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Here is a review of various aspects of ependymal neoplasm, (ependymoma and ependymoblatoma) including incidence, age and sex distributions, anatomic location, clinical features, therapy and prognosis. Ependymomas, are discussed relative to an over all population and then, because they occur most often in the first two decades of life, the aspects of ependymal neoplasm- occurring in childhood are discussed separately.

Ependymal Neoplasms in General

Incidence

The incidence of ependymal neoplasms, in series of primary intracranial tumors is approximately 5 percent, although it varies from series to series, (2 to 9 percent) relative to referral patterns and mean age of patients.

Age and Sex Distributions

Twenty-three years is the mean age of presentation of patients with ependymal neoplasms. At least half the ependymal neoplasms occur during the first two decade. of life. Although there is variation from one series to another, there is a a slight male predominance in the occurrence  of ependymal tumors.

Location

The majority of ependymomas occur beneath the tentorium: a good rule of thumb is, that one-third occur supratentorially and two-third , occur infratentorially. Approximately half the supratentorial tumors arise from the wall of the ventricle; the remainder arise in an area remote from the ventricular wall, presumably from fetal rests of ependymal cells. Almost all infratentorial ependymomas occur in the midline, often involving the floor of the fourth ventricle: these infratentorial tumors are more solid, while those occurring supratentorially have more cystic components.

Seeding of ependymal neoplasms via cerebrospinal fluid (CSF) pathways has been reported. The evidence of this is variable and varies also as was based on autopsy or on clinical data. At autopsy seeding of the spinal subarachnoid space is in 30% of patients with infratentorial ependymal tumors: however no such finding was, noted in patients with supratentorial ependymal tumors. Clinically, patients with intracranial ependymal seeding have place in 3% of cases.

Clinical Features

Symptoms and physical findings are related to the location of the tumor. In general the patients with ependymal tumors involving the cerebral hemispheres present with focal deficits, while those with infratentorial tumors present with nausea and vomiting and ataxia. Most patient, present with headache and papilledema, indicative of increased intracranial pressure. Seizures, are described in approximately one-third of patients with supratentorial tumors.




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