Cavitary retinoblastoma: Clinical features and treatment outcomes

PURPOSE: To describe clinical characteristics and treatment outcomes of children diagnosed with cavitary retinoblastoma (RB). METHODS: Retrospective analysis of children treated for cavitary RB. RESULTS: Of the 40 patients (43 eyes) with cavitary RB, 14 (35%) were female and 26 (65%) were male. At diagnosis, the average age was 25 months (median, 24 months; range 3-84 months). In 3 cases (8%), bilateral cavitary RB was seen. The most prevalent presenting symptoms were leukocoria (n = 21, 53%) and strabismus (n = 6, 15%) with a mean duration of symptoms of 23 weeks (median, 16 weeks; range 0-104 weeks). As per the ICRB classification, 15 eyes (35%) were in group D, 12 eyes (28%) in group C, 10 eyes (23%) in group B, and 6 eyes (14%) in group E. Primary cavities, within the tumor, were seen in 14 eyes (33%), whereas secondary cavities were seen in 29 eyes (67%). Most of the eyes (n = 42, 98%) were treated with intravenous chemotherapy (IVC), and one eye (2%) received intra-arterial chemotherapy. In ten eyes (23%), the tumor recurred following IVC, and two cases exhibited a partial response to IVC. At the last follow-up (mean, 31 months), all eyes (100%) had local tumor control with no evidence of systemic metastasis or death. Regression of the cavities within the tumor was noted in 27 eyes (63%), while it remained stable in 16 eyes (37%). CONCLUSION: Cavitary RB (both primary and secondary) had a good initial response to standard chemotherapy with an overall excellent prognosis for globe salvage.