Contribution to Disease

Ependymomas and ependymoblastomas develop from the ependymal cells surrounding the ventricles and the central canal of the spinal cord. Intracranial ependymomas occur predominantly in children and tend to fill the ventricular lumen. The mean age at diagnosis of ependymoma and ependymoblastoma is 5 years. The incidence of these ependymal tumors in males and females is approximately equal. Clinical symptoms, although related to the location of the tumor, are usually due to blockage of CSF fluid flow, which may lead to a sudden increase in intracranial pressure. Ependymal tumors are sensitive to radiation.

Subependymomas are slow-growing, benign neoplasms originating from the subependymal glial matrix, consisting of a mixture of astrocytic, ependymal, and transitional cell clusters surrounded by their fibers. They generally project into the ventricular lumen. About one-fourth of the symptomatic intracranial tumors have mixed tumor cell populations and consist of a mixture of ependymomas and subependymomas. The prognosis in these cases is worse than that of a pure ependymoma. Another factor that affects the prognosis is the size of the neoplasm; symptomatic tumors tend to be large. Subependymoma is more common in men and has been reported in all decades of life, although no cases of subependymomas have been reported in children less than 2 years of age. The mean age for symptomatic tumors is 39 years, whereas that for asymptomatic lesions is 59 years. Microscopically, subependymomas consist of a nest of glial cells separated by glial fibers. Histologic studies have shown that some of the cells within these glomerate nests have attributes of ependymal cells and others of astrocytes; still others are transitional between the two.

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