Metastatic neuroblastoma arising in an ovarian teratoma with long-term survival. Case report and review of the literature

A case of neuroblastoma arising in an immature teratoma of the ovary in a 22-year-old woman is reported. Differentiation was grade 3 in the primary and metastases. Metastases to retroperitoneal, mediastinal, and supraclavicular lymph nodes and to bone were diagnosed 2 years after presentation of the primary tumor. Electron microscopic study demonstrated dense-core granules of the neurosecretory elements. Treatment with combination chemotherapy followed by radiotherapy resulted in complete remission which continues to the time of this report, more than 4 years after diagnosis of the primary tumor. Prolonged survival of metastasizing grade 3 immature teratoma is distinctly uncommon. The literature pertaining to this unusual tumor and to neuroblastoma of adults is reviewed.