Annals of Diagnostic Paediatric Pathology

87
nancy in ependymomas [7, 21]. Opinions on adjuvant therapy
differ from series to series. It is generally accepted that radiotherapy
and chemotherapy can delay tumor recurrence but their
influence on overall survival is still unclear [1, 5, 23, 28]. The
rationale for agressive removal of ependymomas in certain
locations is debatable. Devastating late complications of
neuroaxis radiotherapy, especially in the youngest age group
are well known [17, 18, 32]. That is why there is a trend to
eliminate radiotherapy in children under 3 years of age and
after total removal of histologically benign tumors. The fields
of irradiation were restricted to tumor bed or tumor bed and
ventricles for partially resected benign and anaplastic lesions
respectively. The neuroaxis irradiation is usually reserved for
disseminated ependymomas [12, 26]. This tendency is supported
by the observations that reccurences are limited to primary
tumor location in almost all cases and CSF dissemination
is relatively rare [13]. Patterns of failure indicate, that
final treatment outcome depends mostly on the local control
of disease. These statements were the reason for the concept
of “second-look” surgery, whenever reoperation was possible
in cases of residual disease [8]. Chemotherapy was introduced
in the treatment of children under 3 years to delay or
eliminate the need for radiotherapy as well as in the treatment
of malignant lesions and tumor recurrences and disseminations
and finally in the treatment of partially resected benign
lesions [15, 37]. Variable response rates to the same treatment
protocols created the need for potential identification of subpopulations
sensitive or resistent to chemotherapy,
developement of novel drugs and intensification of treatment
with subsequent bone marrow reconstruction [10, 14, 22]. As
mentioned above, in our series neuroaxis irradiation correleated
with better actuarial EFS for benign ependymomas. Significant
change of treatment protocols over the years resulted in
fairly confusing data. Before 1996, all cases were treated with
neuroaxis irradiation; after 1996, RT was reserved only for
disseminated ependymomas. It was clearly shown that
neuroaxis RT did not improve EFS in anaplastic ependymomas
in our series. An introduction of standarized treatment
protocols (including chemotherapy) in 1997 improved treatment
outcomes in malignant tumors. Chemotherapy also improved
control of small tumor residues after surgery. Treatment
response of tiny remnants seems to be an interesting
clinical model for evaluation of CHT efficacy in the treatment
of ependymomas.
Conclusions
1. For the entire group of patients, age, extent of surgical
resection, and histological malignancy, significantly influenced
EFS;
2. Extent of surgical resection significantly influenced EFS in
benign tumors and did not influence EFS in anaplastic
ependymomas;
3. Neuroaxis RT did not influence EFS in patients with anaplastic
ependymomas;
4. Treatment results in anaplastic ependymomas improved after
1997 (a potential role of CHT);
5. CHT significantly improved control of small tumor residues.
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