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

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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 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.

Discussion

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.

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Conflict of interest

The authors declare that they have no conflict of interest.

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Authors and Affiliations

  1. Department of Pediatric Neurosurgery, Dana-Dwek Children’s Hospital, Tel Aviv Sourasky Medical Center, Tel Aviv University, 6 Weizman Street, Tel Aviv, Israel, 64239

    E. Winograd, N. Pencovich, L. Beni-Adani & S. Constantini

  2. Department of Pathology, Tel Aviv Sourasky Medical Center, Tel Aviv University, Tel Aviv, Israel

    D. Soffer

  3. Department of Pediatric Hematology/Oncology, Sheba Medical Center, Tel-Hashomer, Israel

    M. Yalon

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  1. E. Winograd
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  2. N. Pencovich
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  5. L. Beni-Adani
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  6. S. Constantini
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Correspondence to S. Constantini.

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Winograd, E., Pencovich, N., Yalon, M. et al. Malignant transformation in pediatric spinal intramedullary tumors: case-based update. Childs Nerv Syst 28, 1679–1686 (2012). https://doi.org/10.1007/s00381-012-1851-4

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  • DOI: https://doi.org/10.1007/s00381-012-1851-4

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