Although retinoblastoma (RB) is one of the most curable pediatric malignant abnormalities, death owing to metastatic disease is a real risk to many of the 8000 children who develop RB worldwide each year.1- 5 Pathologic examination of the enucleation specimen currently guides need for adjuvant therapy. Most retinoblastoma experts agree that risk factors for metastasis include “massive” choroidal invasion over 3 mm in any dimension, postlamina cribrosa invasion into the optic nerve, and any combination of any degree of uveal, optic nerve, anterior segment, or scleral invasion. In general, retinoblastoma without high-risk histopathologic features rarely demonstrates metastatic disease, whereas those with high-risk features show a moderate propensity for metastatic events.
Shields CL, Leahey AM. Detection of Retinoblastoma at Risk for Metastasis Using Clinical and Histopathologic Features and Now mRNA. JAMA Ophthalmol. 2016;134(12):1380-1381. doi:10.1001/jamaophthalmol.2016.2994