Background
Adrenocortical carcinoma (ACC) is a rare, aggressive malignancy with reported incidence of 1-2 cases per 1 million population2. Those who present with hormonal excess have concurrent hypercortisolism and hyperandrogenism. Adrenal tumours that solely secrete androgens are extremely rare (less than 10% of secretory ACCs) and produce hirsutism, virilisation and amenorrhoea in the majority of patients1.
Case Presentation
A 34 year old previously well woman presents with rapidly progressive hirsutism, acne and secondary amenorrhoea. Initial investigations showed elevated testosterone levels (total testosterone 3.9nmol/L [RR: 0.5-2.0nmol/L]) free androgen index (FAI) 17% (RR: 0.4-6.0%) and she was commenced on the oral contraceptive pill. Two months later in the setting of dyspnoea and chest tightness, a V/Q scan revealed unprovoked bilateral pulmonary emboli. A subsequent CT chest and abdomen revealed bilateral occlusive segmental pulmonary emboli with pulmonary infarcts as well as a left adrenal lesion measuring 125mm x110mm x140mm with invasion of the left renal vein and inferior vena cava. There was also paraaortic adenopathy and hepatic metastases.
Further biochemical investigations revealed normal plasma and urinary metanephrines, aldosterone renin ratio, 24 hour urinary free cortisol and chromogranin A levels. Repeat androgen studies showed a total testosterone of 5.5 nmol/L and FAI 14.9%. DHEAS was >27umol/L (RR 1.8-9.2umol/L). These results were suggestive of a pure androgen secreting adrenal tumour. A liver biopsy showed histopathology results consistent with metastatic adrenocortical carcinoma.
Given stage 4 disease with invasion of the left renal vein and inferior vena cava, surgical management was not offered. She was started on chemotherapy and mitotane in addition to hydrocortisone. The development of a pruritic morbilliform rash two weeks after starting mitotane has limited its escalation in dose.
Conclusion
Pure androgen secreting ACCs are very rare. Those who present with rapid onset hirsutism should be screened for an underlying androgen secreting tumour.