High-risk retinoblastoma based on age at primary enucleation: a study of 616 eyes

Abstract

Purpose: To study the high-risk histopathology features of retinoblastoma based on age at primary enucleation. Methods: Retrospective study of 616 patients. Results: The mean age at presentation and primary enucleation for retinoblastoma was 34 months (median, 28 months; range, <1–455 months). Of these cases, 128 (21%) were aged ≤1 year, 149 (24%) were in the age group of 1–2 years, 117 (19%) in 2–3 years, 104 (17%) in 3–4 years, and 118 (19%) were >4 years of age at the time of enucleation. Bilateral retinoblastoma (34%; p < 0.0001) and buphthalmos (20%; p < 0.0001) were more common in children ≤1 year of age. Anterior chamber pseudohypopyon (15%; p < 0.0001) and vitreous seeds (53%; p < 0.0001) were more common in children aged >4 years. Based on 8th edition American Joint Committee on Cancer staging system, pT3 was less common in children ≤1 year of age (13%; p < 0.001). Based on histopathology, 38% patients had high-risk features including 24% children aged ≤1 year, 42% in the age group of 1–2 years, 34% in 2–3 years age group, 45% in 3–4 years age group, and 48% patients were >4 years of age. Post-laminar optic nerve infiltration (6%; p = 0.02) and massive choroidal infiltration (9%; p = 0.04) was least common in children ≤1 year of age. Over a mean follow-up period of 52 months (median, 36 months; range, <1–218 months), systemic metastasis and death occurred in 9% patients despite adjuvant systemic chemotherapy. Conclusion: The predominant high-risk histopathology feature of retinoblastoma varies with age at primary enucleation.