Abstract
Objective To observe the clinical characteristics and survival rate of the patients with extraocular retinoblastoma (RB). Methods This is a retrospective case analysis. From November 2003 to May 2015, 38 eyes of 31 patients with RB in the extra-ocular stage from 213 RB patients were enrolled in this study. There were 18 males and 13 females. Bilateral lesions were observed in 7 patients and unilateral lesions were observed in 24 patients.19 patients were diagnosed at less than 2 years old, 10 patients at 2 to 5 years old, and 2 patients at age over 5 years old. First visit time was less than 1 month in 12 patients, from 1 to 3 months in 15 patients, over 3 months to 6 months in 4 patients. Medical history and family history were record at the first visit. All patients underwent orbital CT, MRI, double color Doppler imaging and wide angle digital retinal imaging system. CT and (or) MRI examination detected tumor extraocular invasion. Histopathological examination showed that there were tumor cells invasion of the scleral, optic nerve root and optic nerve. Chemotherapy was done after surgery. In the extra-ocular stage, 3 to 6 rounds of intensive chemotherapy combined with orbital radiotherapy were done. The average follow-up period was (25.5±4.5) months after treatment. The cumulative survival rate was observed after 6 months, 1 and 5 years after treatment, and the relationship between the initial age, time, sex, single eye, tumor and survival time of the patients was analyzed. Results The extraocular RB accounted 14.55% of all RB patients in this study. There is no family history of RB, no special history. There were 15 patients with leukocoria and yellow-white reflection in the pupil; 5 patients with lacrimation, swelling, photophobia and exophthalmos; 11 patients with strabismus. The cumulative survival rate at 6 months, 1, 5 years after treatment was (78.0±9.0)%, (62.0±11.0)%, (57.0±11.0)% respectively. The average survival time was (53.9±7.8) months; the cumulative survival rate was (59.3±11.3)%. When the age of first visit was less than 1 month, 1 - 3 months, 3 - 6 months, the median survival time was 78, 15 and 18 months respectively, the cumulative survival rate was 100.0%, (40.0±21.9)% and (25.0±21.7)%, respectively. The survival time of the newly diagnosed patients at 1 month was more than at 1 to 6 months, and the difference was statistically significant (t=9.20, P<0.05). Conclusions 14.55% of all RB patients was extraocular RB in this study. One of the most common clinical manifestations is leukocoria at the first visit. The cumulative survival rate of extraocular RB is lower, while the survival rate of patients with the age of first visit time was less than 1 month is higher. Key words: Retinoblastoma/diagnosis; Retinoblastoma/mortality; Neoplasm metastasis; Survival rate
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