Malignant peripheral nerve sheath tumor presenting at the limbal conjunctiva

Abstract

The authors report a 71-year-old white male who underwent enucleation of the right eye for a tumor of the limbal conjunctiva originally thought to be a poorly differentiated squamous cell carcinoma. The diagnosis was questioned five years later when the patient presented with an orbital mass that tested positive for the S-100 protein. Using electron microscopy, the orbital tumor was shown to be a malignant peripheral nerve sheath tumor. The original conjunctival lesion also appears with review to have been a malignant peripheral nerve sheath tumor. This case represents, to the best of the authors' knowledge, the first reported case of a malignant peripheral nerve sheath tumor presenting on the bulbar conjunctiva.

Key Words:

• Orbital-neoplasm
• nerve sheath tumor
• orbital malignancy
• conjunctival neoplasm