Clinical management of retinoblastoma.

Abstract

There have been many recent advances in the diagnosis and management of children with retinoblastoma. Regarding diagnosis, the judicial use of ancillary studies, particularly B-scan ultrasonography, has been instrumental in supplementing the diagnosis when opaque media preclude a clear view of the underlying neoplasm. Computed tomography and magnetic resonance imaging are important in the early detection of associated pinealoblastoma, which occurs as part of the recently described syndrome of "trilateral retinoblastoma." With regard to management, the technique of enucleation continues to improve and the scleralized hydroxyapatite implant is now available to provide a better cosmetic appearance and better motility of the artificial eye. Methods have been described for harvesting fresh tumor tissue immediately after enucleation for special DNA studies. Recent reports have documented a decreasing frequency of enucleation and an increasing tendency to use eye-preserving methods of management. Plaque radiotherapy, which provides more localized irradiation to the specific tumor, is gradually supplanting external beam irradiation for localized retinoblastoma that does not exhibit extensive vitreous seeding. The indications and contraindications for cryotherapy and photocoagulation have been more clearly delineated. The role of chemotherapy is being evaluated in the treatment of primary retinoblastoma and for metastatic disease. New breakthroughs in genetic research are being applied to clinical genetic counseling. The prognosis for life and for preservation of vision has improved greatly in recent years.