Basic understanding of current classification and management of retinoblastoma

Purpose of review The current classification and management strategies for retinoblastoma are discussed. Recent findings The International Classification of Retinoblastoma is a new classification system for retinoblastoma and is based on tumor size, location, and associated seeding: group A = retinoblastoma up to 3 mm in size; group B = retinoblastoma more than 3 mm in size, macular location, or minor subretinal fluid; group C = retinoblastoma with localized seeds; group D = retinoblastoma with diffuse seeds; and group E = massive retinoblastoma necessitating enucleation. This classification was designed to simplify grouping and to assist in predicting treatment outcomes. Chemoreduction is an important therapy for bilateral retinoblastoma and some cases of unilateral retinoblastoma. This International Classification of Retinoblastoma can reliably predict chemoreduction outcome, as success was achieved in 100% of group A, 93% of group B, 90% of group C, and 47% of group D cases. Group E is typically managed with enucleation. Summary The new classification of retinoblastoma allows better judgement for success with chemoreduction for retinoblastoma.