Resources and information for parents of children with cancer . . . by parents of children with cancer.

this page last modified

Retinoblastoma

Note: This section has health/medical information. It was not written by a health care professional. The medical references are:

Recommended: NCI PDQ for retinoblastoma
On this site: Warning Signs of Childhood Cancer: Retinoblastoma

Retinoblastoma is a malignancy of the retinal cell layer of the eye. The tumor is composed mainly of undifferentiated anaplastic cells -- blasts -- that arise from the nuclear layers of the retina. It is the most common eye tumor in children and it usually occurs before the age of five. It can occur in one eye (unilateral) or in both eyes (bilateral).

About Retinoblastoma

In about 40% of the cases, retinoblastoma is hereditary. In fact, retinoblastoma was one of the first cancers recognized as "hereditary". Before surgery, children who had retinoblastoma died before they reached childbearing age. When doctors in the first part of this century began removing the affected eye to treat the disease, the children survived the cancer and lived to have their own children, some of whom in turn developed retinoblastoma. In the 1970s, researchers studied the DNA of retinoblastoma carriers and found one of the first "oncogenes". They found that deletions or mutations of the "retinoblastoma" gene within the q14 band of chromosome 13 are highly associated with a predisposition to retinoblastoma.

Several sites already exist on the Internet that describe retinoblastoma (links just below). On the Digital Journal of Ophthalmology, there are pictures of what the disease looks like.

Patients with retinoblastoma, particularly the hereditary type, have an increased frequency of second malignancies. These cancers are most often bone tumors, and they occur in up to 8% of retinoblastoma patients after 18 years of follow-up.

Treatment

Both the treatment and prognosis for retinoblastoma depend on the spread, or stage, of the tumor.

Retinoblastoma is treated by surgery (enucleation), chemotherapy, cryotherapy, light coagulation, and radiation. The eye is spared whenever possible. Current statistics state a 80-90% 5 year survival rate.

Statistics

Ped-Onc Resources for Retinoblastoma

The following ped-onc resource lists have appropriate sections for parents of children with retinoblastoma:

Links to More Information

The following web sites provide good, general information on retinoblastoma.

General Disclaimer

These pages are intended for informational purposes only and are not intended to render medical advice. The information provided on Ped Onc Resource Center should not be used for diagnosing or treating a health problem or a disease. It is not a substitute for professional care. If you suspect your child has a health problem, you should consult your health care provider.

about this site | privacy statement | terms of use | contact