Unusual Case of a Proptosed Eye: Isolated Right Maxillary Neurofibroma

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 rar...

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Main Authors: Darren Yap, Hannah Fox, Julia Addams-Williams
Format: Article
Language:English
Published: Wiley 2016-01-01
Series:Case Reports in Pathology
Online Access:http://dx.doi.org/10.1155/2016/4294729
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author Darren Yap
Hannah Fox
Julia Addams-Williams
author_facet Darren Yap
Hannah Fox
Julia Addams-Williams
author_sort Darren Yap
collection DOAJ
description 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.
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spelling doaj-art-24fcf7ab15ef4a7bbf0ebf8eb5ddd28a2025-08-20T03:54:52ZengWileyCase Reports in Pathology2090-67812090-679X2016-01-01201610.1155/2016/42947294294729Unusual Case of a Proptosed Eye: Isolated Right Maxillary NeurofibromaDarren Yap0Hannah Fox1Julia Addams-Williams2Department of ENT, Royal Gwent Hospital, Cardiff Road, Newport NP20 2UB, UKDepartment of ENT, Royal Gwent Hospital, Cardiff Road, Newport NP20 2UB, UKDepartment of ENT, Royal Gwent Hospital, Cardiff Road, Newport NP20 2UB, UKNeurofibroma 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.http://dx.doi.org/10.1155/2016/4294729
spellingShingle Darren Yap
Hannah Fox
Julia Addams-Williams
Unusual Case of a Proptosed Eye: Isolated Right Maxillary Neurofibroma
Case Reports in Pathology
title Unusual Case of a Proptosed Eye: Isolated Right Maxillary Neurofibroma
title_full Unusual Case of a Proptosed Eye: Isolated Right Maxillary Neurofibroma
title_fullStr Unusual Case of a Proptosed Eye: Isolated Right Maxillary Neurofibroma
title_full_unstemmed Unusual Case of a Proptosed Eye: Isolated Right Maxillary Neurofibroma
title_short Unusual Case of a Proptosed Eye: Isolated Right Maxillary Neurofibroma
title_sort unusual case of a proptosed eye isolated right maxillary neurofibroma
url http://dx.doi.org/10.1155/2016/4294729
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