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.