Notes to the EditorAbout Retinoblastoma1. Retinoblastoma (Rb) is a rare form of cancer which affects the retina of children, predominantly under the age of 5 years. 2. Statistically it affects 1:20,000 live births each year. This can also be expressed as between 40 and 50 cases a year or about one child a week. It represents 3% of childhood cancers in the UK. 3. Retinoblastoma affects children from all races and occurs in both genders equally. 4. There are many different treatments available in the UK and 9 out of 10 (95-98%) of children diagnosed will survive (5% will not survive). However in the developing world, it is still fatal. 5. In about 45% of cases, the condition is inheritable (with 55% non-inheritable). A person who has had this form of Rb has a 50% chance of passing it on. The retinoblastoma gene (Rb1) was the first inheritable cancer gene to be identified. 6. Rb can affect one (unilateral) or both eyes (bilateral) and there can be anything from 1 tumour in 1 eye to multiple tumours in both. 7. There is an even more rare form of Rb called Tri-lateral retinoblastoma which also affects the pineal gland. This form is usually fatal. 8. About 80% of children will have an eye removed (enucleation) because most cases of Rb are not picked up until the child is about two and half and the outward signs are visible to the naked eye. 85% of unilateral Rb patients will require enucleation and 5% of children currently have had double enucleation (both eyes removed). 9. The average age of children at diagnosis is 28 months (unilateral condition) and 9 months (bilateral condition) 10. The signs of Rb are: the appearance of a whitish light bouncing back out of the eye, like a “cats eye” caught in the light. It is often noticed in photographs where flash photography has been used. One eye will appear red, the other white. (note: there can be other reasons why this reflex occurs but it is ALWAYS best to get it checked out). a squint is sometimes present and the eye can look sore or swollen. 11. Children who have the inheritable form of Rb have a higher risk of developing other cancers later in life. 12. There are two specialist NHS treatment centres in the UK, The Royal London Hospital and Birmingham Children's Hospital. The service is overseen by the National Commissioning Service (NCS) which is part of the National Health Service. 13. Initial diagnosis can be made by examination using an ophthalmoscope but full diagnosis can only be confirmed by a specialist during an examination under anaesthetic (EUA). 14. There are no known causes for the occurrence of this cancer. |
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