CNS embryonal tumour NEC/NOS

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Status
Done

Definition

  • Essential:
    • An embryonal tumour originating in the CNS AND
    • Absence of criteria qualifying for the diagnosis of a more specific type of embryonal CNS tumour
  • Desirable:
    • Focal expression of neuronal markers and absence of glial markers

Microscopy

  • Characterised by poorly differentiated neuroepithelial cells with a variable capacity for divergent differentiation along neuronal, astrocytic, myogenic, or melanocytic lines.
  • Can have histological features overlapping those of other brain tumours, many of which have been reclassified from this group through the identification of unique molecular biomarkers.
  • Poorly differentiated neoplasms composed of cells with round to oval nuclei and a high nuclear-to-cytoplasmic ratio
  • Frequent mitoses and apoptotic bodies
  • Homer Wright rosettes can be found
  • Necrosis and vascular endothelial proliferation may also be seen
  • Calcification is relatively common

Immunophenotype

  • Variable expression of divergent neuroepithelial markers, including proteins associated with both glial differentiation (GFAP) and neuronal differentiation (synaptophysin, NFP, and NeuN)
  • Ki-67 proliferation > 50%
  • Mitoses and apoptotic bodies.