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Published on: August 4, 2011
High-risk retinoblastoma based on age at primary enucleation: a study of 616 eyes
Swathi Kaliki1, Shweta Gupta2, George Ramappa2
1The Operation Eyesight Universal Institute for Eye Cancer, L V Prasad Eye Institute, Hyderabad, 500034, India. kalikiswathi@yahoo.com.
Insights
High-risk retinoblastoma features differ by age at enucleation. Younger children (<1 year) showed more bilateral disease and buphthalmos, while older children (>4 years) had more anterior chamber pseudohypopyon and vitreous seeds.
Area of Science:
- Ophthalmology
- Pediatric Oncology
- Pathology
Background:
- Retinoblastoma is the most common primary intraocular malignancy in children.
- Histopathological features are crucial for risk stratification and treatment planning.
- Age at diagnosis and enucleation can influence disease presentation and outcomes.
Purpose of the Study:
- To investigate the association between age at primary enucleation and high-risk histopathological features of retinoblastoma.
- To identify age-specific patterns in retinoblastoma presentation and pathology.
Main Methods:
- Retrospective analysis of 616 patients with retinoblastoma.
- Data collected included age at enucleation, clinical presentation, and histopathological findings.
- Statistical analysis to determine significant differences in features across age groups.
Main Results:
- Significant differences in clinical and histopathological features were observed based on age at enucleation.
- Children ≤1 year were more likely to present with bilateral retinoblastoma and buphthalmos.
- Older children (>4 years) showed a higher prevalence of anterior chamber pseudohypopyon and vitreous seeds.
- High-risk features, including optic nerve and choroidal infiltration, varied significantly with age.
Conclusions:
- The predominant high-risk histopathological features of retinoblastoma are age-dependent.
- Understanding these age-related variations is essential for tailored risk assessment and management strategies in retinoblastoma.
Purpose:
To study the high-risk histopathology features of retinoblastoma based on age at primary enucleation.
Methods:
Retrospective study of 616 patients.
Results:
The mean age at presentation and primary enucleation for retinoblastoma was 34 months (median, 28 months; range, <1-455 months). Of these cases, 128 (21%) were aged ≤1 year, 149 (24%) were in the age group of 1-2 years, 117 (19%) in 2-3 years, 104 (17%) in 3-4 years, and 118 (19%) were >4 years of age at the time of enucleation. Bilateral retinoblastoma (34%; p < 0.0001) and buphthalmos (20%; p < 0.0001) were more common in children ≤1 year of age. Anterior chamber pseudohypopyon (15%; p < 0.0001) and vitreous seeds (53%; p < 0.0001) were more common in children aged >4 years. Based on 8th edition American Joint Committee on Cancer staging system, pT3 was less common in children ≤1 year of age (13%; p < 0.001). Based on histopathology, 38% patients had high-risk features including 24% children aged ≤1 year, 42% in the age group of 1-2 years, 34% in 2-3 years age group, 45% in 3-4 years age group, and 48% patients were >4 years of age. Post-laminar optic nerve infiltration (6%; p = 0.02) and massive choroidal infiltration (9%; p = 0.04) was least common in children ≤1 year of age. Over a mean follow-up period of 52 months (median, 36 months; range, <1-218 months), systemic metastasis and death occurred in 9% patients despite adjuvant systemic chemotherapy.
Conclusion:
The predominant high-risk histopathology feature of retinoblastoma varies with age at primary enucleation.
