Retinoblastoma in Chinese Children Aged Five to Fourteen Years

Abstract

Objective: To assess the characteristics of retinoblastomas enucleated from Chinese children aged 5-14 years. Methods: This retrospective hospital-based study included all eyes with retinoblastomas consecutively enucleated in the Beijing Tongren Hospital between August 2003 and July 2013. Results: Out of 1,205 patients, 47 (3.9%) were 5 years or older. All tumors in this age group occurred unilaterally, the patients had a negative family history, and the tumors were detected at an age of 6.9 ± 1.8 years (range: 5-14). The main clinical features at the time of examining the as yet untreated children aged 5-7 years (n = 30) or >7-14 years (n = 10) were leukocoria, strabismus, pseudohypopyon, hypertension, vitreous seeds (‘snowballs'), and calcifications. In 12 patients (26%), the retinoblastoma had not initially been diagnosed as a tumor. Histopathology revealed tumor invasion into nonretinal tissue in 19 eyes (40%). Therapy included enucleation only (n = 22; 47%), adjuvant systemic chemotherapy (n = 24; 51%), and additional orbital exenteration (n = 1). After a mean follow-up of 3.0 ± 2.1 years (range: 0.2-9.8), which was done for 40 children, none of these children showed a tumor recurrence. Conclusions: Of the children undergoing enucleation for retinoblastoma in Beijing, 3.9% were aged 5 years or more. As in Western countries, the tumor occurrence was unilateral, their family history was negative, and the survival rate was relatively high in these children. In 1 out of 4 children, the tumor had initially been misdiagnosed due to a masquerade syndrome. Retinoblastoma should be considered in the differential diagnosis of any unclear intraocular situation in children.

Clinical Features and Long-Term Follow-Up of Patients with Retinoblastoma in Turkish Children Older Than 5 Years of Age

“…In our series over a 15-year period, out of 298 consecutive children with retinoblastoma, 6% were older than 5 years of age. Different from the previously reported literature, bilateral involvement (three patients (16.66%)) was higher in our series [4,6,7]. Misdiagnosis before referral was noted in 44.4% of our cases.…”

“…In retinoblastoma patients older than 5 years of age, the disease has been assumed to occur as a sporadic pattern. is finding was supported by the unilateral presentation in all cases [4,6,7]. However, in a retrospective review by Karcioglu et al [5], designed from the Tumor Registry of King Khaled Eye Specialist Hospital, Saudi Arabia, 4 of 18 patients (22%) presented with bilateral involvement.…”

“…Retinoblastoma is rare in older children, ranging from 3.5% to 8.5% of all retinoblastomas in different series [4][5][6][7]. In a recent report from China, it was shown that 47 (3.9%) of the 1,205 retinoblastoma patients were aged 5 years or older [7].…”

“…Retinoblastoma is the most common intraocular malignancy in children [1,2]. e diagnosis is usually made before the age of 5 and nearly less than 10% are older than 5 years of age [1][2][3][4][5][6][7]. Clinically, detection of a yellow-whitish retinal solid tumor with indirect ophthalmoscopy and ultrasonography helps us to confirm the diagnosis of a retinoblastoma in most of the cases.…”

“…According to the limited source of literature, retinoblastoma in older children has been reported to occur between 3.5-8.5% [4][5][6][7]. e epidemiologic data from centers in developing countries is also important, since most patients in these countries are admitted with advanced disease.…”