Journal
BRAIN TUMOR PATHOLOGY
Volume 37, Issue 3, Pages 100-104Publisher
SPRINGER JAPAN KK
DOI: 10.1007/s10014-020-00370-2
Keywords
CNS neuroblastoma; FOXR2 activation; Pediatric brain tumor
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A subset of central nervous system neuroblastomas (CNS NB), rare primary embryonal CNS tumors, has been encompassed in CNS NB withFOXR2activation (CNS NB-FOXR2) and usually shows the primitive neuronal architecture and occasional neurocytic differentiation. Here, we report a rare case of 3-year-old female with uncommon morphology of CNS embryonal tumor withFOXR2activation presenting bidirectional differentiation to neurocytic small primitive cells and astrocytic spindle cells both of which are positive for synaptophysin and GFAP. Ultrastructural study also showed that there were presynaptic structure and intermediate filament in the tumor cells, suggesting glioneuronal differentiation. This case indicates the possibility of CNS neuroblastic tumor to differentiate neuronal and glial lineages.
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