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A Unilateral Adult-Onset Retinoblastoma Treated with Ocular Salvage Treatment Modalities without Recurrence over 25 Years Follow-up Period

Nils Andreas Eide, Jesintha Navaratnam

An 18-year-old female presented with 2-year history of intermittent worsening of vision and floaters in her left eye. Complete ophthalmological examination was performed. The Best Corrected Visual Acuity (BCVA) was 1.25 in both eyes. Cells in anterior chamber and vitreous humor were present in the left eye and the posterior segment examination revealed a localized area with dilated retinal vessels and massive white masses in the vitreous humor in the same eye. Examination revealed normal findings in the right eye. A broad investigation disclosed positive Epstein-Barr virus Immunoglobulin M (IgM), borderline high levels of Toxocara canis antigen and normal levels of eosinophils. The clinical findings were not concurrent with a typical uveitis. Therefore, atypical intraocular lymphoma, retinoblastoma and infectious etiologies were suspected. After informed consent and following negative cerebral and orbital magnetic resonance imaging findings, a Fine Needle Aspiration Biopsy (FNAB) from the left vitreous humor masses was performed. The vitreous sample revealed malign small cell tumor with necrosis, hyperchromatic nuclei and high nucleus-to-cytoplasm ratio most likely compatible with retinoblastoma. Ocular salvage treatment modalities with chemotherapy, plaque brachytherapy and external radiation granted BCVA of 0.5 without tumor recurrence over 25 years’ follow-up. The reduction in visual acuity was related to posterior capsular opacification of the lens secondary to radiation.