If. Pollack et al., THE LONG-TERM OUTCOME IN CHILDREN WITH LATE-ONSET AQUEDUCTAL STENOSISRESULTING FROM BENIGN INTRINSIC TECTAL TUMORS, Journal of neurosurgery, 80(4), 1994, pp. 681-688
Benign intrinsic tumors arising in the dorsal midbrain have long been
recognized as a potential cause of late-onset aqueductal stenosis. Whe
re histopathological studies of such lesions have been performed, the
majority have been reported to be low-grade gliomas. Because these tum
ors often present with a paucity of neurological findings and a charac
teristic radiographic appearance and because there has been substantia
l uncertainty regarding their potential for long-term progression, the
authors have routinely deferred biopsy and/or radiotherapy for these
lesions until there has been clear-cut evidence of disease progression
. Herein, the authors report their experience with 16 children manifes
ting this syndrome who were treated between 1979 and 1992. The patient
s ranged in age from 6 months to 14 years at presentation (median 9.75
years). In general, symptoms of increased intracranial pressure devel
oped insidiously; three of the older children had exhibited profound m
acrocephaly since infancy, which predated the onset of other symptoms
of hydrocephalus by several years. Only one of the 16 children showed
evidence of brain-stem dysfunction at presentation, a partial Parinaud
's syndrome that resolved following placement of a ventriculoperitonea
l shunt. In 12 patients, the tumor was detected by magnetic resonance
(MR) imaging at initial evaluation as a bulbous enlargement of the tec
tal plate. In four patients who presented before the advent of MR imag
ing, initial computerized tomography (CT) scans failed to delineate th
e tectal lesion convincingly:, however, subsequent MR studies clearly
demonstrated the presence of an intrinsic tectal mass. All 16 patients
underwent cerebrospinal fluid diversion initially, with conservative
management of the tectal lesion and close long-term follow-up monitori
ng. Four children ultimately demonstrated clinical signs of progressiv
e tumor growth with the insidious onset of partial or complete Parinau
d's syndrome, despite the presence of a functioning shunt. The median
interval to symptom progression was 7.8 years from the time of shunt i
nsertion and 11.5 years from the onset of initial symptoms and signs o
f hydrocephalus. Follow-up CT and MR studies demonstrated obvious tumo
r enlargement in three of the four patients who then underwent stereot
actic or open biopsy. The histological diagnosis in these three was be
nign mixed glioma, anaplastic astrocytoma, and low-grade astrocytoma.
All four patients with clinical evidence of disease progression were t
reated with conventional radiotherapy; the patient with an anaplastic
astrocytoma also received focal stereotactic radiosurgery. These patie
nts subsequently remained clinically stable, with three showing tumor
regression and one showing stable disease on serial MR studies (median
follow-up period from tumor progression, 4.25 years). One other child
was noted to have progressive tumor enlargement during the 2 years af
ter shunt insertion; she remains asymptomatic and has not yet undergon
e biopsy or radiotherapy. It is concluded that benign intrinsic tectal
tumors, although generally indolent, merit conscientious long-term fo
llow-up monitoring since these lesions may ultimately show evidence of
progressive growth and require therapeutic intervention to maintain d
isease control. These tumors are best visualized on MR imaging, which
should be included in the workup of all patients with late-onset aqued
uctal stenosis.