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Retinoblastoma in Children - Signs, Symptoms, and Treatments

Retinoblastoma in Children Introduction

Most cancers are named for the part of the body where the cancer starts. Retinoblastoma is a cancer that starts in the retina, the very back part of the eye. It is the most common type of eye cancer in children. However, in general, retinoblastoma is a rare disease. Only about 300 children are diagnosed with retinoblastoma each year in the United States. Failure to recognize retinoblastoma early may lead to blindness or death among affected children.

What is retinoblastoma?

Retinoblastoma (RB) is a malignant (highly cancerous) tumor of the developing retina that occurs in children, usually before the age of five years.

Who is at risk?

Age: It is more common in infants and very young children than in older children. The average age of children when they are diagnosed is 2. Retinoblastoma occurs about equally in boys and girls and in different races and ethnicities.

Is there an age cutoff when I can stop worrying about it developing?

While there is no "definite" cut-off factor for the risk of developing retinoblastoma, it rarely occurs in children older than 6.

Is Retinoblastoma genetic?

Retinoblastoma is mainly caused by a genetic defect in the retina. The retinoblastoma gene is a specific tumor suppressor gene. Every cell in the body has one pair of these genes. A tumor suppressor gene encodes a protein that controls cell division. When the gene is not active, cells can divide out of control. Retinoblastoma occurs when the retinoblastoma gene does not make the protein that controls cell division. This causes the photoreceptor cells in the retina to multiply too rapidly and form a tumor.

How did my child get retinoblastoma? I didn't have any mutated genes?

There are two forms of retinoblastoma—hereditary (runs in the family) and sporadic. 60% of children with retinoblastoma have the unilateral sporadic form of the disease.

Why is it the fastest growing cancer in children today?

Retinoblastoma is the fastest growing cancer in the child population today. In the United States, 300 new cases occur each year. It affects one of every 15,000 to 17,000 children. Worldwide, there are 5,000 new cases per year. During the ?rst few years of life, the eye grows rapidly. Sometimes during this rapid growth period, cells within the eye can divide in an abnormal way. This is how the disease begins to develop.

How do I know if my child has it (Signs & symptoms)?

About 3 out of 4 children with retinoblastoma have a tumor in only one eye. In about 1 case in 4, both eyes are affected.

Common signs of retinoblastoma include:

  • A white "glow" or "glint" in the pupil of one or both eyes
  • The presence of a white pupil in color photographs
  • Crossed or misaligned eyes

How is retinoblastoma diagnosed?

One must rely on a doctor's exam and other tests to diagnose the disease. We can perform a limited exam in the of?ce to catch a glimpse of the tumor and to confirm the referring doctor's diagnosis. However, your child will need a more detailed exam, and it must be done in the operating room with your child asleep. The doctor examines the tumor in the retina looking for signs of calcium, dilated (enlarged) blood vessels, parts of the retina that have detached, and overlying vitreous seeds. These seeds are small pieces of tumor that have broken free from the main tumor mass and are floating in the vitreous jelly.

To confirm the diagnosis, CT scan, MRI & ultrasonography can be used as an additional measure.

When should I start having their eyes tested if I decide to have children?

Detection of Retinoblastoma in the first two months (after it occurrence) is essential to prevent blindness. Children with a family history of retinoblastoma should be closely followed by an eye specialist until they reach 28 months. If only one eye is affected, periodic screening of the other eye should be conducted by an eye specialist until the age of 7 years. As well, siblings of a child with retinoblastoma should have their eyes routinely checked by an eye specialist.

How is retinoblastoma treated?

Overall, more than 90% of children with retinoblastoma can be cured, but the outlook is not nearly as good if the cancer has spread to other parts of the body. The high cure rate for this disease in the developed world is the result of early detection (?nding the disease early) and ongoing treatment advances. A variety of therapies, including chemotherapy, cryotherapy (freezing the tumor), laser therapy (light that produces heat), and radiation therapy, may be used to treat your child's cancer. Each type of treatment can be used alone or combined with one or more of the others. Enucleation (removal of the eye) will be performed only if other therapies do not work well or will not work well on your child's tumor.

If I know I have this mutation, should I have children?

Studies show that a child with unilateral sporadic disease Is generally the only person in the family with the disease and has a 4% chance of later parenting a child with the disease. On the other hand, each child of a parent with an abnormal retinoblastoma gene has a 45% chance of having the disease 45% chance of later parenting a child who will have the disease. In simple words, about 1 in 4 cases of retinoblastoma are hereditary, so survivors should consider genetic counseling before having children, to understand the risks of passing the gene change on to their children and perhaps to explore ways to avoid this. For example, an option some people might consider would be to use in vitro fertilization and implant only embryos that don't have the gene change.

If a preventive option is not used, children born into a family with a history of retinoblastoma should be screened for this cancer starting shortly after birth because early detection of this cancer greatly improves the chance for successful treatment.

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