Objectives To evaluate the long-term treatment outcomes in intraocular retinoblastoma (RB) including the associated factors for eventual treatment with external beam radiotherapy (EBRT) and enucleation as well as to analyse the risk factors for metastasis and death in extraocular RB. Methods Retrospective analysis of 390 eyes from 256 (89.8%) intraocular RB and 29 (10.2%) extraocular RB cases diagnosed and treated between October 1998 and May 2018 at one of the largest tertiary care centers in Turkey. Results Of 351 intraocular RB eyes, 53.3% had group D/E disease at presentation. 75 (21.4%) of 351 eyes underwent primary enucleation. Of the remaining 276 eyes undergoing eye-conserving treatments, 201 (72.8%) were salvaged. Most of these eyes were treated using intravenous chemotherapy and/or focal treatments [transpupillary thermotherapy (TTT) and cryotherapy] initially. EBRT was eventually required in 48 (17.4%) eyes and secondary enucleation in 75 (27.2%) eyes. At mean follow-ups of 76.7 and 39.7 months for intraocular and extraocular RB cohorts, respectively, 180 (46.2%) eyes underwent primary/secondary enucleation and exenteration. Overall, 13 cases developed metastasis and 9 died. Two patients with trilateral RB also expired. Multivariable risk factors for enucleation were the presence of vitreous seeds ( p < 0.001), absence of EBRT administration ( p = 0.033), 5–9 TTT applications compared with no TTT ( p = 0.031), and each 1 mm increase in tumour base diameter ( p < 0.001). Univariate factors predictive of metastasis were the presence of extraocular RB detected by imaging methods ( p < 0.001) and extrascleral/optic nerve cut end involvement at histopathological examination ( p < 0.001). Conclusions In our series, 72.8% of the intraocular RB eyes undergoing eye-conserving treatments were saved. The globe salvage rate for all intraocular and extraocular RB eyes was 53.8% and the overall survival rate was 96.1%.

Purpose: To review the authors' experience in the diagnosis of retinoblastoma and to explore the frequency of intraocular conditions that mimic this malignancy according to patient age at presentation. Methods: This was a retrospective observational study including 549 patients (769 eyes) who were referred for confirmation and/or management of retinoblastoma between October 1998 and June 2019 at a single tertiary center. A detailed ocular examination was done by the same ocular oncologist under general anesthesia for every patient. Results: Of 549 patients referred for diagnostic confirmation or management of retinoblastoma, 393 (71.6%) patients were found to have retinoblastoma and 156 (28.4%) patients received the diagnosis of pseudoretinoblastoma. The mean patient age at presentation was 52.1 months, ranging from 1 to 276 months. The most common diagnoses among patients with pseudoretinoblastoma younger than 1 year were persistent fetal vasculature (PFV) (n = 19; 28.8%), Coats disease (n = 7; 10.6%), chorioretinal coloboma (n = 4; 6.1%), retinal dysplasia (n = 4; 6.1%), and retinal detachment (n = 4; 6.1%). In patients with pseudoretinoblastoma who were 1 to 5 years old, the most common diagnoses were Coats disease (n = 10; 25.6%), PFV (n = 7; 17.9%), and optic disc hypoplasia (n = 3; 7.7%). Patients older than 5 years were most likely to have Coats disease (n = 8; 15.7%), optic disc drusen (n = 5; 9.8%), retinopathy of prematurity (n = 4; 7.8%), and combined hamartoma (n = 4; 7.8%). Conclusions: This study shows that 28.4% of patients referred for suspicion or management of retinoblastoma were classified as having pseudoretinoblastoma. The most common conditions causing diagnostic confusion with retinoblastoma included PFV and Coats disease, similar to previous publications from both high-and low-income countries. [ J Pediatr Ophthalmol Strabismus . 2021;58(3):161–167.]

Objectives To report the swept-source optical coherence tomography angiography (SS-OCTA) findings in choroidal and retinal tumors. Methods A retrospective noncomparative interventional case series of 60 eyes having various choroidal and retinal tumors imaged with SS-OCTA (Topcon DR1 Triton Plus, Tokyo, Japan) between September 2018 and February 2020 was conducted. Inclusion criteria were tumor thickness <4 mm, tumor base diameter <10 mm, and tumor location at the posterior pole. Results Choroidal nevi usually demonstrated well-defined borders, hyperreflective internal structure, and no outer retinal involvement on SS-OCTA. Choroidal melanoma, in contrast to nevi, usually had ill-defined borders (p = 0.018), mixed hyperreflective-hyporeflective or hyperreflective internal structure (p = 0.014), and demonstrated outer retinal involvement (p < 0.001). Circumscribed choroidal hemangioma usually presented with well-defined borders, a hyperreflective internal tumor structure with multiple dilated interconnected tumor vessels intermixed with signal void areas representing connective tissue. Optic disc melanocytomas showed a hyporeflective plexus related to blocking of signal by the pigment and an intact radial peripapillary capillary network. There was flow on the surface and slightly deeper within the lesion on B-scan angiography overlay. Retinal astrocytic hamartomas had well-defined borders and a hyperreflective vascular plexus in the superficial and deep retina. Outer retina and choriocapillaris showed hyporeflective change due to shadowing/masking from calcium or high blood flow in the lesion. Conclusions Each of the different retinal and choroidal tumors studied in this series presented with different SS-OCTA features to aid in the differential diagnosis of these conditions. Good quality images are obtained in patients with good fixation and tumors <3 mm in thickness located at the posterior pole.

Objectives: To report treatment results and complications of stereotactic radiosurgery in uveal malignant melanoma and to identify risk factors for development of radiation retinopathy. Materials and Methods: This was a retrospective study of 36 patients diagnosed with uveal melanoma between 2014 and 2019. Best corrected visual acuity, funduscopic findings, basal tumor diameter and tumor thickness were recorded at baseline and at follow-up visits at 3-month intervals. The response of tumors to stereotactic radiosurgery and complications were determined. Results: The mean basal diameter of tumor was 10.2 (range: 4.0-19.4, standard deviation [SD]: ±3.3) mm x 9.7 (range: 4.5-18.0, SD: ±3.3), tumor thickness was 5.1 (range: 2.0-11.0, ±2.4) mm at baseline. The mean follow-up period was 17.2 (range: 6-48, SD: ±10.43) months. The mean visual acuity was 0.5 (SD: ±0.3) logMAR before treatment and 0.6 (SD: ±0.3) logMAR after the mean follow-up period. The most common complications after stereotactic radiosurgery were cataract (38.9%) and radiation retinopathy (27.7%). There was a statistically significant relation between radiation retinopathy development and tumor distance from the optic disc (p=0.04). The rate of eye salvage was 83.3% in this study. Conclusion: Our short-term results show stereotactic radiosurgery was an effective and sustained treatment modality among the other eye conservation therapies.

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