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 two systems.
The Reese-Ellsworth Classification (grouped 1-5) was developed in the 1960s by 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.
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 combination chemotherapy and focal therapy.
Group 5/E retinoblastoma is unlikely to be controlled, and chances for preservation of the eye are poor.
The criteria for classifying eyes are very different in the two systems, and lead to different interpretations of how salvageable an eye may be. For example, an eye classified as 5b by the REC may well be classified as a D eye under the IIRC.
The IIRC has now largely replaced the REC as chemotherapy is now the treatment of choice to save eyes. 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.
Regardless of the stage of intraocular retinoblastoma, so long as the cancer is contained within the eye and treated appropriately, and both eyes receive appropriate follow up, the child’s life will be protected.
Within the context of the IIRC, E stands for enucleation (surgical removal of the eye). This is because E eyes pose a high risk for tumour spread beyond the eye, and therefore a threat to the life of the child.
When cancer has spread beyond the eye, it is staged using the TNM (Tumour, Node, Metastesis) classification. Groups T1 and T2 stage cancer contained in the eye, but the IIRC or REC are favoured globally for staging of cancer confined to the eye, as they are better predictors of treatment outcome.
The TNM classification covers both clinical (cTNM) and pathological (pTNM) staging. The system divides primary retinoblastoma (T) into four groups, with several sub-groups in each, and many additional descriptors. Stages cT1 and cT2 involve tumour contained in the eye, while stage cT3 and pT2 and pT3 involve invasion of surrounding tissues. Stages cT4, pT4 N and M involve major spread beyond the eye.
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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