Primary malignant peripheral nerve sheath tumor at unusual location
A 35-year-old woman presented with 2-month history of rapidly progressive painless swelling in left orbitotemporal region with proptosis and blurring of vision leading to complete blindness. Physical examination revealed a lobular nontender, firm mass of size 15 × 7 cm extending from left orbit to the left temporal region. In addition to axial proptosis, the left eye showed restricted movement in all directions [Figure and ]. She was unable to perceive light in her left eye. Magnetic resonance imaging (MRI) of the orbits and brain showed left sphenoidal-based extra-axial marginated in homoginously enhancing mass at the lateral side of the left optic nerve buckling the ipsilateral anterotemporal lobe []. Other systemic observations of the patient were normal. Fine needle aspiration cytology came to be neurofibroma. Near total dissection of the tumor was done through a left lateral orbitozygomatic approach. The histopathology and immunohistochemistry of the lesion revealed MPNST [Figure and ]. Postoperatively, the patient recovered rapidly with improved cosmoses []. Vision in her left eye improved to finger counting at 1 m and extraocular movements were normal. She received local radiotherapy. On 6-month follow-up, the patient is doing well with no local recurrence or any distant metastasis.