ABSTRACT
Primary malignant tumours of the adrenal gland are rare and present a diagnostic challenge. The adrenal glands are small paired endocrine organs consisting of a medial and lateral limb, body, and apex which lie in the suprarenal space. In adults, 90% of the gland is composed of an outer cortex derived from the mesoderm, and the remainder comprises an inner medulla derived from the neural crest ectoderm. The cortex and medulla are structurally and functionally very different and give rise to different primary malignant tumours. The majority of adrenocortical tumours are benign and non-functioning (1). Adrenocortical carcinomas (ACCs) arise from the cortex whilst malignant pheochromocytomas, ganglioneuroblastomas, neuroblastomas, and other neuroendocrine malignancies arise from the medulla. Other exceptionally rare malignancies include primary adrenal lymphoma, de-differentiated liposarcoma, epithelioid angiosarcoma, and leiomyosarcoma (2).
