Testosterone-producing adrenocortical neoplasm in a 6-year-old boy

Adrenal tumours that predominantly secrete testosterone are virtually unknown in prepubertal male patients. We present the case of a 6-year-old boy with premature sexual development and markedly elevated serum testosterone, but normal urinary steroid levels. Diagnostic imaging demonstrated a spherical tumour of the left adrenal gland. Surgical excision led to normalisation of testosterone levels, and postoperative serial low hormone measurements ruled out tumour recurrence.Although extremely rare, this case illustrates that testosterone-producing adrenal adenomas may be encountered in boys without urinary steroid elevation. Surgical excision promises a definite cure. Testosterone is a useful tumour marker in these patients until the beginning of puberty.