Weill Medical College of Cornell University

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Retinoblastoma Treatment

Retinoblastoma is the most frequent eye cancer in children. It is caused by a gene mutation occurring once in every several thousand births. Often the only cure has been to remove the eye or the disease can be fatal. About 40% of retinoblastoma cases are hereditary, linked to a specific gene mutation. This can be determined by genetic testing, and a blood test can determine the presence of the retinoblastoma gene. If either parent had a retinoblastoma, the child has a significant risk of having a retinoblastoma as well. Doctors recommend that these children receive eye exams every few months until the age of 3. Retinoblastoma tumors surface on the retina and, if left untreated, can spread to the brain via the optic nerve or metastasize to other organs. For half of the children in the world who develop this condition it is fatal.

A retinoblastoma tumor can often be seen with a flashlight, and in flash photographs, where, instead of the usual "red eye," the child's pupil will look white. Indicators include

• an unusual glint or white-looking pupil
• crossed or lazy eyes, or
• vision loss.

A new, highly innovative treatment for this cancer has been developed in collaboration by surgeons at Weill Cornell and Memorial Sloan Kettering. The treatment uses intra-arterial chemotherapy, or "chemosurgery and angiography (x-ray-based imaging). The neuro interventional surgeon directs a microcatheter (a tiny plastic tube) into the artery feeding the eye. There, a stream of pharmaceutical agents is injected directly into the eye's artery. This method allows the concentration of chemicals at far higher levels than when injected into a vein in the arm, where approximately less than 1 percent reaches the eye. The procedure takes approximately one hour, and the tumor has been shown to visibly start to shrink within three weeks. This experimental technique, first performed at Weill Cornell, has been applied to advanced retinoblastoma in which the conventional treatment was enucleation (eye removal). The initial results show that two-thirds of the eyes treated with this technique can be saved.