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Prognostic factors in infants and very young children with intracranial ependymomas
P K Duffner1, J P Krischer, R A Sanford
1Department of Neurology, SUNY at Buffalo, School of Medicine and Biomedical Science, Buffalo, N.Y., USA.
Insights
Younger children (<2 years) with intracranial ependymomas had lower survival rates after prolonged chemotherapy and delayed radiation. Delayed radiation in older children (2-3 years) improved outcomes, suggesting maximal resection and shorter radiation delays are key for pediatric brain tumors.
Area of Science:
- Pediatric Oncology
- Neuro-oncology
- Radiation Oncology
Background:
- Intracranial ependymomas are primary brain tumors in children.
- Treatment often involves surgery, chemotherapy (CT), and radiation therapy (RT).
- Age at diagnosis is a critical factor in treatment response and survival.
Purpose of the Study:
- To evaluate the impact of prolonged postoperative chemotherapy and delayed radiation on survival in young children with intracranial ependymomas.
- To determine the influence of age at diagnosis on treatment outcomes.
- To identify prognostic factors for improved survival in this pediatric population.
Main Methods:
- A cohort of 48 children under 3 years with intracranial ependymomas was studied (1986-1990).
- Patients were divided into two groups based on age: 0-23 months (Gp A) and 24-36 months (Gp B).
- Gp A received 2 years of CT followed by RT; Gp B received 1 year of CT followed by RT. Survival rates were analyzed based on age and surgical resection extent.
Main Results:
- One-year and two-year survival rates were high in both groups (Gp A: 87% and 67%; Gp B: 94% and 82%).
- A significant divergence in survival was noted in subsequent years, with 5-year survival rates of 25.7% for Gp A versus 63.3% for Gp B (p=0.04).
- Maximal surgical resection was a significant positive prognostic factor (5-year survival 66% vs. 25% for subtotal resection). Age at diagnosis was the only other significant factor.
Conclusions:
- Delayed radiation therapy (RT) following chemotherapy (CT) significantly impacts survival in young children with intracranial ependymomas, with shorter delays being more beneficial.
- Maximal surgical resection is crucial for improving outcomes.
- Future pediatric brain tumor trials should prioritize maximal surgical resection and limit radiation delays to one year.
Abstract:
The Pediatric Oncology Group (1986-1990) conducted a study in which 48 children <3 years of age with intracranial ependymomas were treated with prolonged postoperative chemotherapy (CT) and delayed RT. Thirty-one children, 0-23 months of age at diagnosis (Gp A) received 2 years of CT followed by RT; while 17 children, 24-36 months of age at diagnosis (Gp B) received CT for 1 year followed by radiation. One-year survivals were 87% (Gp A) and 94% (Gp B) and 2-year survivals were 67% (Gp A) and 82% (Gp B). In subsequent years a significant divergence in survivals according to age has been noted (p = 0.04). Five-year survivals were 25.7% (Gp A) vs. 63.3% (Gp B). The curves began to diverge 1 year following diagnosis. Other than age, the only significant prognostic factor was degree of surgical resection: 5-year survivals were 66% (total resection) vs. 25% (subtotal resection). Neither the presence of metastases, degree of anaplasia nor the degree of surgical resection varied significantly according to age at diagnosis. The most likely reason for the difference in survivals between the two age groups relates to the timing of radiation following CT, i.e., 1-year delay in children 24-36 months of age compared to a 2-year delay in children 0-23 months of age. An alternative but less likely hypothesis is that ependymomas in the younger children have a more aggressive biology. In contrast, survivals in the 24- to 36-month group are much better than previous reports in the literature suggesting that prolonged postoperative CT may allow both a delay in CRT as well as provide improved survivals. Based on these results, future treatment trials should emphasize maximal surgical resection and a delay in radiation of no more than 1 year.