| Subependymomas |
Spinal Subependymomas |
Childhood Ependymomas
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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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