Ovarian malignant luteinized thecoma--an unusual tumor in an adolescent

Malignant luteinized thecoma is an extremely rare ovarian tumor. The fifth reported case of this tumor presenting in a 13-year-old epileptic child on anticonvulsant therapy with a rapidly enlarging pelvic-abdominal mass is discussed. At laparotomy, bilateral ovarian involvement, massive ascites, and omental and bowel serosal metastases were present. The pathological diagnosis was difficult with initial diagnoses of ovarian edema, endodermal sinus tumor, and ovarian fibrosarcoma. The final diagnosis of malignant luteinized thecoma was made by recognizing the presence of lutein cells with foamy vacuolated cytoplasm and intracytoplasmic staining for oil red O. Electron microscopy also demonstrated vacuolated cells with multiple fat globules. After surgical debulking the patient was treated with combination chemotherapy with complete response. The similarities of this case to three previously reported bilateral thecomas presenting in children on anticonvulsant therapy is discussed.