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: O...

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Hauptverfasser: Chang, Ying (VerfasserIn) , Jonas, Jost B. (VerfasserIn)
Dokumenttyp: Article (Journal)
Sprache:Englisch
Veröffentlicht: April 24, 2015
In: Ophthalmologica
Year: 2015, Jahrgang: 233, Heft: 3-4, Pages: 222-229
ISSN:1423-0267
DOI:10.1159/000381220
Online-Zugang:Verlag, Volltext: http://dx.doi.org/10.1159/000381220
Verlag, Volltext: http://www.karger.com.ezproxy.medma.uni-heidelberg.de/Article/Abstract/381220
Volltext
Verfasserangaben:Ying Chang, Jitong Shi, Junyang Zhao, Xiaolin Xu, Jianmin Ma, Ling Shen, Haixia Bai, Fei Gao, Zhibao Zhang, Bin Li, Jost B. Jonas

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520 |a 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. 
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