The definition of high-risk retinoblastoma differs around the world

A global survey of retinoblastoma specialists aimed to identify the definition of high-risk retinoblastoma and there was significant variability in what current treatment criteria were included from nation to nation.

Study design

Fifty-two ocular and medical oncologists and ocular pathologists working at referral retinoblastoma centers were sent a survey electronically; 27 responses were received. Questions could be answered either “yes” or “no,” and after each question respondents could provide additional comments.

Outcomes

Postlaminar optic nerve infiltration, involvement of optic nerve transection, and extrascleral extension were uniformly considered as high-risk factors for retinoblastoma metastasis. Otherwise, there was significant variability in the definition of high-risk retinoblastoma, from both oncologic and pathologic perspectives. For example, only respondents in Asia included minor choroidal invasion and prelaminar optic nerve invasion in their criteria, and no respondents from the Americas considered anterior chamber seeds to be a feature of high-risk retinoblastoma.

Limitations

The survey response rate was just over 50%, and this level of participation may have influenced the results. In addition, there was higher participation from Asian, North American, and European countries than South American or African countries.

Clinical significance

More uniformity in the definition of high-risk retinoblastoma is needed to ensure adequate treatment, particularly when deciding to prescribe radiotherapy or chemotherapy.