Received: 11 October 1999
Abstract The objective of this ret-
rospective study was to evaluate the
relative effect of surgery and radio-
therapy (RT) on the survival of 25
consecutive children and young
adults treated for ependymoma [18 in
the posterior fossa (PF), 2 in supra-
tentorial locations (ST) and 5 in in-
traspinal locations (IS)]. Five-year
survival rates for patients with PF,
ST and IS tumors were 28%, 0% and
100%, respectively. Total tumor re-
section was a positive prognostic
factor in the case of PF tumors. No
patients with subtotal removal of a
PF tumor survived for longer than
5 years. The effect of RT on survival
of patients with PF ependymomas in
this series was uncertain. No patients
with PF ependymoma had dissemi-
nated disease at diagnosis, and all tu-
mor recurrences were local. Based
on these observations, we see no in-
dications for craniospinal RT of PF
ependymomas, except in rare cases
of disseminated disease. If RT is giv-
en, it should only be targeted to the
tumor site. The two patients with ST
ependymoma died within 3.8 years
after primary treatment. Our series
of ST ependymomas does not allow
any specific treatment recommenda-
tions. One patient with IS ependy-
moma was cured by surgery alone.
Four patients with IS ependymoma
had documented residual tumor after
surgery. RT induced remission in
these patients. For IS ependymomas
we recommend no RT if total tumor
removal can be documented. In pa-
tients with subtotal removal of IS
ependymomas local RT is effective
and should be given.
Keywords Brain neoplasm ·
Ependymoma · Survival · Surgery ·
Radiotherapy
Child’s Nerv Syst (2001) 17:24–30
© Springer-Verlag 2001 ORIGINAL PAPER
Eirik Helseth
Bernt Due-Tønnessen
Knut Lote
Kari Skullerud
Ingebjørg Storm-Mathisen
Finn Wesenberg
Tryggve Lundar
Ependymoma in children
and young adults (0–19 years):
report of 25 consecutive cases
Introduction
Ependymoma is a central nervous system tumor that
arises from the ependymal cells lining the cerebral ven-
tricles and central canal of the spinal cord. Spinal epen-
dymomas have a better prognosis than intracranial epen-
dymomas, and the beneficial effect of radiotherapy (RT)
on survival of patients with spinal ependymomas is doc-
umented [17, 26, 31, 33]. The effect of RT on survival in
the case of intracranial ependymomas is still controver-
sial. It is known, however, that cranial radiation of chil-
dren may cause serious late sequelae, which are rarely
seen in children treated with surgery alone [3, 4, 10, 27,
29]. Some children with intracranial ependymoma are
cured by total surgical resection alone [11, 16]. It is
therefore extremely important to establish whether or not
postoperative RT increases survival in this subgroup of
patients. If no effect can be documented, postoperative
RT should not be given. The objective of this study was
to evaluate the relative effects of surgery and RT on sur-
vival in 25 consecutive children and young adults treated
for ependymoma in our departments from 1970 to 1998.
E. Helseth (
✉
) · B. Due-Tønnessen
T. Lundar
Department of Neurosurgery,
The National Hospital,
0027 Oslo, Norway
e-mail: eirik.helseth@rikshospitalet.no
Tel.: +47-23074342
K. Lote
Department of Oncology,
The Norwegian Radium Hospital,
Oslo, Norway
K. Skullerud
Department of Pathology,
The National Hospital,
Oslo, Norway
I. Storm-Mathisen · F. Wesenberg
Department of Pediatrics,
The National Hospital,
Oslo, Norway