Unusual Case of a Proptosed Eye: Isolated Right Maxillary Neurofibroma.

Darren Yap,Hannah Fox,Julia Addams-Williams

doi:10.1155/2016/4294729

Abstract

Neurofibroma is a slow growing benign tumour of the peripheral nerve sheath which is frequently associated with neurofibromatosis type 1 (Prakash et al., 2014). Isolated solitary occurrence of neurofibroma in the maxillary sinus is rare with only 29 reported cases in the literature. We present a rare case of a 70-year-old gentleman who was referred to ENT with a right maxillary sinus neurofibroma with extension into the right inferior orbit. He has significant proptosis, ptosis, and limitation in abduction of the right eye. He has a complicated past history of multiple neurofibromas which were completely excised. Craniofacial MRI shows a large mass filling the right maxillary antrum extending anteriorly into subcutaneous tissue towards nasal ala and posterolaterally into inferior temporal fossa and superiorly into orbit and cavernous sinus involvement. Biopsy of the right maxillary mass revealed cellular spindle cell tumour with wavy collagen bundles within myxoid stroma which is consistent with a neurofibroma. Patient's case was discussed in the skull-base MDT and he has been referred to a specialist center for surgical removal of the neurofibroma with reconstructive surgery. Despite the rarity of this disease, otorhinolaryngologist should consider a possibility of neurofibroma of the paranasal sinuses.

Highlights

Neurofibroma (NF) is a benign slow growing tumour of the peripheral nerve sheath
We report a case of right maxillary sinus neurofibroma and literature review of this rare condition
He presented with significant proptosis, ptosis, and limitation in abduction of the right eye

Summary

Introduction

Neurofibroma (NF) is a benign slow growing tumour of the peripheral nerve sheath. It may occur as an isolated sporadic lesion or may be associated with neurofibromatosis type 1, which is known as von Recklinghausen’s disease. 1. Introduction Neurofibroma (NF) is a benign slow growing tumour of the peripheral nerve sheath. Neurofibroma of the maxillary sinus is an exceedingly rare tumour. We report a case of right maxillary sinus neurofibroma and literature review of this rare condition.

Results

Conclusion