paediatrics Brussels 17

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(i.e., carboplatin, etoposide, vincristine, and ifosfamide) was
followed by 70.7-Gy bifractionated RT. Similarly, Massi-
mino et al. reported on the Associazione Italiana di
Ematologia-Oncologia Pediatrica strategy using vincristine,
etoposide, and cyclophosphamide with HFRT at a dose
of 70.4 Gy, with a 5-year OS rate of 75% and a PFS rate of
56%. Their results were also very encouraging, with 9 of
12 responses to RT and 15 of the 23 relapses being local.
In the present study, the 5-year OS (74.8%) and PFS
(54.2%) rates were roughly similar, despite lower radiation
doses (range, 60–66 Gy) and the lack of associated chemo-
therapy. The rate of response to RT was 3 of 4 and most
(10 of 11) relapses were local. However, the administration
of HFRT is more complicated than standard RT. Only one-
third of our patients could receive RT within the first 30
days after surgery. This delay was not just related to postop-
erative complications. Whether monofractionated RT would
have resulted in shorter delays is not clear. Most recent stud-
ies have used standard local or craniospinal or conformal
RT The 5-year OS rate was 65–76%, and the PFS rate
was 50–75% The results of the five major
studies are reported in . The most encouraging results
have been reported by Merchant et al. This unicentric
study also included young patients (median age, 2.8 years).
The high rate of complete surgical removal obtained in the
present study might have been because of the high number
of second-look surgeries. The rate and type of postoperative
complications have not been clearly reported. The 1-cm
safety margin is small and requires perfect immobilization
of the patient. The RT procedures are very sophisticated
and thus often require general anesthesia, which can be
difficult to perform in a multi-institutional setting. The
3-year PFS findings have been reported, and longer follow-
up is needed to ensure that relapse will not occur. Whether
such encouraging results will be confirmed by the multi-
institutional ACNS 0121 Children’s Oncology Group study
remains to be demonstrated. The major concern with RT
delivered to young children is long-term neuropsychological
and endocrine sequelae. It is difficult to compare series that
do not always prospectively report such complications and
for which no follow-up data are available. Grill et al.
reported that 11 patients with EP who underwent local RT
of the whole posterior fossa at 55 Gy had a mean full-scale
IQ of 84.2, with the verbal IQ superior to performance IQ.
Of these 11 patients, 94% were able to attend normal school-
ing . Our series showed that about three-quarters of long-
term survivors were free of neuropsychological, endocrine,
or hearing troubles and have normal school results. The
visual sequelae are mostly strabismus, which is more likely
a result of surgery than to RT.
CONCLUSION
The results of the present study have demonstrated that
local HFRT is feasible for the treatment of EP. Whether the
low rate of long-term sequelae resulted from the procedure
remains to be demonstrated. Only one-half of the children
treated were cured. Whether standard 59.4 Gy will result in
greater PFS at 5 years also remains to be clarified. Moreover,
the role of adjuvant treatment by chemotherapy or innovative
treatments deserves additional randomized evaluation.
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