SECONDARY ENUCLEATION IN RETINOBLASTOMA

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PURPOSE: To report the incidence of high-risk histopathology features (HRHF) in secondarily enucleated eyes of retinoblastoma (RB) and to identify its clinical predictors. METHODS: Retrospective study of 295 secondarily enucleated eyes of 289 RB patients. RESULTS: The mean age at presentation of RB was 23 months (median, 18 months, range, <1 to 144 months). Indications for secondary enucleation included persistent/recurrent/progressive active tumor (n=246, 83%) or loss of fundus view (n=49, 17%). HRHFs were noted in 49 (17%) eyes including iris infiltration (n=12, 4%), ciliary body infiltration (n=12, 4%), massive choroidal invasion (n=22, 7%), postlaminar (n=3, 1%), optic nerve transected margin involvement (n=2, <1%), combination of optic nerve and choroidal involvement (n=4, 1%), and scleral infiltration (n=9, 3%). After a mean follow-up of 35 months (median, 26 months; range, 1 to 161 months) from secondary enucleation, systemic metastasis and death occurred in 2 (<1%) patients. The predictors of HRHF in cases of secondary enucleation included age >36 months at the time of enucleation (p<0.001), an interval of >12 months from first treatment to enucleation (p<0.001), an interval of >1 month from last treatment to enucleation (p<0.001), and persistent/recurrent/progressive tumor despite >9 cycles of systemic chemotherapy before enucleation (p<0.001). CONCLUSION: While aiming for global salvage in the management of retinoblastoma, caution should be exercised for patients >36 months of age, with a treatment duration of >12 months and a requirement of >9 cycles of systemic chemotherapy. When secondary enucleation is planned, care providers should be warned against deferring it beyond 1 month.

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