This is the most common intraocular cancer in children, with an estimated 400 - 500 cases in the U.S each year. It is extremely rare in adults.
There are two types of retinoblastoma; one is hereditary and affects both eyes (occurs in 10 percent of cases) and the other type is non-hereditary and affects only one eye. Although the cancer is genetically determined, only 6 percent of newly diagnosed RB patients are found to have a positive family history of the disease. In all cases, genetic counseling is important for children with a germ-line mutation.
Retinoblastoma is graded A through E, with A being the least advanced form of the disease. Because of recent advances, group A can now be treated with a laser or cryotherapy. Groups B through D traditionally received systemic chemotherapy, which can affect other parts of the body. Class D had almost a 50 percent failure rate with systemic chemotherapy. Class E patients needed to have the affected eye removed.
Working together, Sylvester Comprehensive Cancer Center and Bascom Palmer Eye Institute, both part of the University of Miami Health System, provide high quality care for retinoblastoma. Patients travel from great distances because Sylvester is the only facility in the southeastern U.S. and one of only a few in the country that is highly skilled at treating retinoblastoma with intra-arterial chemotherapy.
Tests
Retinal Examination - Using an ophthalmoscope, the doctor will exam the retina while the eyes are dilated.
Optical Coherence Tomography (OCT) - This imaging test creates a cross-sectional, three-dimensional (3-D) view of the inside of the eyes.
Ultrasound - This safe, painless test uses high-frequency sound waves to produce detailed images of the inside of the eyes and eye socket.
Fluorescein Angiography - Using this method, your doctor can see changes in the structure or function of the retinal blood vessels. To perform the test, the doctor injects dye into a vein in the arm or hand, then photographs the retina as the dye outlines the blood vessels.
Electroretinogram - An electroretinography (ERG) test or electroretinogram, measures the electrical response of the light-sensitive cells in your eyes.
Bone scan - Bone scans help show if retinoblastoma has spread into other bones or the skull.
Lumbar puncture (spinal tap) - If retinoblastomas grow on the optic nerve, and if there is a suspected cancer on the brain’s surface, this test can find cancer cells. We analyze a small sample of cerebrospinal fluid (which surrounds the brain and spinal cord) under a powerful microscope.
Bone marrow aspiration and biopsy - These tests are typically only used when we suspect the retinoblastoma is growing outside the eye and that cancer is spreading through the blood and into the bone marrow.
Treatments
Intra-arterial Chemotherapy - With localized intra-arterial chemotherapy, young patients with C-E graded retinoblastomas are spared the long-term effects of systemic chemotherapy. Also, the need to remove the eye drops by 50 percent.
With this chemotherapy, a thin catheter is threaded into the femoral artery in the groin, up into the carotid artery in the neck and into the ophthalmic artery. The chemotherapy is then delivered into the ophthalmic artery. Some patients need two or three treatments three weeks apart. Only about five percent of patients fail to respond to this treatment. When they do, it is usually because of cancer cell spreading into the vitreous, the gel inside the eye. An ophthalmic oncologist can directly inject chemotherapy into the eye to address those cells, without the chemotherapy affecting other parts of the body.
Cryotherapy - This therapy uses cold to freeze and destroy cancer cells and can also be used with retinoblastoma.
Why Choose Bascom Palmer Eye Institute?
National-Leading Partnership with Bascom Palmer. No other South Florida cancer center offers the knowledge and experience we do. You receive treatment from ocular physician-experts at one of the nation’s top academic cancer centers, plus support and/or care from our team at Bascom Palmer Eye Institute, the nation’s #1 eye hospital.
Multidisciplinary Care Teams. Your cancer is evaluated and treated by a team of experts. They represent several specialties: ophthalmology, dermatology, ocular oncology, radiation oncology, and pathology. It adds up to personalized, well-rounded care focused on creating an optimal quality of life.
More Targeted Radiation Therapy. More eyes saved. We are the only center in South Florida offering localized internal plaque radiation therapy. This treatment treats the cancer without removing the eye, preserving vision, independence, and quality of life.
Leading-Edge Intra-Arterial Chemotherapy for Children with Retinoblastoma. Your child has twice the chance his or her eye will be spared. Using intra-arterial chemotherapy, we target chemotherapy directly to the eye tumor. This curative treatment has decreased the number of eye removals for retinoblastoma by half. It is also easier on developing bodies. Sylvester is the only center in the Southeastern U.S. and one of a few in the country that is highly skilled at this treatment.
Top-Ranked Eye Prosthesis (Artificial Replacement) Options. More confidence and better lifelong health after cancer. If your eyeball must be removed to cure your cancer, a prosthetic eye improves your appearance and protects the space and cells behind your eye. We custom design your prosthesis to match the iris and pupil of your other eye.
A Center with a History of “Firsts”. You can be cured. We were the first center in the country to use topical interferon drops to treat eye cancer – without biopsy or surgery. This and many other research advances give you the best possible cancer and eye care for ocular cancers.
Safer, More Accurate Diagnosis. Sylvester and Bascom Palmer offer ultra-high frequency OCT (optical coherence tomography). This optical ultrasound diagnoses melanomas and inner eyelid (conjunctival) tumors without biopsy or radiation exposure.