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EVALUATION OF THE EFFECTIVENESS OF TREATMENT OF FOURTH
VENTRICLE EPENDYMOMAS IN IMMEDIATE AND LONG-TERM PERIODS IN
CHILDREN
Ashrapov J.R., Alikhodjaeva G.A., Atajanov Y.M.
Republican Specialized Scientific and Practical Medical Center of Neurosurgery
Tashkent Medical Academy
Abstract. Background
: Ependymomas of the fourth ventricle are a significant
clinical challenge in pediatric neurosurgery, representing a substantial portion of central
nervous system tumors in children. Prognosis largely depends on the tumor's anaplasia grade,
extent of surgical resection, and subsequent treatment strategies.
Methods
: A retrospective analysis was conducted on 49 pediatric patients (aged 2–
15 years) with fourth-ventricle ependymomas treated between 2022 and 2024. The degree of
tumor resection was assessed using the "Tumor Volume Calculation" program, and
postoperative outcomes, survival rates, and “quality of life” (QoL) were evaluated using
established scales.
Results
: Total tumor resection was achieved in 70.8% of patients, with postoperative
mortality rates of 3.1% and 5.0% for total and subtotal resections, respectively. Five-year
survival rates were 98.7% for Grade I–II tumors and 64.5% for Grade III tumors treated with
adjuvant therapies. QoL assessments revealed that 41.0% of patients had good QoL, 54.2%
satisfactory, and 4.8% poor. Extent of resection removal significantly impacted long-term
QoL, with total resections yielding superior outcomes (53.6% improvement).
Conclusion
: The effectiveness of treatment for pediatric fourth-ventricle
ependymomas depends on the extent of tumor resection and subsequent therapy. Total
resection, where feasible, significantly improves survival rates and long-term QoL.
Keywords:
Ependymomas, fourth ventricle, pediatric neurosurgery, tumor resection,
quality of life
Introduction
Tumors of the central nervous system (CNS) rank as the second most common
malignant neoplasms in children, following leukemia [2, 5]. In pediatric patients,
subtentorial brain tumors often exhibit a predominantly midline localization [5, 7].
Subtentorial hemispheric tumors are predominantly composed of neoplasms of the
ependymal series.
Primary brain tumors, including ependymomas, represent a heterogeneous group of
pathologies and are recognized as the most prevalent solid tumors in pediatric populations.
Diagnostic and classification methodologies increasingly incorporate immunohistochemical
profiling, cytogenetic and molecular genetic analyses, as well as evaluations of mitotic
activity. While histological assessment remains the cornerstone of brain tumor classification,
additional factors - such as anatomical location, extent of dissemination, molecular
characteristics, and patient age - play pivotal roles in determining therapeutic strategies and
prognostic outcomes. Ependymomas arise from ependymal cells that line the ventricles and
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passageways in the brain and the center of the spinal cord. Ependymal cells produce
cerebrospinal fluid (CSF). These tumors are classified as supratentorial, posterior fossa
(infratentorial), or spinal. In children, 65% to 75% of ependymomas arise in the posterior
fossa around the fourth ventricle.[4] Less commonly, ependymomas present in the
supratentorial compartment. Spinal ependymomas are rare in childhood.
The success of surgical treatment for hemispheric ependymomas depends on tumor
infiltration into subcortical structures, which affects the feasibility of radical resection, as
well as the degree of tumor anaplasia. Total resection of Grade I–II anaplastic ependymomas
yields a 10-year survival rate of 80%. Partial resections are often supplemented by
radiotherapy and chemotherapy to prolong patients’ lives. Tumor
invasion
into
vital
structures significantly limits the possibility of radical removal, which is achieved in only 3–
10% of cases [1, 2, 7]. Despite these efforts, postoperative mortality rates remain high.
The use of combined therapy, including radiotherapy and chemotherapy during the
postoperative period, results in a 5-year survival rate of no more than 30% [6, 10].
In recent years, the term "quality of life" has gained prominence in medical literature
as an integral measure of physical, psychological, emotional, and social functioning,
reflecting treatment outcomes. This concept is especially relevant in oncology, where it
serves as both an effectiveness measure and an important prognostic criterion. Over 30
scales have been developed to assess patient quality of life. Thus, quality of life is a
comprehensive indicator of an individual's perceived position in life [4].
