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Research Letter
June 2011

Complete, Pupil-Sparing Third Nerve Palsy in a Patient With a Malignant Peripheral Nerve Sheath Tumor

Author Affiliations

Author Affiliations: Department of Neuro-ophthalmology, Wilmer Eye Institute (Drs Fard and Miller) and Department of Pathology (Dr Montgomery), The Johns Hopkins University School of Medicine, Baltimore, Maryland.

Arch Ophthalmol. 2011;129(6):805-820. doi:10.1001/archophthalmol.2011.122

Pupil-sparing but otherwise complete oculomotor nerve pareses (OMNPs) usually are caused by ischemia, whereas pupil-involved OMNPs or incomplete, pupil-sparing OMNPs usually require an evaluation for a compressive or infiltrative process. There are, however, exceptions. For example, Lustbader and Miller1 reported a case of a complete, pupil-sparing OMNP caused by a giant basilar tip aneurysm. Herein, we report a case of a complete pupil-sparing OMNP caused by a malignant peripheral nerve sheath tumor (MPNST) that arose in the sphenoid sinus.

A 72-year-old man with controlled hypertension had a 5-week history of left ptosis and diplopia. The patient had normal visual acuity, color vision, and visual fields in each eye. The pupils were isocoric and equally and normally reactive to light and near stimulation. The right upper eyelid was in normal position and the right eye moved fully in all directions. The left upper eyelid was completely ptotic. The left eye had no elevation, depression, or adduction, but there was normal abduction and intorsion on attempted downgaze in abduction. The rest of the examination revealed no abnormalities. Magnetic resonance imaging revealed a hyperintense, heterogeneous, multilobulated mass centered in the sphenoid sinus on T2-weighted and fluid-attenuated inversion recovery images (Figure 1A), with heterogeneous enhancement and central nonenhancing parts with evidence of bilateral cavernous sinus invasion on contrast-enhanced T1-weighted images (Figure 1B). The patient underwent a gross total resection of the lesion, which was diagnosed as an intermediate-grade MPNST with the neoplastic cells staining strongly and diffusely for S-100 protein (Figure 2). No mutations were detected in the 4 KIT exons analyzed; ultrastructural analysis revealed no melanosomes.