Malignant neuroectodermal tumor of the ovary, a distinctive form of monodermal teratoma

Abstract

Five cases of ovarian tumor with an exclusive or almost exclusive malignant neuroectodermal composition are reported. Four were unquestionably primary, but one, although probably primary, was possibly metastatic from the adrenal gland. The ages of the patients with tumors in the former category ranged from 13 to 17 with an average of 15 yr; the 5th patient was 18 yr old. The presenting symptoms were similar to those of other ovarian cancers. Two of the definitely primary tumors were pure while 2 others contained minor foci of mature teratoma. Only one patient with an unquestionable primary ovarian tumor survived for more than 7 yr; the others died of tumor from 2 mo. to almost 4 yr postoperatively. The fifth patient presented with unilateral ovarian involvement by neuroblastoma and abdominal metastases; autopsy over 6 mo. 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.