Derived from a single progenitor cell from the neural crest.
Two distinct morphologic maturation pathways within a single benign tumor.
Excision is usually curative.
Can arise sporadically and occasionally with genetic syndromes such as neurofibromatosis type 1, Beckwith-Wiedemann syndrome, Hirschsprung disease, DiGoerge syndrome (Lonergan GJ).
Typically benign, that has been reported to transform into more aggressive lesions such as malignant peripheral nerve sheath tumors.