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Retinoblastoma

Fig. 1 Retinoblastoma pre-treatment.

What is retinoblastoma?

Retinoblastoma is a malignant tumor of the eye(s) that originates from the retina (light-sensitive lining of the eye). One (unilateral) or both (bilateral) eyes may be affected and typically occurs in children less than 5 years old.

How common is retinoblastoma?

Retinoblastoma affects approximately 300 children per year in USA and Canada. Worldwide, about 6000 children develop retinoblastoma each year.

How does a child get retinoblastoma?

The tumor develops when there is a gene abnormality on chromosome number 13. Chromosomes contain the genetic codes that control cell growth and development. 90% of retinoblastoma cases develop “out of the blue” and without warning. 10% have a family member with retinoblastoma. 40% of children with retinoblastoma have a genetic, inherited form of the tumor, even if no-one else in the family has the problem. Children of affected individuals need exams for retinoblastoma. The other type of retinoblastoma is non-genetic and is not passed on from one generation to the next. Retinoblastoma is not caused by external factors such as smoking, drinking, etc.

Fig. 2 Retinoblastoma post-treatment.

How is retinoblastoma diagnosis confirmed?

An eye MD examines the eyes either in the office or in the operating room under anesthesia. A CT scan and/or eye ultrasound are often performed. Other tests may include blood-work and an MRI. Referral to an oncologist may be suggested.

What is the prognosis for retinoblastoma?

Retinoblastoma is one of the most curable (95%) of childhood cancers. Many children have unilateral retinoblastoma which has an excellent prognosis. The prognosis for bilateral involvement depends on the size and location of the tumors.

What treatments are available for retinoblastoma?

• Laser Photocoagulation and Thermotherapy - laser light is used to destroy the tumor.
• Cryotherapy - freezing treatment to destroy the tumor.
• Chemotherapy - drug treatment given via the bloodstream to kill the cancer cells and sometimes utilized in conjunction with laser therapy
• Radiotherapy - high energy radiation to destroy cancer cells administered via external beam radiation or plaque therapy.
• Proton beam therapy - newer, developing treatment.
• Enucleation - removal of the eye.

The type of treatment depends on a number of factors including: unilateral vs bilateral, location of tumor, size of tumor and vision potential of the eye/eyes. Single treatment or a combination of treatments may be used. An operation to surgically remove just the tumor is not feasible since it would risk the spread of malignant cells outside of the eye. The eye MD and Oncologist determine and explain the available treatment options [See figures 1 and 2].

Should siblings be investigated for retinoblastoma?

All siblings and parents of children with retinoblastoma should have an eye examination by an Eye MD. The frequency of examinations depends upon age. All newborn babies of affected families need to be screened EARLY in life.

What resources are available to families affected by retinoblastoma?

The impact of the news and the treatment of the disease affects the entire family. There is much information from many professionals (Eye MD, Ocularist, Oncologist, etc) and this can be a challenging endeavor. Psychologists, social workers, orthoptists, geneticists and nurses may also aid in the process. Parent contact and support groups are often good resources. There are several useful websites that provide background information:

The Childhood Eye Cancer Trust
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The Retinoblastoma Online Support Group
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Retinoblastoma Survivors Support Group
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