Retinoblastoma staging

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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

According to the International classification of retinoblastoma, there are five stages of retinoblastoma based on the tumor size, location, local spread, neovascular glaucoma, intravascular hemorrhage, and orbital cellulitis.

Staging

• There is two known staging system for retinoblastoma:
  • American Joint Committee on Cancer (AJCC) staging system
  • International Retinoblastoma Staging System
    • This staging system is used in the clinical setting greater than the AJCC staging system.

International Retinoblastoma Staging System

Stage	Description
Stage 0	Eye enucleated and no dissemination of the disease
Stage I	Eye enucleated, completely resected histologically
Stage II	Eye enucleated, microscopic residual tumor
Stage III	Regional extension			a.Overt orbital disease b.Preauricular or cervical lymph node extension
Stage IV	Metastatic disease			a.Hematogenous metastasis (without CNS involvement) —Single lesion —Multiple lesions b.CNS extension (with or without any other site of regional or metastatic disease) —Prechiasmatic lesion —CNS mass —Leptomeningeal and CSF disease