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Outcome of children with primary intramedullary spinal cord tumors
Insights
Pediatric primary intramedullary spinal cord tumors (PST) have poor outcomes, with low survival rates. Further research into novel treatment strategies is crucial for improving pediatric PST patient survival.
Area of Science:
- Pediatric Oncology
- Neuro-oncology
- Clinical Outcomes Research
Background:
- Primary intramedullary spinal cord tumors (PST) in children are rare and present significant treatment challenges.
- Outcomes for pediatric PST patients historically show limited long-term event-free survival.
Purpose of the Study:
- To evaluate the impact of clinical and treatment factors on the outcomes of children diagnosed with primary intramedullary spinal cord tumors (PST).
- To identify prognostic indicators and areas for therapeutic improvement in pediatric PST management.
Main Methods:
- Retrospective review of medical records for 26 children diagnosed with PST between 1970 and 1984.
- Analysis of survival and event-free survival (EFS) rates based on tumor type, location, symptoms, and surgical intervention.
Main Results:
- Overall 5-year survival was 39%, with a 5-year event-free survival (EFS) of only 14%.
- Eighteen-month EFS varied significantly by histology: 53% for low-grade astrocytoma, 100% for ependymoma, and 0% for anaplastic astrocytoma/ganglioglioma.
- Tumor location, symptom duration, and extent of surgical removal did not significantly impact 18-month EFS.
- Five of 9 children with recurrent PST who underwent a second surgery survived long-term.
- PST dissemination occurred in 5 children, with some instances at diagnosis or as initial relapse signs.
Conclusions:
- Pediatric primary intramedullary spinal cord tumors (PST) are associated with poor prognoses.
- Histological type is a critical determinant of outcome, while other factors showed less significant impact in this cohort.
- The study highlights the need for evaluating alternative treatment modalities for pediatric PST given the observed outcomes.
Abstract:
The influence of clinical and treatment factors on the outcome of children with primary intramedullary spinal cord tumors (PST) was evaluated by reviewing the records of 26 children diagnosed during the 15-year period 1970-1984. Five-year survival was 39%, but 5-year event-free survival (EFS) was only 14%. Eighteen-month EFS was 53% (9/17) among children with low-grade astrocytoma. 100% (2/2) with ependymoma, and 0 of 7 with anaplastic astrocytoma or ganglioglioma. There was no significant difference in the 18-month EFS by location of tumor, duration of symptoms, or extent of surgical removal. Five of 9 children with locally recurrent PST had a second operation, and 4 were alive a median of 56 months later. PST disseminated to the leptomeninges or the III ventricle in 5 children: 2 at diagnosis, 2 as the first sign of disease relapse, and 1 after local recurrence. Given the poor outcome of our children, different methods of treatment for children with tumors in this location should be evaluated.