title: Malignant transformation in pediatric spinal intramedullary tumors: case-based update.
abstract: BACKGROUND
  In children, intramedullary spinal cord neoplasms are rare. These are typically low-grade neuroepithelial tumors, most commonly astrocytomas, ependymomas, and gangliogliomas. Malignant transformation, while common in recurrent adult low-grade gliomas, is an unusual event in pediatric low-grade neoplasms, specifically in intramedullary spinal cord tumors.
  
  
  METHODS
  We report two cases of malignant transformation in low-grade neuroepithelial tumors of the pediatric intramedullary spinal cord. Two children with intramedullary tumors, one with a WHO grade I ganglioglioma and one with a low-grade astrocytoma, were treated surgically, diagnosed histologically, and followed through the course of their disease. Both patients' tumors transformed to higher grades without prior irradiation or chemotherapy, and without a genetic predisposition to tumorigenesis.
  
  
  CONCLUSIONS
  Malignant transformation can occur in low-grade intramedullary neoplasms in children. This is a novel documented event for pediatric intramedullary spinal cord tumors and a rare event for all pediatric low-grade neuroepithelial tumors without induction by irradiation. A survey of the relevant literature reveals an underwhelming number of studies focusing on malignant transformation in children's CNS tumors relative to adults. Further investigation into molecular mechanisms of pediatric low-grade neoplasms may reveal more aggressive tumor sub-variants predisposed to malignant degeneration.
authors: Department of Pediatric Neurosurgery, Dana-Dwek Children's Hospital, Tel Aviv Sourasky Medical Center, Tel Aviv University, 6 Weizman Street, Tel Aviv, Israel, 64239.
authors: E
authors: E
authors: Winograd
authors: N
authors: N
authors: Pencovich
authors: M
authors: M
authors: Yalon
authors: D
authors: D
authors: Soffer
authors: L
authors: L
authors: Beni-Adani
authors: S
authors: S
authors: Constantini
mesh_terms: D000293:Adolescent; D001254:Astrocytoma / Q000473:pathology* / Q000503:physiopathology* / Q000601:surgery; D002675:Child, Preschool; D018450:Disease Progression; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D013120:Spinal Cord Neoplasms / Q000473:pathology* / Q000503:physiopathology* / Q000601:surgery