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Staging Systems

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Once retinoblastoma has been diagnosed, it is staged to help determine which treatment is most appropriate.  The stage of the disease means the size, number and location of tumours, and whether the cancer has spread beyond the eye.  If both eyes are affected, each eye is staged independently of the other.

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Staging of retinoblastoma helps doctors to summarise the information they have gained from various diagnostic tests and examinations and identify appropriate treatments.  Staging also helps to predict how effective different treatments are likely to be, how much vision may be preserved and the child’s chances of survival. Most cancers staged using the international TNM (Tumour, Node, Metastesis) system.  This divides the cancer into four groups: small, localised growth (stage 1); invasion of surrounding tissues (stages 2 and 3); or spread to other parts of the body (stage 4). Stage four cancer may also be referred to as secondary or metastatic cancer. Retinoblastoma tumours contained inside the eye(s) are usually staged using one of two systems.  The Reese-Ellsworth system (grouped 1-5) was developed in the 1960s to predict outcome from treatment with external beam radiotherapy (EBRT).  This system is is still widely used in developing countries, where radiotherapy is often more readily available than newer chemotherapy treatments. The International Intraocular Retinoblastoma Classification (grouped A to E) was developed to predict outcome from treatment with combination chemotherapy and focal therapy.  Developed by Dr. Linn Murphree, paediatric ocular oncologist at Children’s Hospital of Los Angeles, this simple system is more relevant to modern treatment techniques, and has become the standard staging system in developed countries.

Ranging from 1 or A (lowest stage) to 5 or E (highest stage), the higher the stage within both systems, the lower the chances of controlling tumours, and thus preserving the eye with any useful vision.  Group 1/A retinoblastoma is likely to be well controlled with modern focal therapies, whilst also preserving vision in the eye. Group 5/E retinoblastoma is unlikely to be controlled, and chances for preservation of the eye are poor.  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. Regardless of the stage of intraocular retinoblastoma, so long as the cancer is contained within the eye and treated appropriately, it can be cured with prompt treatment. Back to Top

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