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82 M. Reni et al. / Critical Reviews in Oncology/Hematology 63 (2007) 81–89
8. Follow-up . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 87
8.1. Follow up . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 87
Acknowledgment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 87
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 87
Biographies . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 89
Abstract
Ependymomas are rare tumours of neuroectodermal origin classified as myxopapillary ependymoma and subependymoma (grade I),
ependymoma (grade II) and anaplastic ependymoma (grade III). The more common location is infratentorial (60%). Age <40 years and extent
of surgery appear related to better prognosis, while the role of other prognostic factors, such as tumour grade and tumour site are equivocal.
This emphasizes the role of surgery as the standard treatment. Postoperative radiotherapy is indicated in high-grade ependymomas, and is
recommended in low-grade ependymomas after subtotal or incomplete resection (confirmed by postoperative MR). Deferral of radiotherapy
until recurrence may be considered on an individual basis for patients with MR confirmation of a radical resection. Recommended dose
to involved fields is 45–54 Gy for low-grade (grade II) and 54–60 Gy for high-grade ependymomas (grade III). There is no proof that
postoperative chemotherapy improves the outcome. At recurrence, platinum-, nitrosourea- or temozolomide-based chemotherapy can be
administered, although there is no evidence of efficacy.
© 2007 Elsevier Ireland Ltd. All rights reserved.
Keywords: Ependymomas; Treatment; Prognosis
1. General information
1.1. Definition
Ependymomas are rare tumours of neuroectodermal origin
arising from ependymal cells in the obliterated central canal
of the spinal cord, the filum terminale, choroid plexus or white
matter adjacent to the highly angulated ventricular surface
Additionally, ependymomas can be found in the brain
parenchyma as a result of fetal ependymal cell rests migrating
from periventricular areas
1.2. Incidence
About 500 new cases of ependymoma are diagnosed in
the European Union each year. The annual incidence rate in
Europe is around 2 per million pendymoma is slightly
more frequent in males than females nd occurs in all
age groups. About 15% of all patients are children of less
than 5 years of age In children and in the countries of
Central and South America and Asia, the annual incidence of
ependymoma is less than 2 per million n North America,
Oceania and most of Europe, the rate varies between 2 and 4
per million. In Denmark, Sweden, Finland, the former East
Germany and Slovenia, the incidence is at least 4 per million.
1.3. Survival
In Europe, 1-year survival for adults diagnosed with
ependymoma during 1990–1994 was 82% and 5-year sur-
vival 72%, with no difference between men and women
Five-year survival decreased markedly with age from 79% in
the youngest (15–45 years), to 44% in the oldest age group
(patients 75 years and over) he 1-year survival for chil-
dren with ependymoma in Europe, between 1990 and 1994,
was 80%, at 5-year 55%. Survival was very poor in infants
(24%), then increased with age: in children aged 1–4 years
44%, at 5–9 years 68% and at 10–14 years 75%
1.4. Aetiology and risk factors
Little is known about the aetiology of ependymomas. A
possible relationship between the presence of polyomavirus
SV40 has been suggested ut could not be confirmed by
other studies rom a recent study ver a 55-year
period (69.5 million person years), cancer incidence was not
shown to be associated with exposure to SV40-contamined
poliovirus vaccine. Incidence data on ependymoma were
carefully reviewed because of an increase in ependymoma
incidence among children aged 0–4 years in exposed periods
and compared with the incidence in preceding unexposed
periods. However, the incidence of ependymoma was rela-
tively low in the years of SV40 contamination, with a highest
incidence observed in 1964, when most children were too
young to have received the SV40-contaminated vaccine. An
increased incidence of ependymomas has also been reported
in neurofibromatosis type 2 An inverse association
between maternal consumption of vitamin supplements dur-
ing pregnancy and brain tumours has been observed in five
out of six studies on this subject nd one of these studies
related specifically to the category of ependymomas
2. Pathology and biology
2.1. Histopathology
According to the World Health Organization (WHO)
ependymal tumours are being classified as WHO grade 1:
myxopapillary ependymoma (occurring almost exclusively