Adrenocortical cancer (ACC) is a rare aggressive tumour, with an incidence of 1-2 cases/million/year, accounting for <5% of adrenal incidentalomas.
Three cases of ACC:
1) A 45yr man with symptomatic Cushing’s syndrome with 24h urinary free cortisol (UFC) 1488nmol/day (<180) due to metastatic ACC, underwent early aggressive resection of primary (8cm adrenal) and solitary metastasis (18mm lung) and is currently in remission on mitotane. 2) A 61yr man with uncontrolled hypertension and a 2.6cm adrenal mass. Initial mildly raised plasma metanephrines resulted in a negative phaeochromocytoma work-up only. He presented one year later with rapidly progressive hypercortisolism 24h UFC 1940nmol/24hr (<150) due to metastatic ACC, unresponsive to mitotane and died within 6 months. 3) A 55yr woman with haematuria and a 3.9cm adrenal mass assessed without endocrine input. The significance of an abnormal dexamethasone suppression test was not appreciated and she had symptomatic cortisol deficiency post-adrenalectomy. Endocrine consult at 6 months for consideration of mitotane after metastatic disease confirmed. Early post-treatment CT indicates reduction in metastases.
These cases highlight key issues. All patients with an adrenal mass should have clinical assessment for signs and symptoms of hormone excess, and be screened for phaeochromocytoma, Cushing’s (and hyperaldosteronism if hypertensive). ACC presents with hormone excess in 40-70% with hypercortisolism in 50-80% and hyperandrogenism in 40-60%. Pre-operative work-up also guides the need for peri-operative glucocorticoid use. Operative planning is essential, as complete surgical resection is the most important prognostic factor by an experienced surgeon. Post-operative adjuvant mitotane is advocated due to results of several large retrospective studies, however its benefits on overall survival are not clear. Mitotane is the treatment of choice in metastatic disease.
Patients with ACC should be identified early, managed in a multi-disciplinary setting, complete thorough endocrine work-up and undergo aggressive resection with the aim to improve survival.