Estrogen Secreting Adrenal Adenocarcinoma in an 18-Month-old Boy

Endocrine Journal

NAOMI OHNUMA⁴  NOBUHIRO HARADA¹  TADASHI MATSUNAGA⁴  KUNLO WATAKI³  TOSHIYUKI YASUDA³  KANSHI MINAMITANI³  TOMOYUKI WATANABE³  HIROMASA NODA²  MASANORI MINAGAWA³  YOICHI KOHNO³  KYOKO WADA³  TOMOHIRO SOMEYA³  ITSURO KAZUKAWA³ 

[1] Department of Biochemistry, Fujita Health University;Department of Pediatrics, Funabashi Central Hospital;Department of Pediatrics, Chiba University School of Medicine;Department of Pediatric Surgery, Chiba University School of Medicine

关键词: Aromatase;    Estrogen;    Adrenal;    Carcinoma;   

DOI  :  10.1507/endocrj.47.723

学科分类：内分泌与代谢学

来源: Japan Endocrine Society

【 摘 要 】

References(43)Cited-By(12)We examined clinical, endocrinological and molecular biological aspects of an estrogen-secreting adrenal carcinoma in an 18-month-old male to clarify 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 (349pg/ml) and testosterone (260ng/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, gynecomastia regressed and essentially normal hormone levels were restored. Aromatase activity in the tumor tissue determined by the 3H-water method was 71.0-104.4pmol/min/mg protein. High levels of aromatase protein and mRNA in the tumor tissue were also demonstrated, while neither aromatase activity nor protein was detected in normal adrenal glands. To investigate the regulation of aromatase expression in the adrenal carcinoma, we examined the usage of alternate promoters responsible for aromatase gene transcription. In the present case, the amounts of aromatase mRNA utilizing gonadal types of exon 1c (I.3) and 1d (II) were significantly higher than those that using other exon 1s. This result suggested that the utilization of a gonadal-type exon 1 might be involved in the overproduction of aromatase in estrogen-secreting adrenal carcinoma.

【 授权许可】

Unknown