Solitary neurofibroma of the orbit with intracerebral extension associated with ocular surface melanocytoma: a case report

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

KEYWORDS:

• Melanocytoma
• neurofibroma
• neurofibromatosis type 1
• orbital neoplasm

Authors’ contributions

MJT and HG conceived the idea for the case study and together with RM and MFJ followed the patient. FAA, AK and RM performed the histological examination of the tumor. HG, FAA and MJT wrote the article and EAK, AK and MFJ revised it critically for important intellectual content. All authors read and approved the final manuscript.

Consent for publication

Written informed consents were obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the editors of this journal.

Disclosure statement

The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the article.

Ethics approval and consent to participate

The study was approved by ethics committee of Farabi Eye Hospital, Tehran University of Medical Sciences.