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Solitary Neurofibroma of the Orbit With Intracerebral Extension Associated With Ocular Surface Melanocytoma: A Case Report Publisher Pubmed



Jamshidiantehrani M1 ; Mahmoudzadeh R1 ; Asadi Khameneh E1 ; Asadi Amoli F2 ; Faghih Jouibari M3 ; Kasaee A1 ; Ghadimi H1
Authors
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Authors Affiliations
  1. 1. Eye Research Center, Farabi Eye Hospital, Tehran University of Medical Sciences, Tehran, Iran
  2. 2. Department of Pathology, Farabi Hospital, Tehran University of Medical Sciences, Tehran, Iran
  3. 3. Department of Neurosurgery, Dr Shariati Hospital, Tehran University of Medical Sciences, Tehran, Iran

Source: Orbit (London) Published:2020


Abstract

Solitary or isolated neurofibroma is uncommonly observed in the orbit. Neurofibromas typically involve peripheral nerves and occasionally the cranial nerves. A 29-year-old man presented with recent onset left eye proptosis and exotropia. Physical examination was positive for hyperpigmented lesions of the ipsilateral ocular surface and hard palate. Imaging revealed an infiltrative orbital mass with extension through superior orbital fissure into the brain. There was also bone defect of greater sphenoid wing. Medial orbitotomy was performed to obtain biopsies of the orbital mass and the pigmented ocular surface lesions. Histopathologic diagnosis of neurofibroma was confirmed for the former and melanocytoma for the latter. His symptoms and examinations remained stable during the follow up. This case is unique due to several features, including extensive intracerebral spread of orbital neurofibroma in a patient without definite diagnosis of neurofibromatosis type 1 and association with ipsilateral ocular surface melanocytoma and palatal pigmented lesions. Abbreviations: CT: computed tomography; GFAP: glial fibrillary acid protein; MRI: magnetic resonance imaging; NF-1: neurofibromatosis type 1. © 2019 Taylor & Francis Group, LLC.