Background And Importance: The occurrence of multiple tumors of different histopathologies in the central nervous -system is rare. Here, we report a case of a child with dual neuroepithelial tumors of different histological cell types and -review the available literature for analyzing the causal hypo-thesis.

Case Report: A 7-year-old girl presented with history of headaches associated with multiple episodes of early morning projectile vomiting and visual blurring. Magnetic resonance imaging (MRI) showed a well-defined lesion in the hypothalamic region. The patient underwent resection of the tumor by the transcallosal transfornicial approach. Histopathology was suggestive of pilocytic astrocytoma. After 3 years, the patient presented with low back pain radiating to the right lower limb followed by sudden onset of weakness in both lower limbs. Recent MRI brain and spine scans showed a small residual lesion at the hypothalamus with D1 to D10 syrinx. A T1 hypointense and T2 hyperintense cystic lesion from D10 to S2 with intensely enhancing solid component at the S1, S2 region was also detected. The patient underwent L4 to S2 laminectomy and the histopathological examination revealed myxopapillary ependymoma.

Conclusion: The presence of multiple neuroepithelial tumors of different histopathology in the same patient is extremely rare, and we report one such case of dual gliomas with hypothalamic pilocytic astrocytoma and cauda equina myxopapillary ependymoma in a 10-year-old patient. During the course of development, displacement and dedifferentiation of primitive totipotent neuroepithelial germ cells may be a causal factor for the appearance of the different neuroepithelial tumors in neuraxis concomitantly or subsequently, which might be substantiated by common cytogenetic abnormalities in different neuroepithelial tumor histogenesis.

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http://dx.doi.org/10.1159/000346258DOI Listing

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