Immunohistochemistry of the spindle cells will reveal S-100-positivity, which confirms the tumor’s neural lineage.2 The presence of ganglion cells can be confirmed by staining for neuron-specific enolase and neurofilament; this will allow differentiation from other neurogenic spindle cell lesions such as neurofibromas.2 This evidence concerns the gene ENO2 and plexiform neurofibroma.