Retinoblastoma in Chinese Children Aged Five to Fourteen Years.
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.
Beijing Institute of Ophthalmology, Beijing Tongren Eye Center, Beijing Tongren Hospital, Capital Medical University, Beijing, China.
Ying
Y
Chang
Jitong
J
Shi
Junyang
J
Zhao
Xiaolin
X
Xu
Jianmin
J
Ma
Ling
L
Shen
Haixia
H
Bai
Fei
F
Gao
Zhibao
Z
Zhang
Bin
B
Li
Jost B
JB
Jonas
D000293:Adolescent; D044466:Asian People / Q000208:ethnology; D002648:Child; D002675:Child, Preschool; D002681:China / Q000453:epidemiology; D015353:Eye Enucleation; D005260:Female; D006801:Humans; D008297:Male; D019572:Retinal Neoplasms / Q000453:epidemiology* / Q000473:pathology / Q000601:surgery; D012175:Retinoblastoma / Q000453:epidemiology* / Q000473:pathology / Q000601:surgery; D012189:Retrospective Studies; D012307:Risk Factors