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Timing of pineal trilateral retinoblastoma and retinoblastoma development appear unrelated

American Academy of Ophthalmology News Jun 05, 2020

In this study, researchers assessed the correlation between age at the time of retinoblastoma and pineal trilateral retinoblastoma (TRb) diagnoses.

Study design

This meta-analyses analyzed studies published between 1966 and 2019. The study identified and included 138 patients with pineal TRb.


The age at diagnosis of retinoblastoma did not correlate with age at diagnosis of pineal TRb. Ninety-five percent of asymptomatic pineal TRb patients were diagnosed before the age of 40 months. The lead time between detectable pineoblastoma on MRI to development of symptoms is approximately 1 year.

Prior chemotherapy or radiotherapy for retinoblastoma was not associated with the interval to detection of pineal TRb. The estimated incidence of metachronous pineal TRb is less than 2% in patients with heritable retinoblastoma. By performing a screening MRI scan every 6 months after the diagnosis of heritable retinoblastoma until 36 months of age, hundreds of scans would be required to detect 1 case of asymptomatic pineal TRb and to save a single life. Age at diagnosis of heritable intraocular retinoblastoma and pineal TRb likely are independent.


As pineal TRb is rare, meta-analysis is the only way to obtain larger numbers for analysis. This paper is subject to publication bias limitations inherent with meta-analysis.

Clinical significance

Age at time of pineal TRb diagnosis did not correlate with age at time of retinoblastoma diagnosis, disease laterality or prior treatments, indicating the development of pineal TRb may be independent of retinoblastoma.

Ninety-five percent of patients with an asymptomatic pineal TRb are diagnosed before the age of 40 months, suggesting screening should be pursued only until this age.

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