Brain MRI Screening for Bilateral Retinoblastoma Patients

Area of Science:

• Ophthalmology
• Pediatric Oncology
• Neuro-oncology

Background:

• Trilateral retinoblastoma (TRB) is a rare, fatal intracranial neoplasm associated with heritable retinoblastoma.
• Routine brain imaging screening for TRB is debated regarding its impact on survival and the number-needed-to-treat.
• Ocular oncology centers commonly screen retinoblastoma patients via brain imaging.

Purpose of the Study:

• To evaluate the impact of a recommended screening schedule (baseline brain imaging followed by clinical need-based imaging) on the survival of bilateral retinoblastoma patients.
• To determine if routine screening brain imaging prolongs TRB patients' survival.

Main Methods:

• Retrospective observational study of 109 bilateral retinoblastoma patients diagnosed between 1986 and 2020.
• Data collected included demographics, clinical features, systemic evaluation, treatment, follow-up, and outcomes.
• Analysis of baseline and follow-up brain imaging (MRI/CT) and survival data.

Main Results:

• Of 109 patients, 53% had baseline brain imaging, all within normal limits.
• During a median follow-up of 138 months, one patient (0.9%) developed symptomatic TRB and is disease-free.
• Eight patients (7.3%) expired due to retinoblastoma-related causes; six developed metastatic disease.

Conclusions:

• Routine baseline brain MRI screening for TRB may be adequate, potentially avoiding anesthesia, costs, and distress without compromising survival.
• Periodic brain MRI until age 3 remains a viable option due to TRB's fatality and treatment morbidity.