Secondary Enucleation in Retinoblastoma: Clinical features predictive of high-risk histopathology Features in 295 Eyes.

Abstract

To report the incidence of high-risk histopathology features (HRHF) in secondarily enucleated eyes of retinoblastoma (RB) and to identify its clinical predictors. Retrospective study of 295 secondarily enucleated eyes of 289 RB patients. 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). 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.