Virilizing adrenal ganglioneuroma containing Leydig cells

Abstract

An adrenal medullary ganglioneuroma containing Leydig cells and revealed by a virilizing syndrome in a 56-year old woman is presented. The syndrome, associating with masculinization an elevated serum testosterone level and a normal urinary 17 ketosteroids, is uncommon in adrenal tumours. The tumour was located by computed tomographic (CT) scan and treated by right adrenalectomy. Microscopic examination showed typical features of an adrenal medullary ganglioneuroma containing Leydig cells with Reinke crystalloids. Positive immunohistochemical study confirmed the testosterone secreting nature of the cells. The presence of Leydig cells in the adrenal gland is discussed according embryogenic studies: 1. the common origin of the gonad and the adrenal cortex from the coelomic epithelium, 2. the vicinity of adrenocortical gland and gonad during embryogenesis, 3. the thecal metaplasia of mesenchymal cells, 4. the development of Leydig cells from Schwann cells. Fourth case published in the world literature.