RESUMEN
PURPOSE: To evaluate the effectiveness of baseline screening and follow-up with magnetic resonance imaging (MRI) for detecting trilateral retinoblastoma (TRb) and assessing the risk of TRb development. DESIGN: Prospective multicenter cohort study. METHODS: A total of 607 retinoblastoma patients from 2012 through 2022 were included and followed up until September 1, 2023. At each center, a neuroradiologist categorized pineal glands on baseline and follow-up scans into 4 groups: (A) normal, (B) cystic gland, (C) suspicious gland, or (D) TRb. Different follow-up schedules were assigned to each category. Categories B and C were followed up with MRI after approximately 3 months and repeated 3 months later if suspicion remained. On each MRI, they measured the height and width, evaluated the aspect (solid, partly cystic, and completely cystic) of the pineal gland, and evaluated radiologic features suspicious of pineal TRb. The effectiveness of the current TRb screening method was assessed by evaluating its sensitivity and specificity to detect TRb. Determining the TRb incidence was a secondary outcome measure. RESULTS: Heritable retinoblastoma patients had a risk of 3.78% to develop TRb. One of 4 pineal TRbs was detected during a follow-up scan and 4 of 5 nonpineal TRbs were detected on the baseline MRI. Screening for pineal TRb had a sensitivity of 25% and specificity of 100%; for nonpineal TRb, the sensitivity was 80%. It required 494 follow-up scans to detect 1 pineal TRb. However, when restricting the follow-up to solely suspicious glands, only 22 scans were required to detect 1 pineal TRb. CONCLUSION: During extended follow-up after baseline MRI, only 1 pineal trilateral retinoblastoma was detected in our study. Follow-up after 3 months should be restricted to patients with a suspicious pineal gland defined as irregular thickening of the cyst wall (>2 mm), fine nodular aspect of the cyst wall, or when a solid or cystic gland exceeds the upper 99% prediction interval for size; patients with an unsuspicious cystic gland should not be followed up. Baseline MRI screening was able to detect most nonpineal trilateral retinoblastomas.
RESUMEN
This retrospective multicenter study examines therapy-induced orbital and ocular MRI findings in retinoblastoma patients following selective intra-arterial chemotherapy (SIAC) and quantifies the impact of SIAC on ocular and optic nerve growth. Patients were selected based on medical chart review, with inclusion criteria requiring the availability of posttreatment MR imaging encompassing T2-weighted and T1-weighted images (pre- and post-intravenous gadolinium administration). Qualitative features and quantitative measurements were independently scored by experienced radiologists, with deep learning segmentation aiding total eye volume assessment. Eyes were categorized into three groups: eyes receiving SIAC (Rb-SIAC), eyes treated with other eye-saving methods (Rb-control), and healthy eyes. The most prevalent adverse effects post-SIAC were inflammatory and vascular features, with therapy-induced contrast enhancement observed in the intraorbital optic nerve segment in 6% of patients. Quantitative analysis revealed significant growth arrest in Rb-SIAC eyes, particularly when treatment commenced ≤ 12 months of age. Optic nerve atrophy was a significant complication in Rb-SIAC eyes. In conclusion, this study highlights the vascular and inflammatory adverse effects observed post-SIAC in retinoblastoma patients and demonstrates a negative impact on eye and optic nerve growth, particularly in children treated ≤ 12 months of age, providing crucial insights for clinical management and future research.