Malignant neuroectodermal tumor of the ovary, a distinctive form of monodermal teratoma: report of five cases

Five cases of ovarian tumor with an exclusive or almost exclusive malignant neuroectodermal composition are reported. Four of them were unquestionably primary, but one, although probably primary, was possibly metastatic from the adrenal gland. The ages of the four patients with tumors in the former category ranged from 13 to 17 with an average of 15 years; the fifth patient was 18 years old. The presenting symptoms were similar to those of other ovarian cancers. Two of the definitely primary tumors were pure while two others contained minor foci of mature teratoma. Only one of the four patients with an unquestionable primary ovarian tumor survived for more than 7 years; the others died of tumor from 2 months to almost 4 years postoperatively. The fifth patient presented with unilateral ovarian involvement by neuroblastoma and abdominal metastases; autopsy over 6 months later revealed involvement of both adrenal glands. This case appears to be the first recorded case of neuroblastoma presenting clinically as a primary ovarian cancer.