The inconsistent and often unsatisfactory outcomes of treatment for children with
fourth-ventricle brain tumors highlight the continuing importance of addressing this issue.
Objective
To analyze and predict treatment outcomes for fourth-ventricle ependymomas in
pediatric patients.
Materials and Methods
A retrospective analysis of 49 pediatric patients treated at the Republican Specialized
Scientific and Practical Medical Center of Neurosurgery (RSSPMCN) between 2022 and
2024 was conducted. The patients were aged 2 to 15 years, comprising 26 boys and 23 girls.
Diagnosis was established based on clinical-neurological, instrumental, and
histopathological investigations. The degree of radicality of tumor removal was assessed
using the program “Tumor Volume Calculation.”
Results and Discussion
In terms of anaplasia, 61.8% of ependymal tumors were classified as benign, while
38.2% were classified as malignant. Tumor location in functionally critical brain zones and
spread into subcortical structures limited the possibility of radical removal, which was
achieved in 70.8% of patients. Postoperative mortality overall was 4.6%, with 3.1% for total
resections and 5.0% for subtotal resections.
Five-year survival rates varied based on the degree of tumor removal and anaplasia
grade. Total resection of Grade I–II anaplastic gliomas resulted in a five-year survival rate of
98.7%. For Grade III tumors treated with radiotherapy (RT) and chemotherapy (CT), the
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survival rate was 64.5%. Subtotal tumor removal yielded five-year survival rates of 94.7%
and 39.0% for benign and malignant tumors, respectively.
Quality of life assessments revealed that 41.0% of children with fourth-ventricle
ependymomas had a good quality of life, 54.2% had a satisfactory quality of life, and 4.8%
had a poor quality of life, often due to pronounced hemiparesis. It was found that neither
tumor anaplasia grade nor the type of adjuvant therapy had a statistically significant effect
on quality of life. However, quality of life deterioration correlated with tumor spread into
subcortical structures and, consequently, with the extent of surgical intervention.
To evaluate the relationship between quality of life in the postoperative period and
the radicality of tumor removal, patients were divided into three groups based on the totality
of resection. Quality of life was assessed in the immediate and long-term postoperative
periods using a scale for evaluating the quality of life in patients with supratentorial
neoplasms. Results were categorized into excellent, satisfactory, and unsatisfactory quality
of life. The comparison was based on the baseline quality of life in the early postoperative
period.
Table 1.
Dynamic relationship between resection totality and quality of life in immediate and
long-term postoperative periods
Quality of life
Extent of resection
TOTAL
up to 75 %
partial
75-95%
subtotal
95-100%
total
N
%
n.
%
n.
%
n.
%
G
oo
d
Immediate
postoperative
period
3
27,3
8
47,1
8
38,1
19
38,8
Long-term
postoperative
period
3
30
6
37,5
12
52,2
21
42,8
Sa
tis
fa
cto
ry
Immediate
postoperative
period
5
45,4
7
41,1
11
52,4
23
46,9
Long-term
postoperative
period
3
30
7
43,75 9
39,1
19
38,8
Po
or
Immediate
postoperative
period
3
27,3
2
11,8
2
9,5
7
14,3
Long-term
postoperative
period
4
40
3
18,75 2
8,7
9
18,4
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From Table 1, it is evident that the best neurological outcomes were observed in patients
with subtotal resections during the immediate postoperative period, a trend that persisted in
the long-term period for 37.5% of patients. Poorer outcomes in patients with partial
resections were associated with their initially poor condition, as these surgeries were
generally emergent and life-saving.
In our study, total resection had unfavorable outcomes in 9.7% of cases in the
immediate postoperative period. However, in the long term, it was associated with improved
quality of life and a stable favorable outcome in 53.6% of patients.
Conclusion
1.
The effectiveness of treatment for pediatric patients with fourth-ventricle
ependymomas largely depends on the degree of tumor resection and adjuvant therapy.
2.
The program “Tumor Volume Calculation” was successfully utilized to accurately
determine the radicality of surgical intervention.
3.
Long-term outcomes in the group with total resections were superior to those in the
subtotal resection group due to the complete elimination of the pathological factor.
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