Bilateral giant adrenal myelolipomas: a rare complication of classical congenital adrenal hyperplasia

Adrenal myelolipomas are benign adrenal tumours containing adipose and haematopoietic tissue. Although rare, they are significantly more common in patients with poorly controlled classical congenital adrenal hyperplasia (CAH) due to chronic adrenocorticotropic hormone (ACTH) overstimulation. This case describes a male patient in his 40s with longstanding CAH (21-hydroxylase deficiency) who presented with symptomatic bilateral giant adrenal myelolipomas and testicular adrenal rest tumours (TARTs). He presented acutely to hospital with abdominal pain, nausea and vomiting. Urgent imaging demonstrated giant bilateral adrenal masses with benign appearances in keeping with myelolipomas. Subsequently, he underwent open bilateral adrenalectomy. Postoperatively, his quality of life improved markedly and his steroid requirements reduced. This case illustrates the importance of increased awareness of myelolipomas in patients with poorly controlled CAH and highlights the need for vigilant endocrine management and a multidisciplinary approach to prevent other long-term complications such as TARTs.