Ovarian 'Tumor' of the adrenogenital syndrome - The first reported case

We report the case of a 36-year-old woman with congenital adrenal hyperplas ia from 21-hydroxylase deficiency who had been receiving replacement therap y with corticosteroids since birth. At the age of 35 years, she developed a brupt aggravation of her virilizing symptoms and underwent an adrenalectomy and partial left oophorectomy. Persistent virilization and high testostero ne levels led to right oophorectomy and completion left oophorectomy 6 mont hs later. Each adnexa contained ovarian or paraovarian soft brown masses th at on microscopic examination were identical to the testicular tumor of the adrenogenital syndrome. This represents the first reported case of this pa thology (well known in the testis) in the ovary.