Aromatase p450 expression in a feminizing adrenal adenoma presenting as isosexual precocious puberty.
A 7-yr-old girl presented with isosexual precocious puberty secondary to a feminizing adrenal adenoma. The adrenal tumor was found to express aromatase messenger ribonucleic acid. Enzyme kinetic studies revealed a high level of aromatase activity in the adrenal tumor, with a K(m) of 45 nmol/L and a maximum velocity of 25.6 pmol/mg.h. Aromatase activity was approximately 500-fold higher in the tumor than in adjacent normal adrenal tissue. Although histopathological examination of the tumor was most consistent with a benign adenoma, the aromatase transcripts present in the tumor corresponded to those previously associated with malignant as well as benign tumors. We consider the pattern of aromatase expression sufficient to warrant continued follow-up for tumor recurrence. Our case demonstrates that isosexual precocious puberty secondary to a feminizing adrenal tumor can be due to estrogen synthesis from the tumor itself rather than peripheral aromatization as had been previously theorized.
Phornphutkul
C
C
Department of Pediatrics, Brown University and Rhode Island Hospital, Providence, Rhode Island 02903, USA.
Okubo
T
T
Wu
K
K
Harel
Z
Z
Tracy
T F
TF
Pinar
H
H
Chen
S
S
Gruppuso
P A
PA
Goodwin
G
G
D000236:Adenoma / Q000150:complications / Q000201:enzymology* / Q000235:genetics / Q000601:surgery; D000305:Adrenal Cortex Hormones / Q000652:urine; D000310:Adrenal Gland Neoplasms / Q000150:complications / Q000201:enzymology* / Q000235:genetics / Q000601:surgery; D000315:Adrenalectomy; D000728:Androgens / Q000097:blood / Q000652:urine; D001141:Aromatase / Q000235:genetics* / Q000378:metabolism*; D002648:Child; D004967:Estrogens / Q000097:blood / Q000652:urine; D005091:Exons; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D006854:Hydrocortisone / Q000652:urine; D007700:Kinetics; D011629:Puberty, Precocious / Q000201:enzymology / Q000209:etiology* / Q000235:genetics; D012333:RNA, Messenger / Q000032:analysis; D020133:Reverse Transcriptase Polymerase Chain Reaction; D014481:United States; D044465:White People
D000305:Adrenal Cortex Hormones; D000728:Androgens; D004967:Estrogens; D012333:RNA, Messenger; D001141:Aromatase; D006854:Hydrocortisone