Ependymal Neoplasms in Childhood

Because half of the ependymal neoplasms (ependymomas and ependymoblastomas) occur in children, these ependymal neoplasms of the first two decades of life are considered separately here

Incidence

In a review of published series of primary intracranial neoplasms occurring in children. approximately 10 percent were of ependymal origin. The number of intracranial ependymomas is twice that of their more malignant counterparts ependymoblastomas.

Age and Sex Distributions

The mean age at diagnosis of ependymoma is 5 years and it is approximately the same for children with ependymoblastoma. Supratentorial ependymal tumors occur at a younger mean age than infratentorial tumors. Male and female incidence of intracranial ependymal neoplasms is approximately equal: however, a clear male predominance is noted in supratentorial ependymal tumor.

Location

Approximately half the tumors occur above the tentorium and half below, but this varies with the histologic type of tumor: 61 percent of ependymomas are infratentorial and 39 percent supratentorial. Whereas 81 percent of ependymoblastomas occur supratentorially and 19 percent infratentorially.

Clinical Features

Nausea and vomiting, headache and lethargy are the most common presenting symptoms. The majority of ependymomas occur infratentorially and the presenting physical findings include ataxia (80 percent), papilledema 40 percent and dysmetria 40 percent. Ependymoblastomas, mainly occur supratentorially and presenting physical finding, are papilledema (62 percent, cranial nerve palsies 46 percent and paresis 38 percent).

Preoperative and Operative Approach

The same as in the general group.

Adjunctive Therapy

There is no evidence that steroid therapy has had a statistically significant effect on survival. However, the use of steroids probably contributed to the decrease in operative mortality to 17 percent from a value over twice that before steroid therapy became available. Responsiveness of ependymal neoplasms to radiation has been reported to be greater in younger children, and the recurrence rate has been lowest when doses of more than 4500 rad have been used. Nonirradiated ependymal neoplasms have always recurred.

Prognosis

The longest survivals are in children treated initially by operation, followed by radiation. A median survival of 50 month, is noted in those children with supratentorial ependymal neoplasms and 31 month, in those with infratentorial neoplasms. In children with ependymoma, the 1-, 2-. and 5-year survivals are 81 percent, 71 percent and 21 percent, respectively. while they are 67 percent, 44 percent and 15 percent for children with ependymoblastomas. It is interesting to note that the survival of adult with ependymal neoplasm, is approximately the same as that of children, the clinical course of ependymal tumors appears to be the same regardless of whether the tumor occurs in an adult or a child. This emphasize, that the biological activity of the tumor is related more to the histologic type than to the patient's age.