Non-functioning adrenocortical carcinoma presenting as acute spontaneous retroperitoneal haemorrhage

Summary: Adrenocortical carcinomas (ACCs) are a very rare entity with an incidence of approximately 0.5-2 cases per million per year. They are often difficult to diagnose clinically, attributing to non-specific signs and symptoms, especially with non-functioning tumours, with histopathology required for confirmatory diagnosis. Atraumatic adrenal haemorrhage has been described in the literature as a rare presentation of ACC. We describe a case of a gentleman in his fifties presenting with acute spontaneous unilateral adrenal haemorrhage on a background of a pre-existing known incidental adrenal nodule that was lost to follow-up. The patient underwent angio-embolisation and subsequent laparoscopic adrenalectomy, with histopathology demonstrating ACC. This report aims to highlight adrenal haemorrhage as a presenting feature of ACC and stress the importance of close follow-up and thorough initial investigation of adrenal nodules to ascertain their underlying aetiology and ultimately prevent delay in appropriate management. Learning points: Follow-up is essential for adrenal nodules with unclear aetiology to ensure timely investigation and risk stratification, preventing potential harm from delayed management of non-benign conditions. Spontaneous adrenal haemorrhage is a rare but potential presentation of ACC. There needs to be a high index of suspicion for malignancy with atypical presentations of adrenal nodules. Adjuvant mitotane may not improve recurrence and overall survival in low-risk patients, but may be indicated in patients at a higher risk of recurrence.