RESUMO
Chordomas are malignant tumors derived from remnants of the notochord. These are extremely rare in pediatric patients, accounting for approximately 5% of all chordomas, with most lesions occupying the cranium. Chordomas also can occupy all levels of the spine, demonstrating a broad spectrum of neurologic presentation. Optimal treatment aims for gross total resection with accompanying radiation therapy to prevent recurrence. Their aggressive and infiltrative nature makes clinical management challenging, involving multiple disciplines and close monitoring to ensure optimal outcomes. This comprehensive review aims to cover the genetics, demographics, pathogenesis, neurologic sequelae, radiological considerations, chemotherapeutic management, surgical management, and post-operative considerations of pediatric chordoma patients.