Retinoblastoma in older children

Abstract

Purpose: Retinoblastoma (RB) is known to present with atypical signs and symptoms in older patients. Our article reviews the clinical and histopathological features of RB encountered in children older than 5 years of age.

Methods: A total of 337 consecutive patients with RB were reviewed. Eighteen (5.3%; 10 males, 8 females) who presented after the age of 5 were selected for further analysis of clinical data and histopathological material in this retrospective, non-comparative case series.

Results: The age range was from 5 to 12 years (mean age 6.4). Unilateral disease was seen in 14 children, bilateral disease in 4 children. Eleven children were the product of consanguineous marriages. Seven patients had leukocoria; the others presented with signs of uveitis, cellulitis, and/or trauma. Three patients had flat, plaque-like lesions on histopathologic evaluation.

Conclusions: Clinical and histopathologic features were atypical in 47% and 21% respectively in the retinoblastoma patients who presented after the age of 5 years.