adrenal cortical tumour routinely; it risks seeding or delay and cannot reliably separate adenoma from carcinoma.
- The best chance of cure is complete en-bloc, capsule-intact resection by a high-volume adrenal cancer surgeon, avoiding tumour rupture and unplanned piecemeal laparoscopy.
- Pathology requires expert endocrine reporting of margin status, stage, mitotic activity, invasion, validated malignancy criteria and Ki-67; the current RCPath dataset and TNM edition should be used.
- Mitotane, adjuvant decisions, systemic chemotherapy, radiotherapy and surveillance are specialist choices based on stage, resection, proliferation, hormone secretion, performance status and commissioning.
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.
Severe cortisol excess with infection, uncontrolled diabetes, hypokalaemia, psychosis or thrombosis needs urgent endocrine stabilisation while cancer staging proceeds.
Catecholamine symptoms or uncompleted metanephrine testing before any adrenal procedure demands pause and phaeochromocytoma exclusion.
Inferior vena cava extension, pulmonary embolic symptoms, rapidly increasing pain or haemodynamic compromise requires emergency specialist surgical and oncological assessment.
Vomiting, hypotension, hyponatraemia or collapse during mitotane therapy can represent adrenal crisis and needs immediate stress hydrocortisone rather than waiting for a drug level.
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.
- Assess performance status, thrombosis, infection, metabolic complications and genetic context while determining resectability.
- Agree an oncological operation, systemic plan or carefully justified tissue strategy in the centre responsible for definitive care.