Retinoblastoma is a disease in which malignant (cancer) cells form in the tissues of the retina, the light-sensitive layers of nerve tissue at the back of the eye. If left untreated, retinoblastoma is a potentially blinding and fatal eye cancer. Although it is relatively rare, it is the most common malignant eye tumor in children.
Retinoblastoma generally develops in children from birth to 5 years of age. It can run in families or occur spontaneously, and may involve one or both eyes. It occurs in both genders and in all races. If left untreated, the tumor can spread outside the eye to other organs and cause death.
The cause of the disorder is a mutation in one or more genes in a cell in the retina, which senses light and sends images to the brain by way of the optic nerve. There is no food, infection, vaccine or other environmental risk factor that has been shown to cause the disease.
Retinoblastoma causes a white papillary reflex, called leukocoria in the eye. This may be seen in person or on a flash photograph, when the pupil of the eye appears white instead of red. All children who have this symptom should be checked by a pediatric ophthalmologist and/or an ocular oncologist. Some children have pain or redness in the eye, or develop in-turning or out-turning of the eye called strabismus or lazy eye.
Children with retinoblastoma undergo a physical exam and history and special eye examinations and imaging procedures including photographs, ultrasound, MRIs and other studies. Many of these imaging tests are performed while children are under anesthesia in the operating room.
Because there are only 250 to 350 new cases of retinoblastoma per year in the United States and about 8,000 new cases per year in the world, very few specialists are trained to treat the condition. Treatments include chemotherapy – intra-arterial, intravitreal, systemic/intravenous or periocular. Other treatments include laser, cryotherapy, radiation or surgical removal of the eye.
Children’s Memorial Hermann Hospital is the only hospital in the south-central United States offering intra-arterial chemotherapy for the treatment of retinoblastoma. The treatment requires a large multispecialty team that involves close collaboration between an ocular oncologist, an endovascular neurosurgeon and a medical neuro-oncologist.
Injecting chemotherapy directly into the arteries that feed the eye eliminates the side effects of systemic chemotherapy and allows for the delivery of a high concentration of the medication directly into the tumor bed. The technique, which saves eyes, is new and a paradigm shift in the treatment of retinoblastoma.
Physicians affiliated with Children’s Memorial Hermann Hospital and the Mischer Neuroscience Institute are also engaged in research investigating other new ways to save eyes that have failed conventional therapies.
Some children with retinoblastoma are at risk for developing other cancers throughout their lifetime, such as osteogenic sarcomas, soft-tissue sarcomas, brain tumors, skin melanomas and others. Lifelong follow-up with an ocular oncologist as well as other doctors are important for retinoblastoma patients.
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