Retinoblastoma is an eye cancer that most often occurs in children younger than five years of age. It occurs in hereditary and nonhereditary (sporadic) forms.
Understanding Retinoblastoma
The retina is the nerve tissue that lines the inside of the back of the eye. It senses light and sends images to the brain by way of the optic nerve.
Although retinoblastoma may occur at any age, it most often affects children younger than five years of age. The tumor is usually found in only one eye, but can develop in both eyes. Retinoblastoma rarely spreads from the eye to nearby tissue or other parts of the body, and it can usually be cured.
Retinoblastoma is sometimes inherited (passed from the parent to the child). Such cases of the cancer caused by an inherited gene mutation are referred to as hereditary retinoblastoma. It usually occurs at a younger age than retinoblastoma that is not inherited. Retinoblastoma that occurs in only one eye is usually not inherited (called sporadic retinoblastoma). When it occurs in both eyes, it is always inherited. When the hereditary form first occurs in only one eye, there is a chance it will develop later in the other eye.
After a diagnosis of retinoblastoma in one eye, regular follow-up exams of the healthy eye should be done every 2 to 4 months for at least 28 months. After treatments are finished, it is important that follow-up exams continue until the child is seven years of age.
Treatment for both types of retinoblastoma should include genetic counseling (a discussion with a trained professional about inherited diseases). Brothers and sisters of a child who has retinoblastoma should also have regular checkups and genetic counseling concerning the risk of developing the cancer.
A child who has hereditary retinoblastoma is at risk for developing pineal tumors in the brain. This is called trilateral retinoblastoma. Regular follow-up exams to check for this rare condition are important during treatment and for at least four years after the child is diagnosed. Hereditary retinoblastoma also increases the child's risk of developing other types of cancer in later years.
Symptoms of Retinoblastoma
Symptoms can include the following:
- The pupil of the eye appears white instead of red when light shines into it. This may be seen in flash photographs of the child.
- Eyes appear to be looking in different directions (crossed eyes).
- Pain or redness in the eye.
These possible symptoms are not sure signs of retinoblastoma. Other, less serious, health problems can also cause these symptoms. Anyone with these possible symptoms of retinoblastoma should see a healthcare provider as soon as possible.
If a person has possible symptoms of this condition, the healthcare provider may do a physical exam and ask about the patient's personal and family medical history. In order to help make a diagnosis, the healthcare provider will also recommend additional tests and procedures that examine the eye.
The exams and tests involved in diagnosing retinoblastoma may include the following:
- Eye exam with dilated pupil
- Ultrasound
- CT (computed tomography) scan
- MRI (magnetic resonance imaging).
A retinoblastoma diagnosis is usually made without a biopsy (removal of cells or tissues so they can be viewed under a microscope to check for signs of cancer).
Once a healthcare provider has made a diagnosis, he or she may order more tests to determine if cancer cells have spread to other parts of the body. This is called staging. It is important to know the retinoblastoma stage in order to plan appropriate treatment.
The following tests and procedures may be used in the staging process:
- Eye exam
- Ultrasound
- CT scan
- MRI
- Lumbar puncture.
There are several staging systems for retinoblastoma. For treatment, the most commonly used staging system classifies the cancer as either intraocular (within the eye) or extraocular (outside the eye). Cancer that has returned is called recurrent.
How Is Retinoblastoma Treated?
Different types of retinoblastoma treatments are available. The treatment options vary based on:
- The retinoblastoma stage
- How likely it is that vision can be saved in one or both eyes
- The size and number of tumors.
In general, treatment can include:
- Enucleation
- Radiation therapy
- Cryotherapy
- Photocoagulation
- Thermotherapy
- Chemotherapy
- A combination of these.
Your healthcare provider can describe these retinoblastoma treatments and the expected results of each. You and your healthcare provider can work together to develop a treatment plan that meets your child's medical needs and your personal values. Choosing the most appropriate treatment is a decision that ideally involves the patient, family, and healthcare team.
The prognosis for this condition depends on:
- The stage of retinoblastoma
- How likely it is that vision can be saved in one or both eyes
- The size and number of tumors
- Whether trilateral retinoblastoma occurs.
(Click Retinoblastoma Prognosis for more information.)
- Retinoblastoma is a disease in which cancer cells form in the tissues of the retina
- It is sometimes caused by a genetic mutation passed from the parent to the child
- A child who has hereditary retinoblastoma is at risk for developing trilateral retinoblastoma and other cancers
- Possible signs of retinoblastoma include "white pupil" and eye pain or redness
- Tests that examine the retina are used to detect and diagnose retinoblastoma
- Certain factors (such as the size and number of tumors) affect a person's prognosis and treatment options.
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