Eye Matters
November 11, 2005
Retinoblastoma- malignant tumor of the eye

Before we enter into this conversation, I would like to remind you that the retina is a nervous tissue that covers the interior of the posterior part of the eye. It is responsible for light detection and the sending of images to the brain via the optic nerve to achieve our perception of our environment and the surrounding objects.

Retinoblastoma is the most common eye cancer, in children. It occurs with a frequency of about 1 patient in every 14 live births. There are no determined causes for the cancer, however advanced parental age and excess cancer in relatives have been found to be associated with retinoblastoma. It is usually unilateral (one eye) in approximately 67% of cases and bilateral (in both eyes) in 33% of these cases. {{more}}

Why would the patient’s family bring him/her to see us? Leucocoria (whiteness of the baby’s eye) is the cause in about 50% of these patients and strabismus (cross eye) in another 20%. Other less common causes include poor vision, red eye and glaucoma.

At what age does retinoblastoma typically present? It is usually diagnosed at an average age of 18 months and varies between 9 and 16 months. The results of the physical examination depend on the stage that the tumor is found. The most common sypmtoms are decreased vision, leucocoria, strabismus, nystagmus (rapid, involuntary, oscillatory motion of the eye), hyphema (blood in the anterior chamber of the eye) and proptosis (protruding eye).

What tests are necessary to make a final diagnosis of this disease? First we need to take into consideration what the patient and/or family refers to us then imaging tests are done (X-ray, CT-Scan). The ophthalmologic examination should be done by the ophthalmologist as well as the final diagnosis of this terrible tumor.

The treatment is determined in relation to the size of the tumor; If the tumor is unique or multiple, if it is found in one eye or both eyes and if there is invasion of the optic nerve with the possibility of brain damage and metastasis. Variations in treatment include radiotherapy, photocoagulation, cryotherapy or surgical intervention extracting the eyeball (enucleation) all depending on the aforementioned.

There is one very important fact that we must bear in mind and that is this tumor is a very serious disease that has a reserved prognosis which depends on multiple factors including the response to the actual treatment. This is why it is of extreme importance that the patient is referred or brought to the ophthalmologist as soon as visual deficit or any damage is suspected, because with early diagnosis and treatment the survival rate is greater than 90%.

Remember my fellow readers, today’s children are the hope of tomorrow’s world.