Childhood Cancer Facts

"Retinoblastoma" (Malignant Tumor of the Retina)

Incidence:

• An estimated 200 to 300 children per year will develop retinoblastoma. It represents about 2% of childhood malignancies. 80% of cases are often diagnosed before the age of 5 years.

Survival Rate:

• Intraocular (tumor is contained within the eye) - 90%
• Extraocular (tumor has extended beyond the eye) - Less than 10%

Causes:

• Retinoblastoma is a tumor that occurs in hereditary (40%) and nonhereditary (60%) forms. Patients with the hereditary type of retinoblastoma have an increased frequency of additional malignancies.

Description:

• Retinoblastoma is a malignant tumor of the retina. Patients may be born with the disease, but it is rarely diagnosed at birth. The presentation and treatment course for patients older than 5 years may differ from those of younger patients.

Current Treatment Options:

• Treatment depends on whether the tumor effects one eye (unilateral) or both eyes (bilateral) and if the disease has spread to other parts of the body. Surgery, radiation therapy or cryotherapy could be used as treatment. Because this tumor can be genetically inherited disease, genetic counseling is usually an integral part of therapy. All siblings of patients with retinoblastoma should be examined periodically.
• Clinical trials are testing new chemotherapy drugs in attempt shrink the tumor and avoid radiation therapy or having to surgically remove the eye.