title: [Retinoblastoma].
abstract: Retinoblastoma is the most frequent malignant tumour of the eye in the child; the median age at diagnosis is two years. A model of carcinogenesis by two successive mutations has been devised for this tumour and explains the two forms of the disease, hereditary and non-hereditary. It is most often manifested by a whitish pupillary reflection (leukocoria) or internal strabismus. Funduscopy using general anaesthesia is used to confirm diagnosis and assess endo-ocular extension. When the disease is confined to the retina, local treatment (enucleation, radiation therapy) leads to cure in a very large majority of cases. When diagnosis is made early, particularly as in familial cases, conservative treatment is possible. In intra- and extra-retinal disease, chemotherapy and radiation therapy can improve prognosis.
authors.affiliation: Pédiatrie oncologique, Institut Curie, Paris.
authors.forename: J M
authors.initials: JM
authors.lastname: Zucker
mesh_terms: D002675:Child, Preschool; D005134:Eye Neoplasms / Q000175:diagnosis / Q000453:epidemiology / Q000628:therapy; D006801:Humans; D007223:Infant; D012175:Retinoblastoma / Q000175:diagnosis / Q000453:epidemiology / Q000628:therapy; D013997:Time Factors
vernacular_title: Réinoblastome.