Current management of retinoblastoma

The recommended management of retinoblastoma based on personal experience with the assessment and treatment of more than 450 children with this intraocular malignant lesion is presented.

Although retinoblastoma is usually managed by enucleation, the treatment of each case must be individualized; in an increasing number of children, the techniques of irradiation, photocoagulation, or cryotherapy are being used. In some patients, a combination of these techniques is necessary. Chemotherapy is often used to prevent distant metastatic involvement, although its effectiveness as a prophylactic treatment has not been clearly established. Because metastatic retinoblastoma is often fatal, intense chemotherapy is recommended in this setting.

The prognosis for vision and life in patients with retinoblastoma has improved considerably during the past century, primarily because of earlier recognition of the tumor and use of modern therapeutic methods. All children of parents with a family history of retinoblastoma should be examined by a qualified ophthalmologist immediately after birth in order to detect and treat this condition as early as possible.