Ependymomas and ependymoblastomas in children

Abstract

✓ The authors analyze histologically verified cases of ependymoma and ependymoblastoma (malignant ependymoma) occurring in children in Connecticut from 1935 to 1973. Of the 488 central nervous system tumors diagnosed in that period, 44 (9%) of the 467 intracranial neoplasms and five (24%) of the 21 intraspinal tumors were of ependymal origin. An increase in the incidence of ependymomas was noted since the mid-1950's. The mean ages at diagnosis of ependymomas and ependymoblastomas were 5.6 and 5.0 years respectively. The male to female ratio was 0.6:1 for ependymomas and 1.7:1 for ependymoblastomas. Ependymomas were found above and below the tentorium with similar frequency; however, virtually all of the ependymoblastomas occurred supratentorially. Presenting symptoms and physical findings were reviewed. A significant difference (p < 0.05) was noted in the seizure rates of supratentorial ependymomas (9%) and ependymoblastomas (38%). A significantly increased survival (p < 0.05) was associated with supratentorial ependymal neoplasms relative to infratentorial from 42 months following diagnosis onward. Contrary to the reports of no clinical difference between ependymomas and ependymoblastomas, children with supratentorial ependymomas were noted to have a significantly longer survival (p < 0.05) than those with similarly situated ependymoblastomas, with the difference noted from 18 months following diagnosis onward. The children treated by operation and irradiation had a significantly greater survival (p < 0.05) than those treated by other methods; furthermore, with this treatment, longer survivals were noted in the ependymoma patients as compared to those with ependymoblastomas. This difference became significant (p < 0.05) at 27 months after diagnosis. Operative mortality decreased from 40% to 17% in the last decade of the study as compared to the previous decade. Steroid therapy may have contributed to this decreased operative mortality, but it had no statistically significant effect on length of survival. The clinical course of intracranial ependymal neoplasms in adults and children was compared and appeared to be essentially the same.