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.

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