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.…”

“…Only 37.5% of patients presented with leukocoria. Distinct from the series by Chang et al [7], none of our patients reported pain in their initial examination.…”

“…According to the literature, the misdiagnosis rates are reported as 17-32% in different series. Shields [4][5][6][7]. e misdiagnosis rate before referral was 44.4% and quite high in our series of older children.…”

“…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.…”

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.…”

“…Only 37.5% of patients presented with leukocoria. Distinct from the series by Chang et al [7], none of our patients reported pain in their initial examination.…”

“…According to the literature, the misdiagnosis rates are reported as 17-32% in different series. Shields [4][5][6][7]. e misdiagnosis rate before referral was 44.4% and quite high in our series of older children.…”

“…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 Presentation and Survival: A four-year analysis from a tertiary care hospital

“…None of these children showed a tumor recurrence during an average follow-up of 3 years. 20 Our overall recurrence rate was 68.6% despite management agreed by multidisciplinary decision making. Recurrences were seen after enucleation elsewhere (n=34, 37.6%).…”

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.…”

“…Only 37.5% of patients presented with leukocoria. Distinct from the series by Chang et al [7], none of our patients reported pain in their initial examination.…”

“…According to the literature, the misdiagnosis rates are reported as 17-32% in different series. Shields [4][5][6][7]. e misdiagnosis rate before referral was 44.4% and quite high in our series of older children.…”

“…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 Presentation and Survival: A four-year analysis from a tertiary care hospital

“…None of these children showed a tumor recurrence during an average follow-up of 3 years. 20 Our overall recurrence rate was 68.6% despite management agreed by multidisciplinary decision making. Recurrences were seen after enucleation elsewhere (n=34, 37.6%).…”

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.…”

“…Only 37.5% of patients presented with leukocoria. Distinct from the series by Chang et al [7], none of our patients reported pain in their initial examination.…”

“…According to the literature, the misdiagnosis rates are reported as 17-32% in different series. Shields [4][5][6][7]. e misdiagnosis rate before referral was 44.4% and quite high in our series of older children.…”

“…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 Presentation and Survival: A four-year analysis from a tertiary care hospital

“…None of these children showed a tumor recurrence during an average follow-up of 3 years. 20 Our overall recurrence rate was 68.6% despite management agreed by multidisciplinary decision making. Recurrences were seen after enucleation elsewhere (n=34, 37.6%).…”