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 patientswith a retinoblastoma receiving primary treatment at our hospital over thelast 20 years.Methods: A retrospective series study of 30 retinoblastoma cases treated from January1, 1987 to August 31, 2006 was conducted from a review of medical recordsand histopathological sections. Variables, including age at onset, laterality,treatment modalities, treatment delay, and optic nerve invasion, were analyzedto elucidate the prognostic factors associated with cumulative survival.Results: Most of the cases had an advanced retinoblastoma, and 23 patients receivedenucleation treatment. The average period of delay for treatment was 5.37months after discovery of the disease. The overall cumulative survival ratewas 83.08%. Patients with optic nerve invasion had a significantly lower survivalrate (60.0%) than those without optic nerve involvement (94.75%).Treatment delay in excess of 6 months was correlated with tumor invasion ofthe optic nerve.Conclusions: Tumor invasion of the optic nerve is the most significant prognostic factorfor surviving a retinoblastoma. Delayed treatment increases the risk of opticnerve invasion. Parental awareness of both the risk of this consequence andthe significance of early treatment is vital to achieving improved survivalrates.