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Review
. 2021 Dec;121(6):1407-1414.
doi: 10.1007/s13760-021-01821-z. Epub 2021 Oct 14.

Clinical management of pediatric chordomas: a comprehensive review

Taylor Reardon  1 , Caleb Marsh  2 , Preston Rippe  2 , Donatas Ruzys  2 , Benjamin Ayres  2 , David Cline  2 , Brian Fiani  3
Affiliations
Review

Clinical management of pediatric chordomas: a comprehensive review

Taylor Reardon et al. Acta Neurol Belg. 2021 Dec.

Abstract

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.

Keywords: Chordoma; Neurosurgery; Pediatric chordoma; Pediatric neurosurgery.

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