Ependymomas and ependymoblastomas in children

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✓ 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.

Article Information

Address reprint requests to: George J. Dohrmann, M.D., Ph.D., Sections of Neurosurgery and Neuropathology, Yale University School of Medicine, New Haven, Connecticut 06510.

© AANS, except where prohibited by US copyright law.

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Figures

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    Graph showing patients with ependymoma and ependymoblastoma by year of diagnosis. The incidence of ependymal tumors has been relatively constant in Connecticut children from the mid-1950's.

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    Left: Graph showing incidence of intracranial ependymoma and ependymoblastoma in children. Half of the cases occurred before the age of 5 years, with a peak incidence at 1 year. Right: Graph showing that the distribution of age at diagnosis is similar for ependymoma and ependymbolastoma.

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    Graph showing incidence of ependymoma and ependymoblastoma in children by location. Infratentorial tumors occurred more often in the first 4 years of life than supratentorial tumors.

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    Upper: Graph showing survival time from diagnosis in children with intracranial ependymoma and ependymoblastoma. Half of the children died within 1 year of diagnosis. Lower: Graph showing relative survival times for ependymoma and ependymoblastoma. Overall survival curves are similar for the two histopathological types of ependymal neoplasm.

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    Graph showing relation of survival time to location of ependymoma and ependymoblastoma. Children with supratentorial tumors had a longer survival, the difference becoming significant (p < 0.05) from 42 months following diagnosis.

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