Orbital peripheral nerve sheath tumors

Abstract

Peripheral nerve sheath tumors of the orbit and ocular adnexa are a rare group of neoplasms hallmarked by nonspecific clinical presentations, variable tumor locations, challenging therapeutic efforts, and occasional diagnostic dilemmas. We review these tumor types and provide an updated summary on their clinical, histopathologic, radiological, and emerging molecular features.

Keywords: histology; magnetic resonance imaging; malignant; neurilemmoma; neurinoma; neurofibroma; neurofibromatosis; pathology; peripheral nerve sheath tumor; schwannoma.