| The following provides a short overview of RB. For extended explanations, written by medical professionals, please click on the links at the end of this page Retinoblastoma is a fast-growing eye cancer of early childhood. Tumour(s) typically develop before the age of 5 years and some children are born with retinoblastoma. In approximately two thirds of children with this cancer, only one eye is affected (unilateral Rb), but in one third, tumours develop in both eyes (bilateral Rb). Very rarely, a tumour may develop in an area of the mid-brain called the pineal gland. This tumour is called pineoblastoma, or trilateral rb and is not metastatic rb, but an entirely independent tumour growth seen predominantly in children with retinoblastoma. There are two forms of retinoblastoma: sporadic, and genetic. Chromosomes contain all genetic codes which govern the behaviour and development of cells throughout the body. Abnormal cell growth can occur if a segment of a chromosome is missing or damaged. In about 40% of Rb children, a small piece of the genetic material on chromosome 13 is damaged, causing the development of retinoblastoma. Some children inherit the genetic defect from one a parent, but in the majority of cases, the abnormality appears to be a spontaneous mutation. Childhood cancer is uncommon, and within this group, retinoblastoma itself is rare, accounting for less than 3 out of every 100 cancer diagnoses in children under the 15 years of age. Between 40-50 children are newly diagnosed with retinoblastoma each year In the UK, with approximately 300-350 cases newly diagnosed in the USA. Retinoblastoma occurence is significantly higher in many tropical and sub-tropical countries throughout the world, but the reasons for this trend are unclear. There are two common signs of retinoblastoma to look out for: A squint or crossed eyes may be a symptom of Rb , but the most easily recognised symptom is an abnormal appearance of the pupil which reflects light as a white reflex (leukocoria), like a cat's eye. This is usually only visible in low artificial lighting or in photographs where a flash has been used, such as in the example below. Click on the image for an in depth explanation of leukocoria. If you see a white reflex in your child's photoraph, compare it to other photographs in the same set and, if possible, ask friends/family to examine their own photos taken on the same occasion to see if more photos have the reflex. Look at previous photos of your child too. If the reflex appears in a single picture or in several pictures taken from the same position on the same real, whilst all other photos show normal red reflex, the reflex is likely to be the result of the angle of the flash light. Less common symptoms include: deteriorating vision red and inflamed eye. pain. a dilated pupil, different coloured irises, failure to thrive or delayed development. Current treatments for retinoblastoma include chemotherapy (systematic and localised drug therapy), radiotherapy, brachytherapy (radioactive plaque sewn directly onto the tumour), laser, cryotherapy (freezing the tumour) and enucleation (surgical removal of the eye). Children do not die from primary retinoblastoma, but from metastatic Rb - cancer which has spread beyond the eye. This is entirely preventable. Due to the eye's proximity to the brain, and the rapid growth of retinoblastoma cells, early detection and treatment of this cancer is essential. Spread of Rb beyond the eye (extra-ocular or metastatic Rb) Is critical and almost always fatal, as is trilateral Rb. Despite this, more than nine out of ten children with retinoblastoma can be cured today, creating one of the highest cure rates, and best success stories of all childhood cancers. Children with the genetic form of retinoblastoma have a greater risk of developing other cancers later in life, particularly osteogenic sarcoma (bone cancer), brain tumours (including pineoblastoma), soft tissue sarcomas and cutaneous melanomas (tumours of the muscle, connective tissue and skin). For this reason, regular oncology follow up is essential for this group of survivors. Retinoblastoma is a potentially life threatening cancer, but it can be successfully treated, increasingly also preserving much vision in the process. The majority of survivors grow up to become very successful people (several studies have even consistently linked high IQ with retinoblastoma. Famous rb survivors include Peter Faulk (actor: Columbo) and Caroline Ahern (English comedienne). Check all children's photographs thoroughly, especially those taken in low light conditions. Retinoblastoma specialists recommended that a child's picture be taken once a month in a dimly lit room for the purpose of checking for a normal red-eye reflex. It is essential for the child to be seen immediately by a paediatric ophthalmologist (a children's eye doctor) if you ever find a white reflex, such as in the photograph above. Ensure your child's eyes are examined with an ophthalmoscope in a darkened room at every well child visit. This simply involves shining the ophthalmoscope in the child's eyes. If a white reflection is seen in the pupil of the eye, it suggests that something is blocking light from reaching the retina at the back of the eye. Although there are a few other conditions which cause this symptom (such as Coat's Disease and PHPV), retinoblastoma is the most likely diagnosis in young children, and should be examined as a matter of urgency. Correct monitoring at regular paediatric exams should ensure that Rb never remains undetected. Ask for a dilated eye examination for infants. The diameter of an infant's pupil is very small, so dilating drops are necessary in order for the doctor to see the retina clearly enough to detect tumours and other potential problems. Drops are put into both eyes at the start of the exam. By the end of the exam, the eyes are dilated and the standard ophthalmoscope check can be done. Approximately one in 680 children are born with a treatable eye condition which has the potential to blind if not detected and treated. Cataracts are ten times more prevalent in infants than retinoblastoma and can cause blindness by two months of age without treatment. Only premature babies and those with a known hereditary risk for ocular diseases are automatically given dilated eye exams in the UK and USA to date, so remember to ASK for the test. Early detection and referral can save a child's sight and, in the case of retinoblastoma, their life. To learn more about retinoblastoma, please visit one of the following sites. Formerly known as the UK Retinoblastoma Society, the UK The Childhood Eye Cancer Trust is a national charity working to support families and individuals affected by this rare eye cancer. The trust provides support and information, funds research and raises public awareness. Dr David Abramson is the Director of the Robert M. Ellsworth Ophthalmic Oncology Centre at New York Presbyterian Hospital in New York City, and one of the foremost experts on retinoblastoma in the Eastern USA. Use the links in the ORbIT section to learn more about the different aspects of retinoblastoma which have been touched on above. Return to the ORbIT home page. |
| "Do not go where the path may lead, go instead where there is no path - and leave a trail." ~ Ralph Waldo Emerson ~ |
| So What Is Retinoblastoma? |
| ORPHANS OF THE CANCER STORM |
| Disclaimer This information page has been prepared by an individual who does not have medical training. However its contents have been reviewed by appropriately qualified medical professionals for accuracy and quality. This page is intended for information purposes only and should not be used as a guide to diagnosis or treatment. If this information raises any concerns about your child’s condition or care protocol, discuss them with his or her paediatrician, oncologist, or other appropriate medical professional who has knowledge of the case. |
 |
| The white reflex is not necessarily a sign of retinoblastoma. It is also a symptom of several other eye conditions, such as Coat's Disease or a blond fundus. However, in young children, it should be considered the most likely diagnosis, and investigated promptly with a dilated eye examination. Flash photography may produce the white reflex to appear in perfectly healthy eyes. This depends on the angle at which the light hits the eye. |