Eye Cancers (Retinoblastomas) Support Group

Retinoblastoma is the most common type of malignant intraocular tumors found in children. This type occurs in one out of every twelve to twenty thousand children and develops in the retina of the eye. Retinoblastomas may be hereditary or may develop sporadically. The less common hereditary type is usually present at birth and has its onset near one year of age. In both types, the condition is almost always expressed by the age of five and is often bilateral. Although hereditary retinoblastomas are present in family histories, any occurrence of bilateral retinoblastoma should be considered hereditary and capable of being passed to further generations. Infants of such families should be screened regularly for tumors. Sporadic tumors are not hereditary. They appear at approximately two years of age and generally affect only one eye.

The first symptoms include redness, pain, and inflammation. As the tumor grows, the eyes may cross and the pupil may change from black to white or gray, a condition called leukokoria. The light color is the hue of the tumor visible through the hole of the pupil.

Treatment involves enucleation, radiation, or cryotherapy (freezing treatments). In bilateral cases, the more affected eye may be removed and the other eye may receive chemotherapy or radiation treatments. If left unchecked, the tumor could grow and spread up the optic nerve to the brain.

Cause is the absence of both retinoblastoma genes on the 13th chromosome. Although it has one of the highest rates of spontaneous regression among all tumors, little is known about the cause of the regression. The cure rate for retinoblastoma ranges from 85% to 95%.
Posted on 10/02/08, 06:10 am