SECONDARY SALVAGE INTRAVENOUS CHEMOTHERAPY FOR REFRACTORY/RECURRENT RETINOBLASTOMA: A Study of 41 Eyes.

PURPOSE: To determine the efficacy of secondary salvage intravenous chemotherapy (IVC) for refractory/recurrent retinoblastoma. METHODS: Retrospective, nonrandomized interventional case series of 41 eyes of 33 patients with recurrent retinoblastoma. RESULTS: Of the 33 patients, mean age at the time of commencement of salvage IVC was 5 years (median, 5 years; range, 2-8 years). At presentation, recurrent retinoblastoma in 41 eyes of 33 patients was classified by the International Classification of Retinoblastoma as Group B (n = 7; 17%), Group C (n = 3; 7%), Group D (n = 16; 39%), and Group E (n = 15; 37%). All patients received 6 cycles of IVC as primary treatment. The indication for secondary salvage IVC with focal treatment included recurrent solid tumor (n = 36; 88%), subretinal seeds (n = 22; 54%), or persistent solid tumor (n = 2; 5%). Mean number of cycles of salvage IVC were 8 (median, 6; range, 6-18). Over a mean follow-up period of 43 months (median, 43 months; range, 12-96 months) after completion of salvage IVC, globe salvage was achieved in 22 (54%) eyes, 1 (3%) patient had histopathology-proven bone metastasis, and 1 (3%) patient died because of presumed metastasis. CONCLUSION: Secondary salvage IVC with appropriate focal treatment allows globe salvage in 54% eyes with refractory/recurrent retinoblastoma and thus serves as an alternative to intraarterial chemotherapy or enucleation.

Metastatic retinoblastoma at presentation: Clinical presentation, treatment, and outcomes.

The aim of this study was to retrospectively determine clinical features, treatment outcomes, and overall survival in four patients with metastatic retinoblastoma at presentation. The mean age at diagnosis was 63 months (range: 24-108 months). Three patients had overt orbital disease of at least one eye and one patient had microscopic orbital disease with scleral infiltration on histopathology. Metastatic sites included regional lymph nodes (RLN) (n = 4), bone marrow (BM) (n = 2), and cerebrospinal fluid (CSF) (n = 1). The most common sites of RLN were ipsilateral preauricular nodes (two patients) and contralateral parotid gland involvement (one patient). The treatment administered included primary enucleation (n = 1), high-dose intravenous chemotherapy (n = 4), secondary enucleation (n = 2), orbital external beam radiotherapy (n = 3), and intrathecal chemotherapy (n = 1). High-risk features included massive choroidal and microscopic scleral infiltration in the eye that underwent primary enucleation. At a mean follow-up of 33 months (range, 4-68 months), one patient with CSF involvement deceased in 4 months. The remaining three patients were alive and disease-free at the last mean follow-up period of 43 months (range, 18-68 months). The results of our study showed that RLN and BM metastasis respond well to treatment while CSF metastasis is associated with poor prognosis.