Estrogen Secreting Adrenal Adenocarcinoma in an 18-Month- old Boy: Aromatase Activity, Protein Expression, mRNA and Utilization of Gonadal Type Promoter

摘要

We examined clinical, endocrinological and molecular biological aspects of an estrogen-secreting adrenal carcinoma in an 18-month-old male to clarity the pathogenesis of this condition. An 18-month-old boy was referred for evaluation of progressive bilateral gynecomastia and appearance of pubic hair. The patient had elevated plasma estradiol (349 pg/ml) and testosterone (260 ng/dl) levels that completely suppressed FSH and LH levels, and was subsequently diagnosed with an adrenal tumor on the right side. After removal of a 300-g adenocarcinoma, gy- necomastia regressed and essentially normal hormone levels were restored.