Retinoblastoma classification

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Simrat Sarai, M.D. [2]

Overview

Retinoblastoma may be classified into several subgroups based on the International Retinoblastoma Staging System (IRSS).

Classification

The International Classification for Intraocular Retinoblastoma, based upon the Philadelphia and Murphree systems is currently the classification being used by the Children's Oncology Group in multicenter treatment protocols. The goal of International Classification of Retinoblastoma (ICRB) is to reflect the likelihood of ocular survival based on modern treatment techniques such as chemotherapy and focal therapy. The International Classification of Retinoblastoma (ICRB) was developed to better predict those with intraocular retinoblastoma who are likely to be cured without the need for external-beam radiation treatment or enucleation. International retinoblastoma classification predicts the overall survival in patients with retinoblastoma.

Groups	Features
Group A	Retinoblastoma 3 mm or less in basal dimension or thickness, located at least 3 mm from the foveola and 1.5 mm from the optic nerve
Group B	Retinoblastoma not in Group A with 1 or more of the following: Macular location (≤3 mm to foveola) Juxtapapillary location (≤1.5 mm to optic nerve) Additional subretinal fluid (≤5 mm from margin)
Group C	Retinoblastoma tumor with 1 of the following: Focal subretinal seeds Focal vitreous seeds Both focal subretinal and vitreous seeds.
Group D	Retinoblastoma tumor with 1 of the following: Diffuse subretinal seeds Diffuse vitreous seeds Both diffuse subretinal and vitreous seeds
Group E	Very high-risk eyes with 1 or more of the following: Neovascular glaucoma Massive intraocular hemorrhage Aseptic orbital cellulitis Tumor anterior to the vitreous face Tumor touching the lens Diffuse infiltrating retinoblastoma Phthisis bulbi (also known as end-stage eye, this is a nonfunctioning, atrophic, scarred, and disorganized globe, frequently with dystrophic calcification).