paediatrics Brussels 17

1440 / 1708

CLINICAL INVESTIGATION Brain
INTRACRANIAL EPENDYMOMAS IN CHILDREN: SOCIETY OF PEDIATRIC
ONCOLOGY EXPERIENCE WITH POSTOPERATIVE HYPERFRACTIONATED LOCAL
RADIOTHERAPY
CE´ CILE CONTER, M.D., CHRISTIAN CARRIE , M.D.,y VALE´ RIE BERNIER, M.D.,z ANNE GEOFFRAY, M.D.,x
ANNE PAGNIER, M.D.,{ JEAN-CLAUDE GENTET, M.D.,jj ARIELLE LELLOUCH-TUBIANA, M.D.
SYLVIE C HABAUD, M.D. AND DIDIER FRAPPAZ, M.D
Departments of *Pediatrics, y Radiation Oncology, and **Biostatistics, Centre Le´on Be´rard, Lyon, France; z Department of Radiation
Oncology, Centre Alexis Vautrin, Vandoeuvre les Nancy, France; x Department of Radiology, Fondation Lenval, Nice, France;
{ Department of Pediatrics, CHU de Grenoble, Grenoble, France; jj Department of Pediatrics, Hoˆpital La Timone, Marseille, France; and
# Department of Pathology, Hoˆpital Necker-Enfants Malades, Paris, France
Purpose: To prospectively investigate the role of local hyperfractionated radiotherapy (RT) after surgical resection
in the treatment of intracranial ependymomas in children.
Patients and Methods: Postoperative local hyperfractionated RTwas proposed for every child (>5 years old at di-
agnosis) with localized intracranial ependymoma. The planned dose was 60 Gy after complete resection (CR) and
66 Gy after partial resection, delivered in two daily fractions of 1 Gy, according to the early postoperative imaging
findings.
Results: Between November 1996 and December 2002, 24 children with infratentorial (n = 20) or supratentorial
(n = 4) intracranial ependymoma were included. The median age was 8.6 years (range, 5–17). The World Health
Organization grade was anaplastic in 10 of the 24 patients (not assessable in 1). After a retrospective central review,
a CR was reported in 16 patients, partial resection in 4, and doubtful resection in 4. The radiation dose was 60 Gy in
18 cases (one partial resection), 66 Gy in 5 cases (one CR), and 54 Gy in 1 case (CR). The 5-year overall survival rate
was 74.8%, and the progression-free survival rate was 54.2%. Of the 24 patients, 11 developed a relapse: 7 local
only and 4 metastatic and local. The histological grade and extent of resection were not prognostic factors. More
than 3 in 4 children had no sequelae of RT at a median follow-up of 7 years (95% confidence interval, 66.4–90.0
months).
Conclusion: The results of our study have shown that hyperfractionated RT is safe but provides no outcome benefit
compared with other strategies of RT such as standard fractionated regimens. 2009 Elsevier Inc.
Ependymoma, Bifractionated radiotherapy, Long-term effects, Children.
INTRODUCTION
Ependymomas (EPs) account for 6–12% of all primary cen-
tral nervous system tumors occurring in children. The mean
age at diagnosis is 3 years, and one-half of cases occur before
5 years old More than 90% of childhood EPs are intracra-
nial, and two-thirds occur below and one-third above the ten-
torium The main signs and symptoms are not specific and
depend on the tumor location and the associated increased
intracranial pressure . The management of EP is one of
the most controversial issues in pediatric oncology, because
most of our knowledge has been derived from single-institu-
tional series spanning many years. For older children,
because EP is classically considered a chemoresistant
‘‘local’’ disease, surgery and postoperative local radiotherapy
(RT) are considered essential for successful treatment. Re-
search has thus focused on precision RT techniques enabling
local dose escalation to increase local control. The aim of hy-
perfractionated RT (HFRT) is to improve the therapeutic ra-
tio, by enhancing the antitumor effect without increasing the
incidence of late effects. Some studies have reported promis-
ing results with HFRT for medulloblastoma and EP
For children <5 years old, because RT, in particular, results
in the risk of side effects that negatively affect cognition, as
Reprint requests to: Cecile Conter, M.D., Department of Pediat-
rics, Centre Le´on Be´rard, 28 rue Laennec, Lyon 69008 France.
Tel:(+33) 478-78-2881 ; Fax:(+33) 478-78-2703 ; E-mail:
Conflict of interest: none.
Acknowledgments—The authors are indebted to P. Thiesse for the
radiologic review; V. Fouillat for data management; the physicians
from the Society of Pediatric Oncology centers for including pa-
tients (E. Plouvier and F. Demeocq, M.C. Baranzelli, F. Doz, A. Ba-
bin, and F. Millot); M.M. Ruchoux and A. Jouvet for the pathology
review; and M.D. Reynaud for editorial assistance.
Received July 4, 2008, and in revised form Sept 6, 2008.
Accepted for publication Sept 28, 2008.
1536
Int. J. Radiation Oncology Biol. Phys., Vol. 74, No. 5, pp. 1536–1542, 2009
Copyright 2009 Elsevier Inc.
Printed in the USA. All rights reserved
0360-3016/09/$–see front matter
doi:10.1016/j.ijrobp.2008.09.051