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Retinoblastoma
What is it?
What Causes it?
Who are at risk?
What are the symptoms?
How is it diagnosed?
What is the treatment?
What are the surgical options?
 
What is it?
 

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. The retina is made up of nerve tissue that senses light as it comes through the front of the eye. The retina sends signals through the optic nerve to the 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.

What Causes it?
 

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.

Who are at risk?
 

Retinoblastoma most commonly affects young children, but can rarely occur in adults. The retina is made up of nerve tissue that senses light as it comes through the front of the eye. The retina sends signals through the optic nerve to the 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.

What are the symptoms?
 

A white color in the center circle of the eye (pupil) when light is shone in the eye, such as when taking a flash photograph

Eyes that appear to be looking in different directions

Eye redness

Eye swelling .The red reflex: checking for a normal reddish-orange reflection from the eye's retina with an ophthalmoscope or retinoscope from approximately 30 cm / 1 foot, usually done in a dimly lit or dark room.The corneal light reflex / Hirschberg test: checking for symmetrical reflection of beam of light in the same spot on each eye when a light is shined into each cornea, to help determine whether the eyes are crossed.

How is it diagnosed?
 

Eye exam. The eye doctor will conduct an eye exam to determine what's causing child's signs and symptoms. For a more thorough exam, the doctor may recommend using anesthetics to keep child still.

Imaging tests. Scans and other imaging tests can help child's doctor determine whether retinoblastoma has grown to affect other structures around the eye. Imaging tests may include ultrasound, computerized tomography (CT) scan and magnetic resonance imaging (MRI), among others.

Consulting with other doctors. child's doctor may refer you to other specialists, such as a doctor who specializes in treating cancer (oncologist), a genetic counselor or a surgeon.

What is the treatment?
 

What treatments are best for child's retinoblastoma depends on the size and location of the tumor, whether cancer has spread to areas other than the eye,child's overall health and own preferences. When possible, child's doctor will work to preserve your child's vision.

Chemotherapy is a drug treatment that uses chemicals to kill cancer cells. Chemotherapy can be taken in pill form, or it can be given through a blood vessel. Chemotherapy drugs travel throughout the body to kill cancer cells.

In children with retinoblastoma, chemotherapy may help shrink a tumor so another treatment, such as radiation therapy, cryotherapy, thermotherapy or laser therapy, may be used to treat the remaining cancer cells. This may improve the chances that your child won't need surgery.

Chemotherapy may also be used to treat retinoblastoma that has spread to tissues outside the eyeball or to other areas of the body.

In advance cases surgery is the only option.

What are the surgical options?
 

Surgery to remove the affected eye (enucleation). During surgery to remove the eye, surgeons disconnect the muscles and tissue around the eye and remove the eyeball. A portion of the optic nerve, which extends from the back of the eye into the brain, also is removed.

Surgery to place an eye implant. Immediately after the eyeball is removed, the surgeon places a special ball — made of plastic or other materials — in the eye socket. The muscles that control eye movement are attached to the implant. After the child heals, the eye muscles will adapt to the implanted eyeball, so it may move just as the natural eye did. However, the implanted eyeball cannot see.

Fitting an artificial eye. Several weeks after surgery, a custom-made artificial eye can be placed over the eye implant. The artificial eye can be made to match the child's healthy eye. The artificial eye sits behind the eyelids and clips onto the eye implant. As the child's eye muscles move the eye implant, it will appear that the child is moving the artificial eye.

What are the outcomes?
 

The overall and event-free survival rates of retinoblastoma were satisfactory and eye- saving was possible with primary chemotherapy. Development of new chemotherapeutic regimens and a team approach are necessary to improve the eyeball-saving rate.

What are the complications?
 

Children treated for retinoblastoma have a risk of cancer returning in and around the treated eye. For this reason,the child's doctor will schedule follow-up exams to check for recurrent retinoblastoma. The doctor may design a personalized follow-up exam schedule for the child. In most cases, this will likely involve eye exams every few months for the first few years after retinoblastoma treatment ends.

Additionally, children with the inherited form of retinoblastoma have an increased risk of developing other types of cancers in any part of the body in the years after treatment. For. this reason, children with inherited retinoblastoma may have regular exams to screen for other cancers.

What is the time course?
 

Regular follow ups are required after the treatment as well as before the treatment.

What is the expense?
 

The expense depends upon the grades of treatment.

 
Author: Dr. Sanjay Dhawan
Last Updated on: 1 March, 2014
   

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