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Leptomeningeal
dissemination of low-grade gliomas in childhood
LA
Civitello, RJ Packer, LB Rorke, K Siegel, LN Sutton and L Schut
Division
of Neurology, Children's Hospital of Philadelphia, PA 19104.
Although
leptomeningeal spread (LMS) of primary CNS tumors in children has
been well documented in the literature, it has rarely been
reported in children with low-grade gliomas.
Between
1975 and 1985, 6 of 162 children (3.7%) with low-grade
gliomas treated at Children's Hospital of Philadelphia had
LMS.
LMS
was present at diagnosis of the original tumor in one
patient, was the first sign of relapse in one patient, occurred simultaneously
with local relapse in two patients, and after local relapse in
two patients.
Pathology
of the original tumor was low-grade astrocytoma in five and
low-grade oligodendroglioma in one.
Primary
tumor site was cervical cord in three, chiasm in one,
frontal lobe in one, and cerebellum in one.
All
of the children with LMS had undergone surgical treatment at the
time of diagnosis of the primary tumor; four had total resections at
some point in their course.
Three
of the six patients died; three are still alive after
treatment with radiation therapy and/or chemotherapy.
The
longest survival to date has been 3 1/2 years after diagnosis of
LMS.
We
compared clinical characteristics of these six patients with 131
children with low-grade tumors without dissemination
treated at our institution during the same time
period.
LMS,
although relatively infrequent, does occur in children with
low-grade gliomas, especially spinal cord tumors.
LMS
may occur at any time during illness and diagnosis may be difficult
unless LMS is suspected.
Treatment,
at times, results in clinical improvement and considerable
disease control.
Copyright
© 1988 by American Academy of Neurology
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