Congenital Retinoblastoma: A Case Report

Abstract

Retinoblastoma is usually diagnosed during the first three years of life, with an average age of diagnosis of 18 months. It is almost never found at birth except when there is a positive family history which prompts an early fundus examination. We report a case of unilateral nonfamilial retinoblastoma which was so advanced at birth that it had already produced a perforated globe. Despite enucleation, radiation, and chemotherapy, massive orbital recurrence developed which necessitated a modified orbital exenteration. In spite of this aggressive therapy the child died of metastatic retinoblastoma at age eight months.

Neonatal Retinoblastoma

“…Although it is considered unusual to diagnose a neonatal retinoblastoma in the absence of a positive family history, [33] series from various cancer centers have included 28%-76% of such cases [Table 3]. In a retrospective case series from New York, leukokoria led to diagnosis in 13% of neonatal retinoblastoma patients.…”