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Updated: Jul 5, 2025
Reconstruct Human Retinoblastoma In Vitro
Published on: October 11, 2022
Clinical and Histopathologic Predictors of Survival Among Children With Retinoblastoma From Two Tertiary Health
Raymond Atwine1, Damaris Laffita2, Abraham Birungi1
1Pathology, Mbarara University of Science and Technology, Mbarara, UGA.
Insights
Retinoblastoma survival in Ugandan children is predicted by clinical signs like leukocoria and proptosis, along with factors such as optic nerve invasion and gender. Early detection and understanding these predictors are crucial for better patient outcomes.
Area of Science:
- Ophthalmology
- Pediatric Oncology
- Pathology
Background:
- Retinoblastoma (RB) is the most common pediatric intraocular cancer.
- It originates from immature retinal cells and is curable.
- Clinical and histological findings post-enucleation are vital for prognosis and secondary care.
Purpose of the Study:
- To identify clinical and histopathological predictors of survival in children with retinoblastoma.
- To analyze data from two tertiary health facilities in Uganda.
Main Methods:
- Retrospective analysis of enucleated eye specimens (2014-2016).
- Histopathological examination using hematoxylin and eosin staining to confirm RB, stage, and features.
- Retrieval of clinical data including leukocoria, proptosis, and orbital extension.
Main Results:
- The study included 78 children, with males (55.1%) predominating.
- Leukocoria (69.2%) was the most common sign; Stage 1 was the most frequent histopathological stage (41%).
- Optic nerve invasion (38.5%), choroidal invasion (29.5%), and orbital extension (16.7%) were observed. Two-year survival was 61.5%.
- Significant survival predictors included leukocoria (RR 1.1), female gender (RR 1.4), intralaminar optic nerve invasion (RR 7.6), and lack of orbital extension (RR 7).
Conclusions:
- Leukocoria and proptosis are key clinical indicators of retinoblastoma.
- While many patients present at Stage 1, Stage 4 is also common.
- Leukocoria, optic nerve invasion, orbital extension, and gender significantly predict survival in retinoblastoma patients.
Background:
Retinoblastoma (RB) is a malignant tumour that develops from the immature cells of the retina. It is the most frequent type of paediatric intraocular cancer and is curable. Clinical and histological findings after enucleation of the affected eye dictate not only the patient's secondary care but also their prognosis. We assessed the clinical and histopathologic predictors of survival among children with RB from two tertiary health facilities in Uganda.
Methods:
This retrospective research utilized archived formalin fixed and paraffin-embedded blocks of eye specimens enucleated between 2014 and 2016 at Mbarara University of Science and Technology (MUST) Pathology Department and Ruharo Eye Centre (REC) in Mbarara, Uganda. The specimens were then processed and stained with haematoxylin and eosin. The confirmation of RB was made to include the histologic stage and features of the tumor. Biographic data of the patients and clinical features, such as leukocoria, proptosis, phthisis, staphyloma and buphthalmos, were retrieved from the records.
Results:
Males (55.1%, n=43) dominated the study population (N=78). The median age was 31 months. The most common clinical sign was leukocoria (69.2%, n=52), and the most predominant histopathological stage was stage 1 (41%, n=32). Optic nerve (ON) invasion was seen in 38.5% (n=30), choroidal invasion in 29.5% (n=23), scleral invasion in 7.7% (n=6) and orbital extension in 16.7% (n=13) of the cases. Flexner-Wintersteiner rosettes were seen in 34.6% (n=27). Necrosis was a prominent feature (71.8%, n=56). The two-year survival was estimated to be 61.5% (n=48). Leukocoria (risk ratio (RR) 1.1), female gender (RR 1.4), intralaminar ON invasion (RR 7.6) and a lack of orbital extension (RR 7) were significant predictors of survival.
Conclusion:
Leukocoria and proptosis are noticeable clinical signs of RB. Most patients present while in stage one although stage four presentation is also common. Leukocoria, ON invasion, orbital extension and gender are significant factors predictive of survival in patients with RB.
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