Malignant peripheral nerve sheath tumor
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- enhancing tumor at medial and superior aspect of the left orbit above the medial culpable fissure (1.7 x 0.8cm).
- extension of tumor along supra-orbital and frontal branches back to the left cavernous sinus demonstrated by nerve enlargement and abnormal enhancement.
- the nerve lies in its position between the periosteum of the orbit and levator palpebrae superioris.
- no extension into: intra-conal space, Meckel's cave or along cisternal portion of 5th nerve.
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.