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Infratentorial ependymomas in childhood: prognostic factors and treatment
G B Nazar1, H J Hoffman, L E Becker
1Division of Neurosurgery, Hospital for Sick Children, Toronto, Ontario, Canada.
Insights
Childhood infratentorial ependymomas are rare brain tumors. Factors like complete tumor removal and specific histology improve survival, while younger age and tumor invasion are associated with poorer outcomes.
Area of Science:
- Pediatric Oncology
- Neurosurgery
- Pathology
Background:
- Childhood infratentorial ependymomas are a significant cause of pediatric brain tumors.
- Understanding prognostic factors is crucial for optimizing treatment strategies and improving patient outcomes.
Purpose of the Study:
- To analyze prognostic factors and survival data for childhood infratentorial ependymomas.
- To identify factors associated with improved or diminished 5-year survival rates.
Main Methods:
- Retrospective analysis of 35 children diagnosed with infratentorial ependymomas between 1970 and 1987.
- Tumor histology reviewed and categorized (I-III) for survival analysis.
- Evaluation of clinical factors including age, tumor characteristics, and treatment modalities.
Main Results:
- Overall 5-year survival rate was 44.6% post-perioperative mortality exclusion.
- Improved survival linked to total tumor removal, noninvasive tumors, Category I histology, age >6 years, and absence of neurological deficits.
- Poorer survival associated with Category III histology, brain-stem/cranial nerve signs, age <2 years, tumor invasion, and subtotal resection.
Conclusions:
- Prognostic factors significantly influence survival in childhood infratentorial ependymomas.
- Surgical excision followed by radiation therapy remains the primary treatment approach.
- Further research into radiotherapy volume and adjuvant chemotherapy is warranted.
Abstract:
The prognostic factors and survival data were analyzed for 35 children (aged under 16 years at diagnosis) with childhood infatentorial ependymomas treated surgically at The Hospital for Sick Children in Toronto during the years 1970 to 1987. Tumor histology was reviewed individually and grouped into three categories (Categories I to III) for survival analysis. An overall 5-year survival rate of 44.6% was obtained after the exclusion of perioperative mortality. Factors associated with an improved 5-year survival rate were: total tumor removal, noninvasive tumors, Category I histology, age greater than 6 years, and absent physical signs of parenchymal invasion or lower cranial nerve involvement. The 5-year survival rate was lower when associated with Category III histology, brain-stem or cranial nerve signs, age less than 2 years, tumor invasion and/or cranial nerve involvement, and subtotal tumor removal. Clinical evidence of spinal metastases was found to be uncommon (3.1%). Surgical excision followed by radiation therapy was the primary mode of treatment for these tumors. Different approaches regarding the volume of radiotherapy to be delivered and the use of adjuvant chemotherapy are discussed.