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artículo
Publicado 2024
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Purpose: To assess histopathology and outcomes following primary enucleation of eyes with retinoblastoma presenting with neovascular glaucoma (NVG). Methods: This was an international multi-centre case series study across five continents. Retrospective review of patient charts was performed for all patients undergoing primary enucleation for retinoblastoma (n=1420) using a standardised data-collection spreadsheet. Clinical features, pathological grade, and outcomes were compared between NVG patients and those with an American Joint Commission on Cancer (AJCC) 8th edition clinical stage of cT2. High-risk histopathology was defined as AJCC 8th edition pathological stage ≥pT2b. Results: NVG was seen in 224/1420 (16%) patients. Mean age at presentation of those with NVG was 30 months (median 25, range 0-120 months) and 131(58%) patients had high-risk histopathology. The univariate logistic...
2
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Publicado 2024
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PURPOSE. Little is known regarding differences in childhood growth between somatic and heritable retinoblastoma (Rb) populations. We aimed to compare childhood growth parameters between somatic and heritable Rb cohorts at birth and at time of diagnosis with Rb. METHODS. A multinational, longitudinal cohort study was conducted with patients from 11 centers in 10 countries who presented with treatment naïve Rb from January to December 2019. Variables of interest included age, sex, and size characteristics at birth and at time of presentation, as well as germline mutation status. After Bonferroni correction, results were statistically significant if the P value was less than 0.005. RESULTS. We enrolled 696 patients, with 253 analyzed after exclusion criteria applied. Between somatic (n = 39) and heritable (n = 214) Rb cohorts, with males and females analyzed separately, there was no signif...
3
artículo
Publicado 2024
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Purpose: To compare the clinical outcomes of children with unilateral retinoblastoma (Rb) and high-risk histopathology features (HRHF) following upfront enucleation with/without adjuvant chemotherapy, and investigate cases locally considered non-HRHF but converted to a standardized HRHF definition. Design: Retrospective multinational clinical cohort study. Methods: Children with Rb who presented to 21 centers from 12 countries between 2011-2020, and underwent primary enucleation were recruited. Centers retrieved clinical data and were asked to report detailed histopathology findings, as well as indicate cases defined locally as high-risk. For analysis, only unilateral cases with standardized HRHF, defined as retrolaminar optic nerve invasion, massive choroidal invasion, scleral invasion, anterior-segment involvement, and/or combined nonmassive choroidal and prelaminar/laminar optic nerve...
4
artículo
Publicado 2024
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Purpose: To evaluate the outcomes of retinoblastoma (RB) based on the 8th edition of the American Joint Committee on Cancer (AJCC) pathological classification in a global cohort of patients. Design: Retrospective, multicenter, intercontinental, collaborative study. Participants: A total of 1411 patients. Intervention: Primary enucleation with or without adjuvant chemotherapy or radiotherapy. Main Outcome Measures: Orbital tumor recurrence, tumor-related metastasis, and tumor-related death. Results: According to the 8th edition AJCC pathological classification, 645 eyes (46%) belonged to pathological T (pT)1, 164 (11%) to pT2, 493 (35%) to pT3, and 109 (8%) to pT4 categories. At a mean follow-up of 38 months (median, 35 months; < 1–149 months), orbital tumor recurrence was seen in 8 (1%), 5 (3%), 22 (4%), and 25 (23%) of pT1, pT2, pT3, and pT4 (P < 0.001) categories, respectively;...
5
artículo
Publicado 2023
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Background/Objectives: Retinoblastoma is a common childhood intraocular malignancy, the bilateral form of which most commonly results from a de novo germline pathogenic variant in the RB1 gene. Both advanced maternal age and decreasing birth order are known to increase the risk of de novo germline pathogenic variants, while the influence of national wealth is understudied. This cohort study aimed to retrospectively observe whether these factors influence the ratio of bilateral retinoblastoma cases compared to unilateral retinoblastoma, thereby inferring an influence on the development of de novo germline pathogenic variants in RB1. Subjects/Methods: Data from 688 patients from 11 centres in 10 countries were analysed using a series of statistical methods. Results: No associations were found between advanced maternal age, birth order or GDP per capita and the ratio of bilateral to unilate...
6
artículo
Publicado 2024
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Purpose: To evaluate high-risk histopathological features following primary enucleation of eyes with retinoblastoma and assess the patient outcomes across continents. Methods: A retrospective study of 1,426 primarily enucleated retinoblastoma eyes from five continents. Results: Of all, 923 (65%) were from Asia (AS), 27 (2%) from Australia (AUS), 120 (8%) from Europe (EUR), 162 (11%) from North America (NA), and 194 (14%) from South America (SA). Based on the continent (AS vs. AUS vs. EUR vs. NA vs. SA), the histopathological features included massive choroidal invasion (31% vs. 7% vs. 13% vs. 19% vs. 27%, P = 0.001), postlaminar optic nerve invasion (27% vs. 0% vs. 16% vs. 21% vs. 19%, P = 0.0006), scleral infiltration (5% vs. 0% vs. 4% vs. 2% vs. 7%, P = 0.13), and microscopic extrascleral infiltration (4% vs. 0% vs. <1% vs. <1% vs. 4%, P = 0.68). Adjuvant chemotherapy with/withou...
