Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2016:2016:4294729.
doi: 10.1155/2016/4294729. Epub 2016 Sep 28.

Unusual Case of a Proptosed Eye: Isolated Right Maxillary Neurofibroma

Darren Yap  1 , Hannah Fox  1 , Julia Addams-Williams  1
Affiliations

Unusual Case of a Proptosed Eye: Isolated Right Maxillary Neurofibroma

Darren Yap et al. Case Rep Pathol. 2016.

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.

PubMed Disclaimer

Figures

Figure 1
Figure 1
Axial MRI showing lobulated mass in the right maxillary antrum extending anteriorly into the subcutaneous tissue of the cheek and posteriorly into the infratemporal fossa.
Figure 2
Figure 2
Postgadolinium MRI coronal sequence showing superior extension into the right orbit.
See this image and copyright information in PMC

References

    1. Prakash M. D., Viswanatha B., D'Souza G. E. A rare case of isolated nasal neurofibroma mimicking sinonasal polyp. Research in Otolaryngology. 2014;3(3):49–51.
    1. Solomon M. C., Shergill A. K., Pai K. M., Chandrashekar C., Mutalik V. S., Carnelio S. Intra-osseous neurofibroma in the maxilla of a young girl—a report of a rare case. Innovative Journal of Medical and Health Science. 2013;3(5):235–237.
    1. Komorski J., Petz Ł., Nienartowicz J., Pałka Ł. Neurofibroma of sinus maxillae. Otolaryngologia Polska. 2014;68(2):94–98. doi: 10.1016/j.otpol.2012.07.001. - DOI - PubMed
    1. Boedeker C. C., Ridder G. J., Kayser G., Schipper J., Maier W. Solitary neurofibroma of the maxillary sinus and pterygopalatine fossa. Otolaryngology—Head and Neck Surgery. 2005;133(3):458–459. doi: 10.1016/j.otohns.2005.01.006. - DOI - PubMed
    1. Hachem R. N. A., Bared A., Zeitouni J., Younis R. T. Single-stage total endoscopic resection of a plexiform neurofibroma of the maxillary sinus in a child with type 1 neurofibromatosis. International Journal of Pediatric Otorhinolaryngology. 2010;74(4):426–429. doi: 10.1016/j.ijporl.2009.12.012. - DOI - PubMed

LinkOut - more resources