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Table 7. International Retinoblastoma Staging System

Stage Description 
CNS = central nervous system; CSF = cerebrospinal fluid.
Stage 0Eye has not been enucleated and no dissemination of disease (refer to the International Classification of Retinoblastoma section of this summary for more information).
Stage IEye enucleated, completely resected histologically
Stage IIEye enucleated, microscopic residual tumor
Stage IIIRegional extensiona. Overt orbital disease
b. Preauricular or cervical lymph node extension
Stage IVMetastatic diseasea. Hematogenous metastasis (without CNS involvement)
1. Single lesion
2. Multiple lesions
b. CNS extension (with or without any other site of regional or metastatic disease)
1. Prechiasmatic lesion
2. CNS mass
3. Leptomeningeal and CSF disease