Abstract
Purpose :
In Retinoblastoma (RB) the crystalline lens is believed to have hypothetic “tumorous immunity” and is usually unaffected and clear. We aim to highlight four cases of RB with lens involvement with a histopathological (HPE) correlation of the same after enucleation. We believe that lens features should be defined in classification systems of RB.
Methods :
We report 4 eyes with lens involvement with RB at presentation in 4 children aged between 2 months to 2 years. 3 out of 4 children had bilateral RB. 3 out 4 eyes and subluxation of clear lens out of which one eye also had tumor infiltrating into the lens. The mechanism of subluxation was hypothesized as secondary to ciliary staphyloma possible due to zonular stretching in one eye and zonular degradation secondary to orbital cellulitis in another, both confirmed on HPE. The subluxation was noted clinically in all 3 eyes and confirmed on imaging and HPE.1 out of 4 case had absent lens, also confirmed on imaging and HPE. 3 out of 4 cases received systemic chemotherapy before enucleation. All 4 eyes were ultimately diagnosed to have advanced RB, (3 out of 4 had group E tumors as per IIRC, and 1 eye had orbital RB) all requiring enucleation ultimately.
Results :
4 of 4 eyes needed Enucleation.
Unexplained anterior segment findings should raise suspicion and mass lesions must be ruled out before any planned interventions. RB presenting with a subluxated or absent lens is scarcely reported and most often the mechanisms are not clearly understood. Lens involvement may be a consequence of the clinical course of the tumor, possibly a surrogate marker suggesting advanced nature or chronicity. Visual rehabilitation is impossible in such eyes with active tumor and eye salvage is seldom possible due to advanced anatomical damage.
Conclusions :
In our experience as these eyes ultimately require enucleation, we propose that lens features should be defined and considered in classification systems of retinoblastoma and lens subluxation and infiltration could be considered a feature of Group E.
This is a 2021 ARVO Annual Meeting abstract.