Diagnosis and treatment of intraocular tumours in children

Purpose: To remind of the absolute necessity for early diagnosis in the pre sence of ocular signs in children giving rise to possible intraocular tumou rs. Method: Based on our own experience of intraocular tumours in children, tog ether with findings from the literature, diagnostic criteria and methods of treatment are presented. Results: Retinoblastoma is the predominant cause of intraocular tumours in children, representing over 80 % of cases under the age of 15 years. Other diseases may give rise to the same initial signs, usually leukocoria, somet imes strabismus, more rarely other atypical signs. Elements taken into acco unt for diagnosis include age, sex, laterality, heredity, size of the globe , clinical aspect of the tumours, presence of calcifications and vitreous s eeding. Full fundus examination under general anaesthetic is usually necess ary. Biological examination, ultrasonography, computerized tomography and M RI enable an accurate diagnosis to be made in the majority of doubtful case s. The management of retinoblastoma is adapted for each individual case fro m the wide range of treatments available. Enucleation, radioactive applicat ors (...), brachytherapy (...), cryo- and photocoagulation represent classi cal measures. Primary chemotherapy, combined with other treatments such as thermotherapy, has become the treatment of choice in those cases where exte rnal beam radiotherapy has been used up to now, or in some instances before enucleation. Enucleation is usually carried out for medullo-epitheliomas, but brachytherapy may offer an alternative. Conclusion: Any unexplained ocular sign in children should be considered as a possible retinoblastoma, making an accurate and certain diagnosis impera tive. Early treatment may save not only the life but also the vision of pat ients carrying this highly malignant lesion.