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

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