Review of 20 years' clinical experience with retinoblastomas in southern Taiwan.

BACKGROUND Retinoblastomas are the most common malignant intraocular tumor of childhood. We describe the survival outcomes and prognostic factors of patients with a retinoblastoma receiving primary treatment at our hospital over the last 20 years. METHODS A retrospective series study of 30 retinoblastoma cases treated from January 1, 1987 to August 31, 2006 was conducted from a review of medical records and histopathological sections. Variables, including age at onset, laterality, treatment modalities, treatment delay, and optic nerve invasion, were analyzed to elucidate the prognostic factors associated with cumulative survival. RESULTS Most of the cases had an advanced retinoblastoma, and 23 patients received enucleation treatment. The average period of delay for treatment was 5.37 months after discovery of the disease. The overall cumulative survival rate was 83.08%. Patients with optic nerve invasion had a significantly lower survival rate (60.0%) than those without optic nerve involvement (94.75%). Treatment delay in excess of 6 months was correlated with tumor invasion of the optic nerve. CONCLUSIONS Tumor invasion of the optic nerve is the most significant prognostic factor for surviving a retinoblastoma. Delayed treatment increases the risk of optic nerve invasion. Parental awareness of both the risk of this consequence and the significance of early treatment is vital to achieving improved survival rates.