Severe virilization in a girl with a steroid cell tumor of the ovary.
We report a 6-year-old girl with striking signs of virilization, as well as elevated concentrations of testosterone and 17-hydroxyprogesterone. Although the elevated 17-hydroxyprogesterone concentration initially suggested late-onset 21-hydroxylase deficiency, she was found to have an ovarian mass by sonography which at surgery proved to be a hilus cell subtype of steroid cell tumor. This intra-ovarian tumor appears to be the smallest tumor of its type ever reported. The testosterone concentration was normal three days after tumor resection and has remained so two years later.
Snyder
D
D
Department of Pediatrics, Oregon Health Sciences University, Portland 97210, USA.
LaFranchi
S
S
D019326:17-alpha-Hydroxyprogesterone / Q000097:blood; D000236:Adenoma / Q000097:blood / Q000150:complications*; D002648:Child; D002987:Clitoris / Q000473:pathology; D005260:Female; D006801:Humans; D010051:Ovarian Neoplasms / Q000097:blood / Q000150:complications*; D013739:Testosterone / Q000097:blood; D014770:Virilism / Q000097:blood / Q000209:etiology*; D014845:Vulvar Diseases / Q000209:etiology
D013739:Testosterone; D019326:17-alpha-Hydroxyprogesterone