Rb Fast Facts
Introduction to Rb
Global Incidence
Signs and Symptoms
Referral and Diagnosis
Staging Systems
Exam Under Anaesthetic
Treatment Options
Clinical Trials
Follow Up and Prognosis
RB1 Genetics
Glossary Of Terms
Retinoblastoma is caused by errors (mutations) on the RB1 gene. These errors may be inherited from a parent, or occur spontaneously during the baby’s early development.
Incidence
Retinoblastoma affects approximately 1 in 15,000 live births, and an estimated 8,400 - 9,000 children are newly affected each year around the world. Due to global population distribution, 92% of children with retinoblastoma live in developing countries.
Symptoms
The most easily recognisable early sign of retinoblastoma is an abnormal appearance of the pupil, which reflects light as a white reflex (leukocoria), like a cat's eye at night. In the early stages of retinoblastoma, this reflex is usually only visible in low artificial lighting or in photographs where a flash has been used. When the cancer fills the eye, this reflection may be visible to the naked eye in dim light.
the "cat's eye reflex".
Treatment will depend on the number, size and location of tumour(s). If both eyes are affected, each will be treated on its own merit. Current treatments include laser, cryotherapy (freezing the tumour), systemic and focal chemotherapy, brachytherapy (a radioactive plaque sewn directly onto the surface of the eye above the tumour), radiotherapy and surgical removal of the eye (enucleation). Prompt removal of the eye is essential throughout the world when there is a risk of cancer escaping the eye.
When retinoblastoma has spread from the eye to the socket, brain or other parts of the body, or when Trilateral Rb has been diagnosed, the child will require very intensive chemotherapy and bone marrow (stem cell) transplant to give the best chance of survival. Radiotherapy may also be used.
Only about 8% of children in the world have access to modern therapies. Most children have access only to eye-removal surgery as the costs of other treatments are very high.
Prognosis
Children do not die from tumours contained within the eye. More than 96% of children with retinoblastoma are cured today in developed countries, creating one of the best success stories of all childhood cancers.
However, without early intervention, the cancer quickly spreads beyond the eye, invading the brain or bone marrow. Even with intensive treatments available in developed countries, prognosis is very poor when cancer has spread extensively beyond the eye. In developing countries, minimal spread of cancer beyond the eye is frequently a death sentence due to poor access to specialist care. Globally, 7,000 children die every year from this highly curable cancer.
Trilateral Rb is extremely difficult to treat, and the majority of children die within 12-18 months of diagnosis. However, a slowly increasing number of survivors around the world is inspiring hope for children diagnosed with this pernicious form of retinoblastoma.
Five things to remember
1) Retinoblastoma is no one’s fault. It is not a curse and is not contagious.
2) Early signs of retinoblastoma are a "white pupil" or misalignment of the child’s eye(s),
3) Take regular flash photos of your child, with your camera’s red-eye reduction function turned OFF. Check photos for any unusual appearance of the eyes.
4) Ask the doctor to check your child’s eyes for normal red reflex at every well child visit, using an ophthalmoscope (or pen light if ophthalmoscope is not available). Ensure the doctor turns off the ceiling lights before performing the exam. Ask for a referral to an ophthalmologist for a dilated eye exam if you observe white pupil in your child's eye/s.
5) Retinoblastoma is highly curable when diagnosed early and treated correctly by specialists familiar with its management.
Prompt treatment is essential to protect the child's life.
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