Material and Methods:
Forty-nine children operated for a central nervous system ependymoma
between 2002 and 2018: 33 were located in the cerebral posterior fossa.
Among these patients, seven were recurrences (five patients were
operated on in another center for the first surgery). The mean time for
diagnosis was 1 month. The clinical picture was dominated by an
aspecific intracranial hypertension in 18 patients (54.5%), a
torticollis in six patients (18.18%) and cerebellar troubles in four
patients (12.1%). All children had a cranio-spinal MRI scan before
removal of the tumor. All patients had a anatomopathological study of
cerebrospinal fluid (CSF) after an endoscopic third
ventriculocisternostomy (ETV) for 42.4% of cases or by a lumbar
puncture at 15 days after surgery for the remaining 57.6%. Fourteen
patients (42.4%) had an ETV before surgery. The mean time between VCS
and surgery was 3 days. Surgery was performed in sitting position in 32
patients and in prone position in 1 patient for an in interventricular
cardiac shunt defect. The removal was assessed by a post-operative MRI
performed within 24/48 hours (Figure 1). A residue was reported in seven
patients on the post-operative MRI (78.12% total resection) while the
resection has been judged macroscopically complete in all but three by
the surgeon. In five patients the complete resection was obtained after
a second-look surgery before further treatments. Sixty-five surgeries
were performed for 33 patients (33 initial + 32 for recurrence). Two
patients were operated on for a spinal metastasis. All patients had a
complementary treatment: chemotherapy alone for children younger than 3
years of age and/or combined with local radiotherapy even for infants.
Eleven patients received postoperative chemotherapy (6 neoadjuvant and 5
relapse) and 35 radiotherapy (24 neoadjuvant and 11 cases at relapse)
including neoadjuvant proton therapy (4 neoadjuvant cases and 2 at the
relapse). The dose varied from 54 to 59.4 Gy according the SFOP
protocols. Concerning chemotherapy: 2 protocols were used the ”BB-SFOP”
with 3 cycles alternating every 21 days with 7 cycles in total and the
SIOP EPENDYMOMA II.
All patients, except one who died in the 1st year of surgery, benefited
during the follow-up of a cerebrospinal MRI every 3 months in the first
year, every 6 months for two consecutive years and every year until the
fifth year. Endocrine sequels after radiotherapy were observed in 3
patients (9.09%). A neuropsychological assessment was performed in all
children at 1 and 3 years of surgery by a pediatric neuropsychologist
with an age-appropriate IQ evaluation and the Vineland Adaptive
Behaviour Scales (VABS). Quality of life was analyzed in all 21 alive
patients. Survival curves (OS), were calculated according to
Kaplan-Meier curves for overall survival, 5-year survival,
progression-free survival (PFS). Differences with a p < 0.05
were considered statistically significant. Quality of life and overall
motor function were scored with the Barthel Index and the Euro Qol 5
Dimensions (EQ-5D) scale. Educational outcomes were classified as normal
and assisted schooling or specialized institution. No patient was lost
to follow-up. The study was accepted by the local ethical committee
Results :
The mean age at diagnosis was 5.8 years old (median 4.7 years old,
range: 9 months to 18 years). The most represented age range was between
3 and 6 years old with 12 cases (36.4%). There was a predominance of
males, with 23 males for 10 females. The lesions were located in the
fourth ventricle in 28 patients (84.8%), in the cerebellum and the
cerebellopontine angle in 3 (9.1%) and 2 cases (6.1%) respectively. At
the time of diagnosis, tumor cells were found in one patient’s CSF.
Twenty patients had a Grade II and 13 had a Grade III, and we noted a
Grade II to Grade III mutation at recurrence in 2 patients. The majority
of the EpPCF were of the PFA group (90%). The main complications in the
early postoperative period were cranial nerve palsy in 14 patients. A
patient could have several nerves affected at the same time; facial
nerve damage in 4 patients, abducens nerve damage in 5 cases, and
oculomotor nerve damage in 2 patients. Six had mixed nerve damage, 2 of
which required tracheotomies. A cerebellar mutism was observed in 2
patients. The overall survival rate of our series was of 63,6% and the
mortality was of 36,3 % (n=12). The median survival was of 122 months
and the median was of 104,7 months. The 10-year survival was 42,4 %
(n=14) and the five years’ survival was of 63,6 % (n=21). The
progression-free survival rate at 5 years was 57.69%. Male’s and
female’s OS (65.2% and 66.7% respectively) did not differ
significantly(p> 0.05) (Figure 2). OS was of 73.7% and
53.8% for WHO grade II and III (p> 0.05) (Figure 3).
Sixteen patients (48%) had a recurrence (10 patients Grade II and 6
patients Grade III). Eight were children (47%) aged 0 to 3 years. Six
patients (20%) were submitted to a second-look surgery to obtain a
complete removal that was possible in four patients after the control
MRI. In two patients, histology revealed an inflammatory granuloma. One
patient refused surgery after a first recurrence and 2 others after a
4th recurrence. The metastasis rate after the initial surgery was 18.2%
(6 patients) (Figure 4).
Twelve patients died: 10 for a progression (despite an initial complete
removal) after relapse surgery and additional treatment. One patient
died after a recurrence and the family refused a new operation and
another after post-operative bulbar respiratory complications.
Morbidity was represented by: facial nerve palsy in 4 cases and
swallowing troubles in 3 cases. Four patients had transient cerebellar
syndrome. Five patients needed a ventriculo-peritonea shunt. Fifteen
patients had a normal school program; six patients have had special
schooling (Auxiliary for School Life). All had been assessed by the
Service Mobile d’Accompagnement d’Evaluation and de Coordination
(SMAEC). Among the 9 adult patients, 6 completed their studies and 3 are
still students. Eight adults have normal full-time employment. We could
identify 3 groups of patients: patients with a complete removal and
alive (18 patients), patients with a complete removal and dead (8
patients) and patients with an incomplete removal (7 patients).
In the first group, four patients presented a recurrence (in the
cerebello-pontine angle one case, two in the midline, at level of the
floor of the fourth ventricle one case. All 18 patients are alive with a
mean survival of 12 years (range: 6 to 19 years).
In the second group, one patient presented an extension at level of the
floor of the fourth ventricle, three patients an extension in the
cerebello-pontine angle of the left side and two of the right side and
one in the cerebellar hemisphere.
All received a complementary treatment: only radiotherapy (5 cases),
chemotherapy associated with radiotherapy (two cases) and only
chemotherapy (one patient aged two years old).
Five patients presented an ependymoma of grade III with PFA signature in
3. All patients presented a recurrence and all were operated with a new
complete removal and the survival varied from two months to 109 months
with a median survival of three years and one months.
In the third group, only two patients are alive and 5 died. In this
group, four patients had radiotherapy and three were treated with a
radiotherapy associated with chemotherapy.
Two patients were operated three times and one patient was operated five
times.
In this group, patient who presented a recurrence died after four years,
twenty-two years, one year and two years.
Five patients were treated with radiotherapy and two patients with
chemotherapy.