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      Extraovarian Steroid Cell Tumor ‘Not Otherwise Specified’ as a Rare Cause of Virilization in Twelve-Year-Old Girl

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          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 congenital 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’. Within the next months the signs of virilization resolved and menarche occurred. Conclusions: Steroid cell tumor should be considered in differential diagnosis of virilization in childhood. Regarding the age of our patient and pathohistological findings of the tumor, her prognosis is favorable.

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          Author and article information

          Horm Res Paediatr
          Hormone Research in Paediatrics
          S. Karger AG
          05 December 2001
          : 55
          : 5
          : 254-257
          aDepartment of Pediatrics, University Hospital Zagreb-Rebro; bDepartment of Gynecology and Obstetrics, University Hospital Zagreb and cEndocrinological Laboratory, University Hospital Zagreb-Rebro, Zagreb, Croatia
          50006 Horm Res 2001;55:254–257
          © 2001 S. Karger AG, Basel

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          Page count
          Figures: 2, Tables: 1, References: 8, Pages: 4
          Case Report


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