Cavitary retinoblastoma: A review of literature

Abstract

Cavitary retinoblastoma is a rare tumor variant manifesting in the form of ophthalmoscopically visible translucent cavities within the tumor. The pathogenesis of the cavitary variant is unknown. Histopathologically, the cavitary spaces represent photoreceptor differentiation in the areas adjacent to the cavitations, which might explain variable response to chemotherapy with lower risk of reactivation and good prognosis. There is, however, little understanding about its origin, frequency, pathogenesis, and clinical significance. In this systematic review of the literature, we analyze epidemiology, clinical presentation, genetic implications, imaging, and treatment outcomes of various regimens (intravenous, intra-arterial, and focal) in terms of local control, recurrences, and globe salvage rates.