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 tumours contained inside the eye(s) are usually staged using one of the following three systems.
The Reese-Ellsworth Classification (grouped 1-5) was developed in the 1960s by Dr. Algernon Reese and Dr. Robert Ellsworth, two prominent New York physicians specialising in retinoblastoma. It predicts outcome from treatment with external beam radiotherapy (EBRT), which was the primary vision-salvage therapy at the time of its creation.
Today, chemotherapy Is the primary eye salvage treatment, while radiation is reserved by most specialists as an absolute last resort to save a second eye, where all other treatment options have failed or are unavailable. As a result, the REC has become increasingly obsolete and is now rarely used.
The International Intraocular Retinoblastoma Classification (grouped A to E) was developed by Dr. Linn Murphree, paediatric ocular oncologist at Children’s Hospital of Los Angeles. This system is more relevant to modern treatment techniques as it is designed to predict outcome from treatment with combination chemotherapy and focal therapy. However, it is difficult to enforce internationally and has been open to manipulation by researchers.
The TNM classification for retinoblastoma was redeveloped in 2010 to incorporate the International Intraocular Retinoblastoma Classification which has been favoured by physicians worldwide in staging tumour within the eye.
The system includes both clinical (cTNM) and pathological (pTNM) findings. Primary retinoblastoma (T) is divided into four groups, with several sub-groups in each, and many additional descriptors. The stages from cT1a – cT3b closely reflect the five stages of the IIRC. Stage cT3 (IIRC Group E) indicates high risk for invasion of tissues surrounding the eye, and the need for urgent removal of the eye. Stage pT2 and pT3 confirm invasion of surrounding tissues. Stages cT4, pT4 N and M involve significant disease invasion of tissues beyond the eye.
When both eyes are affected, each eye is staged independently. When only one stage is stated in patient reports for a bilateral child, this refers to the stage of the worst eye, as an indicator of risk to the child’s life.
From June 2010, researchers are required to state TNM classification alongside or in place of IIRC / REC in manuscripts submitted for journal publication or conference presentation.
Broadly speaking, the following can be used as a guide.
• IIRC Groups A - C / TNM T1a - T1c: retinoblastoma is likely to be well controlled with modern therapies, whilst also preserving vision in the eye.
• IIRC Group D / TNM T2 retinoblastoma is unlikely to be controlled, and chances for preservation of the eye are poor.
• IIRC Group E / TNM T3 retinoblastoma poses a serious threat to life and immediate surgery to remove the eye is indicated to protect the child.
So long as cancer is contained within the eye and treated appropriately, and both eyes receive appropriate follow up, the child’s life will be protected.
As other staging systems may be used, remember to ask you child’s doctor which system is being used, and what your child’s stage means.
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