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Primary Subretinal Seeding in Retinoblastoma: Clinical Presentation and Treatment Outcomes
Komal Bakal1, Vishal Raval1, Sai Krishna Gattu2
1The Operation Eyesight Universal Institute for Eye Cancer, L V Prasad Eye Institute, Hyderabad, India.
Insights
Primary subretinal seeding in retinoblastoma presents a challenge, with moderate response to intravenous chemotherapy and significant recurrence rates. Globe salvage was achieved in 78% of cases, but 29% experienced recurrence.
Area of Science:
- Ophthalmology
- Pediatric Oncology
- Retinal Diseases
Background:
- Retinoblastoma (RB) is the most common primary intraocular malignancy in children.
- Primary subretinal seeding (SRS) is a challenging manifestation of RB, impacting treatment outcomes.
- Understanding the clinical features and treatment responses of SRS is crucial for improving patient management.
Purpose of the Study:
- To describe the clinical characteristics of primary subretinal seeding (SRS) in intraocular retinoblastoma (RB).
- To evaluate the treatment outcomes for patients with primary SRS.
- To identify challenges and recurrence patterns associated with SRS in RB.
Main Methods:
- A descriptive analysis was conducted on 47 patients (50 eyes) diagnosed with primary SRS in intraocular RB.
- Clinical features, treatment modalities, and follow-up data were retrospectively collected and analyzed.
- Statistical analysis was performed to determine the incidence of SRS, associated features, treatment responses, and recurrence rates.
Main Results:
- Primary SRS involved multiple quadrants in 88% of eyes, typically appearing yellowish-gray and located near the main tumor.
- Subretinal fluid (100%), total retinal detachment (56%), and vitreous seeds (40%) were common associated features.
- Intravenous chemotherapy (IVC) was the primary treatment (94%), with adjunct focal therapies used in 40%. Retinal tumor control was achieved in 76% of eyes, but SRS recurrence was noted in 29% of eyes.
Conclusions:
- Primary subretinal seeding (SRS) in retinoblastoma poses a significant therapeutic challenge.
- Systemic intravenous chemotherapy (IVC) shows a moderate response, with approximately one-third of cases experiencing SRS recurrence.
- Despite challenges, globe salvage was achieved in 78% of patients, though enucleation was required in some cases.
Introduction:
The aim of this study was to describe the clinical features and treatment outcomes of primary subretinal seeding (SRS) in patients with intraocular retinoblastoma (RB).
Methods:
Descriptive analysis of primary SRS in 47 patients (50 eyes) with RB was performed.
Results:
Mean age was 19 months (range, 2-72 months), and 55% (n = 26) of the subjects were male. At presentation, the SRS involved two or more quadrants in 88% of eyes. Most seeds appeared yellowish gray (66%) and round to oval in shape (48%). Two-thirds of SRS were seen posterior to the equator and within 5 mm from the main tumor. Associated features included subretinal fluid in 50 eyes (100%), total retinal detachment in 28 eyes (56%), and vitreous seeds in 20 eyes (40%). Treatment included intravenous chemotherapy (IVC) (n = 47; 94%), enucleation (n = 2; 4%), and intra-arterial chemotherapy (n = 1; 2%). SRS treatment included adjunct use of focal transpupillary thermotherapy and/or cryotherapy (n = 20; 40%). Retinal tumor control was achieved in 36 eyes (76%) with 32 eyes (78%) showing a type 3 regression pattern, while SRS completely regressed in 24 (48%) eyes, partially in 15 (30%) and worsened in 2 (4%) eyes. Over a mean follow-up of 30 months (range, 3-68 months), SRS recurrence was noted in 12 eyes (29%), globe salvage was achieved in 39 eyes (78%), and 1 (4%) patient died of presumed metastasis.
Conclusion:
Primary SRS pose a therapeutic challenge during RB treatment. The SRS responds moderately to systemic IVC, with one-third cases showing SRS recurrence and one-fifth ultimately requiring enucleation.
