Orbital schwannomatosis in the absence of neurofibromatosis.

Abstract

PURPOSE:

The aim of this study was to describe 3 cases of primary orbital schwannomatosis without associated systemic neurofibromatosis.

METHODS:

This is a retrospective interventional study of 3 patients who presented with multiple, distinct masses in the orbit (n = 3) as well as in the hemiface (n = 1). The clinical presentation, imaging features, surgical procedures, and outcomes were defined.

RESULTS:

Two women and a man presented with of exophthalmos and diplopia. Pain was the most prominent complaint in 2 patients. None of the patients had associated systemic neurofibromatosis by history or examination. Radiologic evaluation with computed tomography or magnetic resonance imaging of orbit revealed multiple well-demarcated intraconal and extraconal masses. Masses were excised, and histopathology confirmed all masses to be schwannomas. Postoperative follow-up was uneventful with alleviation of primary complaints in all patients.

CONCLUSIONS:

Multiple orbital schwannomas (primary orbital schwannomatosis) may be observed in patients without systemic association of neurofibromatosis. Management includes surgical excision of the tumors to achieve relief from their mass effects.

• Authors: Bengu Ekinci Koktekir, H Jane Kim, Mike Geske, Michelle Bloomer, Reza Vagefi, Robert C Kersten
• Journal: The Journal of craniofacial surgery (J Craniofac Surg) Vol. 25 Issue 6 Pg. 2109-11 (Nov 2014) ISSN: 1536-3732 [Electronic] United States
• PMID: 25304146 (Publication Type: Case Reports, Journal Article)

Topics

• Adult
• Diplopia (diagnosis)
• Exophthalmos (diagnosis)
• Facial Neoplasms (diagnosis, surgery)
• Female
• Follow-Up Studies
• Humans
• Magnetic Resonance Imaging
• Male
• Middle Aged
• Molecular Weight
• Neoplasm Recurrence, Local (pathology)
• Neoplasms, Multiple Primary (diagnosis, surgery)
• Neurilemmoma (diagnosis, surgery)
• Neurofibromatoses (diagnosis, surgery)
• Orbital Neoplasms (diagnosis, surgery)
• Retrospective Studies
• Skin Neoplasms (diagnosis, surgery)
• Tomography, X-Ray Computed
• Treatment Outcome