Retinoblastoma Outcomes in the Americas: A Prospective Analysis of 491 Children With Retinoblastoma From 23 American Countries.

PURPOSE: Globally, disparities exist in retinoblastoma treatment outcomes between high- and low-income countries, but independent analysis of American countries is lacking. We report outcomes of American retinoblastoma patients and explore factors associated with survival and globe salvage. DESIGN: Subanalysis of prospective cohort study data. METHODS: Multicenter analysis at 57 American treatment centers in 23 countries of varying economic levels (low income [LIC], lower-middle income [LMIC], upper-middle income [UMIC], and high income [HIC]) of 491 treatment-naïve retinoblastoma patients diagnosed in 2017 and followed through 2020. Survival and globe salvage rates analyzed with Kaplan-Meier analysis and Cox proportional hazard models. RESULTS: Of patients, 8 (1.6%), 58 (11.8%), 235 (47.9%), and 190 (38.7%) were from LIC, LMIC, UMIC, and HIC groups, respectively. Three-year survival rates in LICs were 60.0% (95% confidence interval [CI] 12.6-88.2) compared with 99.2% (95% CI 94.6%-99.9%) in HICs. Death was less likely in patients >4 years of age (vs ≤4 years, HR = 0.45 [95% CI 0.27-0.78], P = .048). Patients with more advanced tumors (eg, cT3 vs cT1, HR = 4.65 × 109 [95% CI 1.25 × 109-1.72 × 1010], P < .001) and females (vs males, HR = 1.98 [95% CI 1.27-3.10], P = .04) were more likely to die. Three-year globe salvage rates were 13.3% (95% CI 5.1%-25.6%) in LMICs and 46.2% (95% CI 38.8%-53.3%) in HICs. At 3 years, 70.1% of cT1 eyes (95% CI 54.5%-81.2%) vs 8.9% of cT3 eyes (95% CI 5.5%-13.3%) were salvaged. Advanced tumor stage was associated with higher enucleation risk (eg, cT3 vs cT1, subhazard ratio = 4.98 [95% CI 2.36-10.5], P < .001). CONCLUSIONS: Disparities exist in survival and globe salvage in American countries based on economic level and tumor stage demonstrating a need for childhood cancer programs.

Association of Optical Coherence Tomography-Measured Fibrovascular Ridge Thickness and Clinical Disease Stage in Retinopathy of Prematurity.

Importance: Accurate diagnosis of retinopathy of prematurity (ROP) is essential to provide timely treatment and reduce the risk of blindness. However, the components of an ROP examination are subjective and qualitative. Objective: To evaluate whether optical coherence tomography (OCT)-derived retinal thickness measurements at the vascular-avascular junction are associated with clinical diagnosis of ROP stage. Design, Setting, and Participants: This cross-sectional longitudinal study compared OCT-based ridge thickness calculated from OCT B-scans by a masked examiner to the clinical diagnosis of 2 masked examiners using both traditional stage classifications and a more granular continuous scale at the neonatal intensive care unit (NICU) of Oregon Health & Science University (OHSU) Hospital. Infants who met ROP screening criteria in the OHSU NICU between June 2021 and April 2022 and had guardian consent were included. One OCT volume and en face image per patient per eye showing at least 1 to 2 clock hours of ridge were included in the final analysis. Main Outcomes and Measures: Comparison of OCT-derived ridge thickness to the clinical diagnosis of ROP stage using an ordinal and continuous scale. Repeatability was assessed using 20 repeated examinations from the same visit and compared using intraclass correlation coefficient (ICC) and coefficient of variation (CV). Comparison of ridge thickness with ordinal categories was performed using generalized estimating equations and with continuous stage using Spearman correlation. Results: A total of 128 separate OCT eye examinations from 50 eyes of 25 patients were analyzed. The ICC was 0.87 with a CV of 7.0%. Higher ordinal disease classification was associated with higher axial ridge thickness on OCT, with mean (SD) thickness measurements of 264.2 (11.2) µm (P < .001), 334.2 (11.4) µm (P < .001), and 495.0 (32.2) µm (P < .001) for stages 1, 2, and 3, respectively and with continuous stage labels (ρ = 0.739, P < .001). Conclusions and Relevance: These results suggest that OCT-based quantification of peripheral stage in ROP may be an objective and quantitative biomarker that may be useful for clinical diagnosis and longitudinal monitoring and may have implications for disease classification in the future.

Epidermolytic acanthoma: A case report.

BACKGROUND: Epidermolytic acanthoma (EA) is a rare benign skin lesion, usually found in the genital area of men and women, with epidermolytic hyperkeratosis as its distinguishing histologic characteristic. It is commonly misdiagnosed as condyloma accuminatum, verruca, and seborrheic keratosis. Since this lesion is benign, treatment is not necessary. However, it is often misdiagnosed, and patients are likely to undergo incorrect counseling and unnecessary treatment, causing undue burden to the patient. This study seeks to increase awareness of this rare condition to prevent future misdiagnoses. CASE SUMMARY: A 55-year-old man living with human immunodeficiency virus presented for anal cancer screening. His physical examination revealed a flesh colored papule at the anal margin. The initial differential diagnosis included molluscum contagiosum, anal condyloma, and basal cell carcinoma. The lesion was excised to obtain a definitive diagnosis and was discovered to be EA. CONCLUSION: EA is often misdiagnosed due to its similarity to other dermatologic conditions. Careful examination and pathologic evaluation should be obtained to ensure proper diagnosis.