The Childrens Cancer Group (CCG) has developed a classification for extraocular spread of retinoblastoma. The extent of disease correlates with long-term survival.
Extraocular Extent
Class
microscopic involvement of the scleral emissaries
I
microscopic involvement of the resected end of the optic nerve
II
orbital disease
III
spread to central nervous system (brain mass, positive CSF cytology)
IV
distant metastases (bone, lymph node, bone marrow)
V
where:
• Tumor may spread along the optic nerve to the central nervous system.
• Distant metastases may travel by blood or lymphatics.
Group
Probability of 3 Year Survival
I to III
80%
IV or V
17%
from Figure 3, page 1296
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