A Distinctive Case Report Highlighting the Occurrence of a Rare Gigantic Adrenal Myelolipoma in a Patient with Sickle Cell Anaemia

Adrenal myelolipomas (AMLs) are rare slow-growing non-malignant tumors that comprise both adipose and haematopoietic tissue and are caused by metaplasia of the reticuloendothelial cell. It is a type of incidentaloma which is an uncommon adrenal mass usually asymptomatic and identified during imaging investigation for reasons other than adrenal gland disorders. Myelolipomas vary in size but can be as large as 30 cm. These large myelolipomas can cause pressure on nearby structures resulting in clinical symptoms and signs.

This case report and literature review detail the diagnostic, medical and surgical management of a 14-year-old male with sickle cell anaemia who had a giant adrenal myelolipoma which was symptomatic. He had a one-week history of bilateral leg swelling and physical examination showed a mass in the right lumbar region. Abdominopelvic ultrasound revealed a suprarenal right-sided mass displacing the right kidney downwards. It was characterized as encapsulated, echo-complex, rounded and well-defined. The mass was also exerting pressure on the upper part of the kidney, and significantly compressing the inferior vena cava. A computed tomography (CT) scan revealed a large mass tenanting the right adrenal gland. He subsequently had an exploratory laparotomy with excision of the adrenal tumour. Immunohistochemistry analysis of the mass confirmed the diagnosis of an adrenal myelolipoma.

Conclusively, the diagnosis of an adrenal myelolipoma necessitates a high index of suspicion, a detailed medical history, a thorough physical examination, imaging studies and a meticulous histopathology investigation. Surgical intervention is the primary treatment method, while effective postoperative care ensures rapid recovery with minimal complications.