Elsevier

American Journal of Ophthalmology

Volume 160, Issue 6, December 2015, Pages 1116-1126.e5
American Journal of Ophthalmology

Original article
The Incidence of Trilateral Retinoblastoma: A Systematic Review and Meta-Analysis

https://doi.org/10.1016/j.ajo.2015.09.009Get rights and content
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Purpose

To estimate the incidence of trilateral retinoblastoma in patients with retinoblastoma.

Design

Systematic review and meta-analysis.

Methods

We searched Medline and Embase for scientific literature published between January 1966 and July 2015 that assessed trilateral retinoblastoma incidence. We used a random-effects model for the statistical analyses.

Results

We included 23 retinoblastoma cohorts from 26 studies. For patients with bilateral retinoblastoma the unadjusted chance of developing trilateral retinoblastoma across all cohorts was 5.3% (95% confidence interval [CI]: 3.3%–7.7%); the chance of pineal trilateral retinoblastoma was 4.2% (95% CI: 2.6%–6.2%) and the chance of nonpineal trilateral retinoblastoma was 0.8% (95% CI: 0.4%–1.3%). In patients with hereditary retinoblastoma (all bilateral cases, and the unilateral cases with a family history or germline RB1 mutation) we found a trilateral retinoblastoma incidence of 4.1% (95% CI: 1.9%–7.1%) and a pineal trilateral retinoblastoma incidence of 3.7% (95% CI: 1.8%–6.2%). To reduce the risk of overestimation bias we restricted analysis to retinoblastoma cohorts with a minimum size of 100 patients, resulting in adjusted incidences of 3.8% (95% CI: 2.4%–5.4%), 2.9% (95% CI: 1.9%–4.2%), and 0.7% (95% CI: 0.3%–1.2%) for any, pineal, and nonpineal trilateral retinoblastoma, respectively, among patients with bilateral retinoblastoma. Among hereditary retinoblastoma we found an adjusted trilateral retinoblastoma incidence of 3.5% (95% CI: 1.2%–6.7%) and a pineal trilateral retinoblastoma incidence of 3.2% (95% CI: 1.4%–5.6%).

Conclusion

The estimated incidence of trilateral retinoblastoma is lower than what is reported in previous literature, especially after exclusion of small cohorts that were subject to overestimation bias in this context.

Cited by (0)

Marcus C. de Jong, MD, MSc, is a resident and PhD student at the department of Radiology and Nuclear Medicine at the VU University Medical Center, Amsterdam (the Netherlands). With a background in medicine and clinical epidemiology (he received both degrees from the Erasmus University Rotterdam) he has been working on various topics in the field of retinoblastoma (with a focus on the use of magnetic resonance imaging, but also on epidemiological topics) since 2012.

Supplemental Material available at AJO.com.