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Treatment & Prognosis: About 30-40% of ovarian steroid cell tumors are malignant. About 20% of patients with malignant steroid cell tumor present at advanced stages. These patients often have Cushing syndrome. Features associated with malignancy include: large size (>7cm), necrosis, hemorrhage, grade 2-3 nuclear atypia, and mitotic rate ≥ 2 mitoses/10HPF.

In young women with organ-confined tumors (Stage IA), the treatment consists of unilateral salpingo-oophorectomy along with staging. When fertility preservation is not an issue and in older women, the treatment consists of total abdominal hysterectomy and bilateral salpingo-oophorectomy with or without full staging.

In advanced stages, tumor debulking is performed followed by platinum-based chemotherapy. Virilizing symptoms usually regress following surgery. Recurrences develop in 10% of cases, usually more than 5 years after surgery.

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