Purpose: Eye globe enucleation due to other than a malignant tumor is very rare today. Solitary intraocular neurofibroma without other signs of neurofibromatosis is a rare benign tumor and few cases have been reported to date.
Material and methods: In 10 year interval from Jan 1 2007 to Dec 31 2016 we analyzed non-malignant eye globe enucleations.
Results: Of the 49 enucleated blind eyes, each patient had visual acuity with no light perception, 34 (69.4%) were indicated for enucleation due to complications following previous postoperative surgery after trauma, 14 patients (28.6%) were due to secondary glaucoma and other complications following previous intraocular surgery, and in one patient (2%) the primary isolated intraocular neurofibroma was verified after enucleation.
Case report: A patient with isolated intrabulbar neurofibroma has been monitored since childhood for intraocular lesion and histologically verified at adult age. At the time of enucleation, he was 25 years old, squint since childhood and was observed for hamartoma in his right eye since he was 13 years old. Due to the progression of intrabulbar lesion, loss of visual acuity (functional state - no light perception) and secondary glaucoma, the right eye globe was enucleated at adult age and histopathological examination confirmed intraocular neurofibroma in the absence of neurofibromatosis.
Conclusion: Every enucleated eye globe should be subjected to a thorough histopathological examination. Isolated intraocular neurofibromas can occur as isolated orbital or intrabulbar masses without systemic features.
Keywords: enucleation; eye globe; neurofibromatosis; solitary intrabulbar neurofibroma.