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Pediatric spinal pilomyxoid astrocytoma
Sarah T Garber1, Robert J Bollo, Jay K Riva-Cambrin
1Department of Neurosurgery, Clinical Neurosciences Center, University of Utah, Salt Lake City, Utah.
Journal of Neurosurgery. Pediatrics
|September 24, 2013
Summary
Pediatric spinal pilomyxoid astrocytoma (PMA) is a rare spinal tumor. Early recognition and aggressive treatment, including surgery and chemotherapy, may improve outcomes for affected children.
Area of Science:
- Pediatric Oncology
- Neuro-oncology
- Spinal Cord Tumors
Background:
- Pilomyxoid astrocytoma (PMA) is an extremely rare pediatric spinal cord tumor.
- Recognition as a distinct entity is crucial for understanding and treatment.
Observation:
- An 11-year-old boy presented with an intramedullary thoracic spinal PMA.
- Symptoms included back pain, scoliosis, and lung nodules.
- Near-total resection was performed, with final pathology WHO Grade II PMA.
Findings:
- The patient experienced tumor progression after 14 months.
- Literature review suggests gross-total resection and alkylating agent chemotherapy improve prognosis.
- Long-term recurrence-free survival is possible, but dissemination and dedifferentiation can occur.
Implications:
- Intramedullary PMA in children requires specific diagnostic and therapeutic strategies.
- Developing optimal treatment guidelines is essential due to the rarity of these tumors.
- Further research is needed to establish definitive treatment protocols for pediatric spinal PMAs.
