Retinoblastoma is an eye cancer that begins in the retina — the sensitive lining on the inside of your eye. Retinoblastoma most commonly affects young children, but can rarely occur in adults. Your retina is made up of nerve tissue that senses light as it comes through the front of your eye. The retina sends signals through your optic nerve to your brain, where these signals are interpreted as images. A rare form of eye cancer, retinoblastoma is the most common form of cancer affecting the eye in children. Retinoblastoma may occur in one or both eyes.

Types of Retinoblastoma

Unilateral retinoblastoma affects one eye and accounts for 60% to 75% of cases. About 85% of these diagnoses are the sporadic form of the disease. The rest are hereditary cases.

Bilateral retinoblastoma affects both eyes. It is almost always hereditary. Patients with bilateral retinoblastoma are typically diagnosed at a younger age (less than one year old) than those with unilateral disease.

PNET (primitive neuroectodermal tumors) retinoblastoma occurs when an associated tumor forms in primitive nerve cells in the brain. This form of the disease occurs only in children with hereditary retinoblastoma. It affects just 5% of patients with bilateral retinoblastoma. It also is known as trilateral retinoblastoma.

Symptoms of Retinoblastoma

The first clue and most obvious symptom is that the eye doesn’t look right. Specifically, its normally black pupil may look white. In a photo, instead of "red eye," a child with retinoblastoma will have one pupil that glows white when light shines on it.

Others symptoms include:

• An eye or eyes that appear larger than normal
• Cloudiness or discoloration in the center of the eye
• Eye pain
• Eyes that cross or look in different directions
• Redness in the white of the eye
• Vision problems

Causes of Retinoblastoma

Retinoblastoma occurs when nerve cells in the retina develop genetic mutations. These mutations cause the cells to continue growing and multiplying when healthy cells would die. This accumulating mass of cells forms a tumor.

Retinoblastoma cells can invade further into the eye and nearby structures. Retinoblastoma can also spread (metastasize) to other areas of the body, including the brain and spine.

In the majority of cases, it's not clear what causes the genetic mutations that lead to retinoblastoma. However, it's possible for children to inherit a genetic mutation from their parents.

Treatments

The sooner the cancer is found, the greater the chances are that your child’s eyesight can be saved. Doctors usually use a combination of treatments such as:

• Cryotherapy: A touch with a super-cooled metal probe freezes and kills cancer cells. This works best on small tumors near the front of the eye.
• Thermotherapy: A special laser kills cancer cells with heat. Doctors use it by itself on small tumors or along with other treatments for larger tumors.
• Laser Therapy: A different kind of laser targets and destroys the blood vessels that supply the tumor. This works for small tumors in the back of the eye.
• Radiation: There are two kinds of radiation therapy. For small tumors, a surgeon can sew a disc that has radioactive material inside it onto the eyeball near the tumor. Your child stays in the hospital a few days while it works, and then the disc is removed. Older technology uses a machine to focus beams of radiation on the tumor. This usually is used only if other strategies don’t work.
• Surgery: If the tumor is very large by the time it’s found, it may not be possible to save the child’s vision. In these cases, the eye may be removed.

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Sarah Rose

Journal Manager Journal of Eye Diseases and Disorders

Email: eyedisorders@emedsci.com

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