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
When a child has unilateral retinoblastoma with enucleation and no disease spread beyond the eye, and advanced molecular genetic testing predicts risk for presence of a non-detectable RB1 mutation to be less than 1%, the child can be followed with eye examinations in clinic. No oncology clinic follow-up is necessary for these children if they did not receive chemotherapy or radiotherapy, since their risk for other cancers is similar to that of the normal population.
Clinic based retinal exam cannot fully assess the peripheral retina. As new tumours develop on the periphery of the retina, any tumour would be larger at diagnosis than if the eye had been examined under anaesthesia. However, in this group of children the risks associated with general anaesthesia far outweigh the less than 1% of a tumour being missed on clinic examination.
Follow up for heritable retinoblastoma
When a child has bilateral Rb or is at risk of developing bilateral Rb (due to family history, identified RB1 mutation in blood, unilateral multifocal diagnosis or young age at diagnosis), both eyes should be closely monitored beyond active treatment. Initially, EUAs should be carried out every 3-4 weeks for at least three months after the last tumour activity, then gradually reducing in frequency over the next three years. 6-12 monthly retinal exams in clinic should continue until age 10 years, then every 2 to 3 years for life.
Carriers of a heritable RB1 mutation should attend a paediatric oncology clinic annually until 18 years of age, then every 1-2 years for the rest of their life at an childhood cancer follow-up clinic or adult oncology clinic. They should also receive information regarding their lifelong risk of developing second primary cancers, and should be given opportunities to discuss this with doctors who specialise in retinoblastoma.
As diagnostic radiation can increase the risk of second malignancies in this group of people, CT scans, bone scans and other x-rays should be avoided in the process of follow-up care.
Follow up when there is high risk of extraocular retinoblastoma
When post-operative pathological examination of eyes or clinical signs suggests risk for spread of retinoblastoma to the orbit, bone marrow or other parts of the body, children should undergo bone marrow aspirate and MRI of the head and orbits every 3-4 months for five years.
When there is high risk for spread of the cancer to the optic nerve or cerebrospinal fluid, the child should undergo lumbar puncture and MRI of the head, orbits and spine every 3 to 6 months for 5 years.
Both groups of children should undergo whole body MRI if this is available, and they should attend an oncology clinic annally for the rest of their life.
Follow up after chemotherapy or radiotherapy
Any child treated with chemotherapy or external beam radiotherapy should also be followed by the treating oncology clinic every 3-6 months for 3 years after the end of treatment, then annually until 18 years old, and thereafter every 1-2 years for the rest of their life at an childhood cancer follow-up clinic or adult oncology clinic.
Follow up when an artificial eye is warn
If the child has an artificial eye, the eye socket and fitting of the eye should be monitored with 6-12 monthly visits to the ocularist. Tthe artificial eye may be adjusted or replaced as necessary.
If follow up is not possible
In many countries around the world, genetic testing and MRI are not available, and follow-up oncology clinics do not exist. If follow-up is not possible, it is vital that medical advice be sought immediately for unexplained health concerns. Consultation with the team who treated the retinoblastoma is preferable.
For all survivors of retinoblastoma and carriers of an RB1 mutation
When the child is old enough to understand, genetic counselling should be offered to help work through the genetic issues relating to retinoblastoma, and to counsel the person on their risk for second primary tumours.
Prognosis
97% of children with retinoblastoma are cured today in developed countries such as Canada and the UK. This is one of the highest cure rates, and best success stories of all childhood cancers in the developed world..
However, without early intervention, this cancer will quickly spread beyond the eye, travelling directly to the brain, or invading the bone marrow. Tumours which have spread to the brain or bone marrow have a very poor prognosis, even with the most advanced treatment protocols. Untreated retinoblastoma is universally fatal,
Globally, less than 10% of children with retinoblastoma are able to access essential treatment. More than 80% of children around the world die due to lack of appropriate and timely medical care.
Trilateral retinoblastoma 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 are inspiring hope for future children diagnosed with this particularly pernicious form of retinoblastoma.
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