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We reanalysed the current staging systems for retinoblastoma, the classification of retinoblastoma for prognosis by Reese and Ellsworth and the new Essen prognosis classification for conservative sight-saving therapy by univariant analysis. We compared the value of those classifications with one another.

The study is based upon a review of data from 583 consecutive patients with retinoblastoma over the years 1956 to 1986. Mean follow-up was 8 years, and median was 5.5 years. In 41 patients, metastases developed within 5 years. The influence of clinical and histopathologic risk factors on the occurrence of metastases was first analyzed by univariate tests. Significant variables were then reevaluated using the Cox proportional hazards method. Four factors were found to be independently associated with the development of metastases: optic nerve invasion with and without involvement of the resection line, choroidal invasion and enucleation of an affected eye more than 120 days after initial diagnosis. The 5-year metastatic risks associated with these factors were 67%, 13%, 8%, and 4%, respectively. The relative risk estimate, calculated from the Cox model, was used for a score classification with groups of low, medium, and high metastatic risk. The 5-year incidence of metastases was 4%, 43%, and 68%, respectively.

The retinoblastoma is the most frequent intraocular tumour in early childhood occurring in a hereditary as well as non-hereditary form and showing an increasing incidence since the beginning of this century, which has nowadays stabilized near 1/16,000. Further differentiation is necessary in view of unilateral and bilateral as well as sporadic and familiar occurrence. Estimates of the relative frequencies of the resulting different groups are given und summarized in two tables in two different aspects.

Radiogenic or non-radiogenic osteosarcomas were found in approximately 15% of the observed bilateral retinoblastoma cases, and only exceptionally in the unilateral cases. Such osteosarcomas are also seen in subjects without retinoblastoma, but belonging to affected families. Late second malignant extraocular tumours were only observed when we were dealing with a germinal mutation, that is in hereditary retinoblastoma. They are due to a pleiotropic effect of a single gene situated on the chromosome D13 (q14), or to two different but very close genes.

At present there is no satisfactory classification or staging system. In order to compare the Reese classification (Reese, 1963), the Essen classification (Höpping, 1983) and a new classification, a method of evaluating different parameters was created in order to get an optimal classification of survival.

At present there is no satisfactory classification or staging system. In order to compare the Reese classification (Reese & Ellsworth, 1963), the Essen classification (Höpping, 1983) and a new classification, a method of evaluating different parameters was created to get an optimal classification of globe saving treatment. A computer score table for the Essen eye clinic was established and this table could be valuable for all clinics using modern methods of treatment.