Treatment and prognostic analysis of retinoblastoma patients with choroid invasion

Objective To observe the treatment and prognosis of choroid invasion of retinoblastoma (RB) in children. Method A total of 149 children who had been diagnosed with unilateral RB and received enucleation disclosing tumor invasion to choroid from January 2006 to December 2013 in Beijing Tongren Hospital were recruited in this study. Choroid involvement was classified as massive choroid invasion and focal choroid invasion. Massive choroid invasion was defined as a maximum diameter of invasive tumor focus of 3 mm or more in diameter that might reach the scleral tissue. Focal choroid invasion was defined as a tumor focus of less than 3 mm in diameter without involvement of sclera. The treatment was delivered according to the invasive status of tumor with combination of histopathological high risk factors. The prognosis of different degrees of choroid invasion was observed. They were divided into two groups according to whether the merger of other high histopathologic risk factors, the survival situation was compared. The subjects were followed up for 1 to 9 years (the median follow-up time: 4 years and 1 month). Result Among the 149 subjects, 90 were boys and 59 were girls. The right eye was affected in 81 patients and the left eye in 68 patients. Sixteen patients died, resulting in an overall survival rate of 89.3%. Among massive choroid invasion in 47 cases, 9 patients experienced disease recurrence and death resulting in a survival rate of 80.9%. While the focal choroid invasion was found in 102 cases, only 7 children had disease relapsed and died resulting in a survival rate of 93.1% which was statistically significant (χ2=5.067, P=0.024). Among 8 patients with massive choroid invasion without pathological high-risk factors, no death occurred, while in other 39 patients with high-risk factors, 9 died with a mortality rate of 23.1%, however, the difference was not statistically significant (Fisher's exact probability method, P=0.323). Among 60 patients with focal choroid invasion without pathological high-risk factors, no death was observed, while among the other 42 patients presenting high-risk factors, 7 of them died with a mortality rate of 16.7% (Fisher's exact probability method, P=0.003). Cox multivariate analysis showed that massive choroid invasion and surgical margin of the optic nerve were influential factors of prognosis. Conclusion Patients with focal choroid invasion have a low disease recurrence and may not receive adjuvant chemotherapy. Patients with massive choroid invasion without presenting pathological high-risk factors warrant further prospective study to assess whether adjuvant chemotherapy is needed. However chemotherapy is recommended for those with massive choroid invasion presenting with risk factors to avoid the high disease recurrence in such patients. Key words: Retinoblastoma; Choroid; Prognosis; Child