Retinoblastoma is the most common cancer of the eye in children and accounts for 2.5 to 4% of all childhood cancers. When a child is diagnosed with retinoblastoma, the cancer may be limited to the eye globe (intraocular) or have spread beyond the eye globe (extraocular). The spread may be local (to the orbit and local lymph nodes) or more distant i.e metastatic. Delays in presentation in India lead to more than half of the children with retinoblastoma having the cancer spread outside the globe at diagnosis.
Sameer Bakhshi and his colleagues describe the treatment and outcome of 25 children with extraocular but non-metastatic retinoblastoma over a 5 year period (2003 to 2008) from one of the premier treatment centres in India. The overall survival figures are not given but at a median follow up of 28.5 months (nearly 2½ years) 13 of the 25 children had progressed/relapsed.
All those who progressed or relapsed, either refused or did not respond to second line treatment which implies that they would all ultimately die of the cancer. One also needs to consider that there were 51 children with extraocular non-metastatic retinoblastoma during this time period but only 25 opted for treatment. Many if not all of those who did not opt for treatment for various reasons are also likely to have succumbed to the cancer. These poor outcomes are all the more significant as majority of the children in India with retinoblastoma present late and the disease has spread locally or systemically. Hence, it is not surprising that the five year overall survival of children with retinoblastoma (all stages combined) is less than 50% while that in Europe and North America is 95% and above.