Abstract
Purpose :
Advanced intraocular retinoblastoma can be cured by primary enucleation, but delay may increase risk of tumor metastasis. We hypothesized that delay in enucleation over a defined length of time would increase metastatic death.
Methods :
Ethics Boards of 29 Chinese Centers, in accordance with the Declaration of Helsinki, approved the retrospective study of 600 children enucleated 2006–2010 for advanced retinoblastoma. Excluded were 46 children who died without metastasis or had bilateral advanced disease.
Results :
Studied were 554 enucleated eyes/children (202 Group D International Intraocular Retinoblastoma Classification; 352 Group E). Compared to primary enucleation (275 eyes, Group D/E: <0.1 months median time from diagnosis to enucleation), pre-enucleation treatment delayed enucleation (279 eyes, Groups D: 8.4 months; Group E: 2.8 months, p<0.001). Eyes that received pre-enucleation chemotherapy had less high-risk pathology (pT3/pT4) than primarily enucleated eyes (Group D, p=0.04; Group E, p=0.003).
Of 554 children, 22 died from metastasis (6/22 Group D, 16/22 Group E): 14/22 had pre-enucleation chemotherapy, 11/22 had post-enucleation chemotherapy and 5/22 had both. Pathology stages were 4 pT1, 1 pT2b, 2 pT3a, 4 pT3b, 2 pT3c and 9 pT4.
Disease-specific survival (DSS) was lower with prolonged delay between diagnosis and enucleation (Group D, >3.5 vs <3.5 months, p=0.02; Group E, >2 vs <2 months p=0.02).
Secondarily excluded from analysis of the effect of pre-enucleation chemotherapy cycles on DSS were children who received <1 cycle or had other non-chemo pre-enucleation therapy; 510 eyes were included. Too few children with Group D eyes died for analysis. Of children with Group E eyes, those with 1–3 cycles of pre-enucleation chemotherapy had the same DSS as children who had primary enucleation, but those with >4 cycles had lower DSS than children primarily enucleated (p=0.03). Children with high-risk eyes (pT3/pT4) after pre-enucleation chemotherapy had lower DSS than children with high-risk eyes primarily enucleated (p=0.002).
Conclusions :
We show that delay from diagnosis to enucleation >3.5 months (Group D) and >2 months (Group E) increases the risk of death from metastasis.
This abstract was presented at the 2019 ARVO Annual Meeting, held in Vancouver, Canada, April 28 - May 2, 2019.