Malignant mixed müllerian tumor with malignant neuroectodermal components (teratoid carcinosarcoma) of the ovary: Report of a case with clinicopathologic findings.

Malignant mixed müllerian tumor (MMMT) or carcinosarcoma of the female genital tract is a rare neoplasm. Malignant ovarian tumor composed of müllerian epithelial tumor and malignant germ cell tumor is also rare, with most cases composed of endometrioid adenocarcinoma and yolk sac tumor. Ovarian MMMT with malignant neuroectodermal components resembling immature teratoma is extremely rare. We report a case of teratoid carcinosarcoma of the ovary occurring in a 40-year-old female. The resected tumor measuring over 20 cm in diameter consisted of cystic and solid components and was very fragile. Microscopic examination showed a heterogenous mixed tumor composed of malignant epithelial, malignant mesodermal and malignant neuroectodermal components. The cells of ganglioneuroblastoma-like area were positive for neural markers (Synaptophysin, S-100 protein, neuron-specific enolase). There was no tumor immunoreactivity to alpha-fetoprotein, carcinoembryonic antigen, human chorionic gonadotropin, and inhibin. In spite of aggressive combination chemotherapy and three times of laparotomy, the patient died of disease 3 years 10 months after the initial treatment. This quite rare ovarian tumor closely resembled nasopharyngeal tumors described as 'teratoid carcinosarcoma' is biologically aggressive. We report the fourth case of ovarian teratoid carcinosarcoma. Further cases need to be accumulated to make diagnosis and to determine a successful treatment modality.