Malignant peripheral nerve sheath tumor of the left V1 involving supra-orbital and frontal branches.

The patient went on to have surgical excision of the left eye, adjacent skin and soft tissue. Pathology confirmed the tumor type and involvement of the supra-orbital nerve, orbital apex and other extrinsic eye nerves.

The supra-orbital nerve margin was involved by a plexiform neurofibroma, as well the architecture of the tumor was plexiform on histology which is suggestive of neurofibromatosis type 1. Up to 70% of malignant nerve sheath tumors are associated with neurofibromatosis type 1.

 Case submitted by Dr. Smita Deb and A/Prof. Pramit Phal.