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complete remission, 1 had stable disease, and 1 had disease
progression.
Relapse developed in 11 patients. The rates of relapse were
similar between patients with Grade 2 and Grade 3 tumors
(6 of 13 vs. 5 of 10, respectively;
Table 2 ). The patient whose
histologic grade was not assessable did not develop a relapse.
One-half of the patients with infratentorial and the one-fourth
of patients with supratentorial EP developed a relapse. The
rate of relapse was similar among the patients with CR, in-
complete resection, or doubtful resection (7 of 16, 2 of 4,
and 2 of 4, respectively). Disease recurred once in 8 patients,
twice in 2, and five times in 1 patient. The median time to the
first relapse was 22 months (range, 4–46). Seven patients had
only local failure; one had an isolated distant failure; and
three had combined failure
( Table 2 ). Of the 10 local relapses,
9 were infield relapses and 1 occurred outside the radiation
field.
The treatment of failure was surgery alone in 1 patient,
chemotherapy alone in 2, RT alone in 1, surgery and chemo-
therapy in 3, surgery, RT, and chemotherapy in 3, and palli-
ative treatment in 1 patient. The median survival after relapse
was 12.5 months (range, 0–44).
Long-term side effects
The long-term side effects among the 16 patients alive at
completion of the study are reported in
Table 3 .Three-fourths
of the patients had normal psychomotor development, as as-
sessed by the local physician. Of the 16 patients, 9 underwent
an IQ test. Wechsler scale III or IV were used, depending on
the age of the patient. After a median follow-up of 41 months
(range, 7–66), the mean verbal IQ was 93 (range, 54–130),
and the mean performance IQ was 88 (range, 42–111).
Two patients required placement in a special school, and
two were
$
2 years behind at school. One patient had tran-
sient growth hormone deficiency requiring growth hormone
supplementation with rapid normalization of the size. No
other endocrine disorder was reported, but 4 of the 24 patients
were in prepuberty stage. Their size was normal (with 1 to
+1 standard deviation for age and gender) in all patients. One
patient with a supratentorial tumor had a severe decrease of
visual acuity secondary to high intracranial pressure with
optic atrophy and required adapted schooling.
DISCUSSION
Treatment of EP is one of the most controversial issues in
pediatric oncology. The published data in the field have
mostly been mono- or oligocentric studies and mainly retro-
spective. Few randomized studies have been reported. The
prognostic factors and oncologic strategies are a matter of de-
bate. CR is usually shown as the main prognostic factor
(2, 14, 15) ,although some series
(6), including ours, have failed
to confirm this finding. In most series, CR was obtained in
about one-half of patients, although surgery is generally
less successful in patients with infratentorial lesions
(14) .Pathologic grading is a matter of major debate. Some series
have described it at a major prognostic factor
(16–18) ,and
others have failed to show any difference with grade
(9, 19–21). The results obtained in our series failed to show
any difference. Whether this was a result of the low number
of patients included or differences in the grading assessment
remains to be demonstrated. Recently, discrepancies between
histologic assessments have been reported in infants
(9, 10) .Whether this applies only to infants or also is true for older
children remains unresolved. An international panel of
experts is currently reviewing such cases to answer this
question.
Table 2. Pattern of failures
Variable
Local
(
n
= 7)
Distant
(
n
= 1)
Distant and
local (
n
= 3)
Grade
2 (
n
= 6)
3
1
2
3 (
n
= 5)
4
0
1
Location
Infratentorial (
n
= 10)
6
1
3
Supratentorial (
n
= 1)
1
0
0
Extent of resection
Complete (
n
= 7)
5
0
2
Partial (
n
= 2)
0
1
1
Doubtful (
n
= 2)
2
0
0
Table 3. Long-term side effects among 16 living patients
Variable
Patients
evaluated (
n
)
Sequelae
Auditory
15
Normal
12
Loss <40 dB at 1,000–8,000
Hz on one ear
1
Loss >40 dB at 1,000–8,000
Hz or <40 on both ears
2
Schooling
16
Normal school or university
(possibly 1 y behind)
12
Normal school but >2 years
behind
2
Special school and currently
employed
1
Special school and currently
unemployed
1
Vision
16
Normal
9
Diplopia
5
Mild decrease of visual acuity
1
Severe decrease of visual
acuity
1
Psychomotor development
16
Normal
12
Mild retardation
2
Severe retardation
2
Endocrine deficit
16
None
15
Hypothyroidism
0
Growth hormone deficiency
1
Premature or delayed puberty
0
*
* Four in prepubertal stage.
Bifractionated RT in childhood ependymoma
d
C. C
ONTER
et al
.
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