Abstract
Gaurav Kumar
Background Retinoblastoma (RB) is the most common primary intraocular malignancy in children.
We sought to provide a comprehensive assessment of epidemiological profile and treatment
outcomes of children with RB.
Methods In this retrospective study, we analyzed 189 children diagnosed with RB at our center
between 2004 and 2017. Survival was analyzed with the Kaplan–Meier method and log-rank
test.
Results Median age at presentation was 14 months with male: female ratio 1.2:1. Mean duration
between onset of symptoms and presentation was 49 days (standard deviation ± 79).
Most common presenting symptom was white pupillary reflex in 60% of children. Family
history of RB and other cancers was found in one (0.5%) and seven (4%) children, respectively.
Primary mode of diagnosis and staging was ocular ultrasonography (bone scan) in 87%
of patients. Computed tomographic scan and magnetic resonance imaging were done in
124 (66%) and 30 (16%) patients, respectively. International staging system grade
E disease was found in 144 (76%), extraocular disease in 55 (29%), bilateral disease
in 49 (26%), and trilateral disease in 3 (1.5%) children. Out of 189 children with
RB, 33 (18%) refused treatment and 156 children received treatment (24 children [15%]
abandoned treatment midway and 132 [85%] completed treatment). One hundred children
(64%) received systemic therapy as neoadjuvant or adjuvant chemotherapy and 20 (13%)
received local therapy. Eyeball and vision salvage rate with chemotherapy were 20
(13%) and 9 (6%), respectively. Cryotherapy was the most common modality of local
treatment used in 11 (55%) children. Five-year survival for patients who received
treatment was 76% (median survival not reached). In the treatment refusal group, median
survival was 9 months.
Conclusion In developing countries, RB is mostly detected in advanced stages resulting in poor
outcomes. Increased awareness and accessibility to dedicated centers for treating
childhood malignancy can lead to early diagnosis, better prognosis, and increased
vision salvage.
Keywords
advanced stages - chemotherapy - childhood malignancy - retinoblastoma - survival
- treatment refusal
