Malignant transformation in pediatric spinal intramedullary tumors: Case-based update

E. Winograd, N. Pencovich, M. Yalon, D. Soffer, L. Beni-Adani, S. Constantini

Research output: Contribution to journalArticlepeer-review

14 Scopus citations

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. Illustrative cases 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 aWHO 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. Discussion Malignant transformation can occur in lowgrade 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.

Original languageEnglish
Pages (from-to)1679-1686
Number of pages8
JournalChild's Nervous System
Volume28
Issue number10
DOIs
StatePublished - 1 Oct 2012
Externally publishedYes

Keywords

  • Intramedullary
  • Low-grade gliomas
  • Malignant transformation
  • Pediatric
  • Spinal cord

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