Gliomatosis
cerebri
Gliomatosis
cerebri
(infiltrative
diffuse
astrocytosis)
is a rare
primary
brain tumor.
It is
commonly
characterized
by diffuse
infiltration
of the brain
with
neoplastic
glial cells
that affect
various
areas of the
cerebral
lobes.
While
gliomatosis
cerebri can
occur at any
age, it is
generally
found in the
third and
fourth
decades of
life. It may
affect any
part of the
brain or
even the
spinal cord,
optic nerve
and compact
white
matter.
Clinical
manifestations
are
indefinite,
and include
headache,
seizures,
visual
disturbances,
corticospinal
tract
deficits,
lethargy,
and
dementia.
Before the
advent of
MRI,
diagnosis
was
generally
not
established
until
autopsy.
Even with
MRI,
however,
diagnosis is
difficult.
Typically,
gliomatosis
cerebri
appears as a
diffuse,
poorly
circumscribed,
infiltrating
non-enhancing
lesion that
is
hyperintense
on
T2-weighted
images and
expands the
cerebral
white
matter. It
is difficult
to
distinguish
from highly
infiltrate
anaplastic
astrocytoma
or GBM. The
prognosis
for
gliomatosis
cerebri is
generally
poor, with a
median
survival
time of only
12 months.
Surgery is
not
practical
considering
the extent
of the
disease,
standard
chemotherapy
(nitrosourea)
has been
unsuccessful,
and while
brain
irradiation
can
stabilize or
improve
neurologic
function in
some
patients,
its impact
on survival
has yet to
be proven. |
