PURPOSE: The management of retinoblastoma (Rb) has gradually changed over the past years achieving a satisfactory result (increasing overall survival and avoiding enucleations). METHODS: Three hundred and nineteen patients with retinoblastoma were studied. From January 1986 to November 1996, 257 patients with Rb were referred to "Hospital do Cancer AC Camargo". The treatment changed according to the period, from 1986 to 1990 and from 1991 to 1996. From January 1996 to December 2000, 62 patients presenting intraocular (IO) tumors were treated with chemoreduction and adjuvant treatment to avoid enucleation or external beam radiation therapy. RESULTS: From January 1996 to December 2000, 62 patients with IO Rb (47 bilateral and 15 unilateral tumors) with potential vision were treated with chemoreduction and ophthalmological adjuvant treatment. In the patients with bilateral tumors 49.8% preserved the eye vs. 26.7% for patients with unilateral tumors. Among 257 patients 59 presented extraocular Rb according to CCG classification. Overall five-year survival was improved in the second period (63.7 x 41% - p=0.059). CONCLUSION: Chemorreduction plus focal treatment plays an important role in the management of children with Rb. Gene therapy perhaps will increase the number of preserved eyes. A multicenter trial should be considered to evaluate patients with CNS Rb or metastatic disease with ominous prognosis. The lag time between the first sign and symptoms and the early diagnosis is the best weapon to achieve the cure in this patients.
Retinoblastoma; Survival; Vision
