Case Report Horm Res 2001;55:254–257 Extraovarian Steroid Cell Tumor ‘Not Otherwise Specified’ as a Rare Cause of Virilization in Twelve-Year-Old Girl Miroslav Dumic ´ a Velimir S ˇ imunic ´ b Jadranka Ilic ´ -Forko b Marijana Cvitanovic ´ a Vesna Plavs ˇic ´ c Nevena Janjanin a Jasenka Ille a a Department of Pediatrics, University Hospital Zagreb-Rebro; b Department of Gynecology and Obstetrics, University Hospital Zagreb and c Endocrinological Laboratory, University Hospital Zagreb-Rebro, Zagreb, Croatia Received: January 9, 2001 Accepted after revision: August 1, 2001 Prof. Miroslav Dumic ´ , MD Department of Pediatrics University Hospital Zagreb-Rebro Kis ˇpatic ´eva 12, HR–10000 Zagreb (Croatia) Tel. +385 1 2388 556, Fax +385 1 2421 894 E-Mail maricvit@mef.hr ABC Fax + 41 61 306 12 34 E-Mail karger@karger.ch www.karger.com © 2001 S. Karger AG, Basel 0301–0163/01/0555–0254$17.50/0 Accessible online at: www.karger.com/journals/hre Key Words Virilizing tumor W Steroid cell tumor W Ovary Abstract Background: We present a 12-year-old girl with a 5-year history of progressive virilization. Results: Regarding elevated plasma levels of 17-hydroxyprogesterone (17- OHP) and androgens, normal ultrasound and CT scan of ovaries and adrenal glands, the nonclassic form of con- genital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency was presumed the cause of virilization. As the glucocorticoid therapy did not normalize high levels of 17-OHP and androgens, and the DNA analysis did not demonstrate a mutation causing CAH, a laparotomy was performed. Near the right ovary a tumor was found and extirpated. Pathohistological studies determined it to be a rare steroid cell tumor, ‘not otherwise specified’. With- in the next months the signs of virilization resolved and menarche occurred. Conclusions: Steroid cell tumor should be considered in differential diagnosis of viriliza- tion in childhood. Regarding the age of our patient and pathohistological findings of the tumor, her prognosis is favorable. Copyright © 2001 S. Karger AG, Basel Introduction Virilization in girls in the prepubertal period is pre- sented by enlargement of the clitoris, hirsutism, acne, deep voice and increased muscular development. It can be caused by hypersecretion of androgens from the adre- nal gland cortex (virilizing forms of congenital adrenal hyperplasia (CAH) or adrenal gland tumor) or, rarely, from the ovary (polycystic ovarian syndrome, hypertheco- sis, ovarian tumor). It can be also of iatrogenic origin. We present a 12-year-old girl in whom progressive vir- ilization was evident from the seventh year of age. She was originally thought to have CAH, a nonclassic form due to 21-hydroxylase deficiency (NC 21-OHD). How- ever, further evaluation confirmed the presence of a rare ovarian tumor – a steroid cell tumor, ‘not otherwise speci- fied’ (NOS), as a source of hypersecretion of androgens and 17-hydroxyprogesterone (17-OHP). Methods Serum and urine steroid hormones were assayed by standard radioimmunological methods. The amount of 17-ketosteroids (17- KS) in 24-hour urine was measured by standard spectrophotometric method. Stimulation test was performed by measuring cortisol con-