Epithelioid malignant peripheral nerve sheath tumor in the infraorbital nerve

Epithelioid malignant peripheral nerve sheath tumors are rarely found in cranial nerves. This report describes one such tumor originating from the infraorbital nerve in a 55-year-old man who presented with diplopia and right cheek numbness. Imaging showed a mass along the floor of the right orbit, which was excised. Histology suggested melanoma or carcinoma. Immunohistochemical staining was positive for S-100 and negative for HMB-45 and cytokeratin. Four other cases from the literature involving cranial nerves are discussed.