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%.
Dr Fred

Intraocular Melanoma (Melanoma of the Eye)

Intraocular melanoma (also called uveal melanoma) although rare, is the most common type of cancer that develops within the eyeball in adults. Melanomas of the skin are much more common than uveal melanomas and develop from pigment-producing cells called melanocytes. When melanoma develops in the eyeball, it is usually in the part of the uvea called the choroid. Choroid cells have the same kind of pigment as melanocytes in the skin, so it is not surprising that these cells sometimes form melanomas. Approximately 90% of intraocular melanomas develop in the choroid.

Nearly all of the remaining 10% of intraocular melanomas are melanomas of the iris. They are the easiest for the patient and doctor to see because they often arise in a pigmented spot on the iris that has been present for many years and then begins to grow. These melanomas usually are relatively slow growing, and they rarely spread to other parts of the body. For these reasons, people with iris melanomas generally have a good prognosis (outlook for survival).

Intraocular melanomas are generally composed of 2 different kinds of cells.


spindle cells -- These are elongated cells.

epithelioid cells -- These cells are almost round but with some straight edges.
Most of the tumors are composed of both kinds of cells. The outlook is best if the tumors are mostly spindle cells and worse if they are mostly epithelioid cells. Epithelioid tumors are more likely to metastasize (spread) to distant sites and be fatal. If you have ocular melanoma, your doctor can tell you which type of cells were found.
Dr Fred