Browsing by Author "Elder, JE"

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High-risk histopathological features of retinoblastoma following primary enucleation: A Global Study Of 1,426 Patients From 5 Continents
(Lippincott Williams and Wilkins, 2024) Kaliki, S; Vempuluru, VS; Bakal, KR; Dorji, S; Tanna, V; Shields, CN; Fallon, SJ; Raval, V; Ahmad, A; Mushtaq, A; Hussain, M; Yousef, YA; Mohammad, M; Roy, SR; Huque, F; Tatiana, U; Yuri, S; Vladimir, P; Zambrano, SC; Alarcón-León, S; Valdiviezo-Zapata, C; Vargas-Martorellet, M; Gutierrez-Chira, C; Buitrago, M; Ortiz, JS; Diaz-Coronado, R; Tripathy, D; Rath, S; Patil, G; Berry, JL; Pike, S; Brown, B; Tanabe, M; Frenkel, S; Eiger-Moscovich, M; Pe'er, J; Shields, CL; Eagle, RC,, Jr; Laiton, A; Velasco, AM; Vega, K; Desimone, J; Bejjanki, KM; Kapoor, AG; Venkataraman, A; Bryant, V; Reddy, MA; Sagoo, MS; Hubbard, GB; Azarcon, CP; Olson, TA; Grossniklaus, H; Rolfe, O; Staffieri, SE; O'day, R; Mathew, AA; Elder, JE; Mckenzie, JD; Fabian, ID; Shemesh, R; Vishnevskia-Dai, V; Ali, MH; Jakati, S; Mishra, DK; Palkonda, VAR
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/without orbital radiotherapy was given to 761 (53%) patients. Based on Kaplan-Meier estimates in different continents (AS vs. AUS vs. EUR vs. NA vs. SA), the 6-year risk of orbital tumor recurrence was 5% versus 2% versus 0% versus 0% versus 12% (P < 0.001), systemic metastasis was reported in 8% versus 5% versus 2% versus 0% versus 13% (P = 0.001), and death in 10% versus 3% versus 2% versus 0% versus 11% (P < 0.001) patients. Conclusion: There is a wide variation in the infiltrative histopathological features of retinoblastoma across continents, resulting in variable outcomes. SA and AS had a higher risk of orbital tumor recurrence, systemic metastasis, and death compared to AUS, EUR, and NA. Copyright © 2024 The Author(s). Published by Wolters Kluwer Health, Inc.
Neovascular Glaucoma as a Predictor of Retinoblastoma High-Risk Histopathology in an International Multicentre Study
(Lippincott Williams and Wilkins, 2024) Negretti, GS; Ushakova, T; Yuri, S; Vladimir, P; Berry, JL; Pike, S; Shields, CL; Hubbard, GB,, III; Eiger-Moscovich, M; Pe'er, J; Staffieri, SE; Elder, JE; McKenzie, JD; Ahmad, A; Hussain, M; Casavilca-Zambrano, S; Alarcon-Leon, S; Yousef, YA; Mohammad, M; Tanabe, M; Arazi, M; Fabian, ID; Goldstein, S; Kaliki, S; Sagoo, MS; Reddy, MA
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 regression odds ratio for NVG predicting high-risk histopathology was 1.73 (95% confidence interval: 1.3 to 2.31) and from multivariate logistic regression was 1.77 (95% confidence interval: 1.23 to 2.56). Patients with a longer duration of symptoms (p=0.03), buphthalmos (p=0.02) and ectropion uveae (p<0.01) were more likely to have high-risk histopathology. Patients with NVG were more likely to develop metastasis than cT2 patients (p=0.04). Conclusions: There is a significant association between NVG at presentation, high-risk histopathology and metastatic risk.
Retinoblastoma Outcomes Based on the 8th Edition American Joint Committee on Cancer Pathological Classification in 1411 Patients
(Elsevier Inc., 2024) Vempuluru, VS; Shields, CL; Berry, JL; Kaliki, S; Ahmad, A; Bejjanki, KM; Diaz-Coronado, R; Eiger-Moscovich, M; Elder, JE; Fabian, ID; Frenkel, S; Grossniklaus, H; Hubbard, GB,, III; Kapoor, AG; Mohammad, M; McKenzie, JD; Pe'er, J; Rath, S; Reddy, MA; Rolfe, O; Roy, SR; Sagoo, MS; Staffieri, SE; Tanabe, M; Tatiana, U; Tripathy, D; Vishnevskia-Dai, V; Vladimir, P; Yousef, YA
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; tumor-related metastasis was seen in 7 (1%), 5 (3%), 40 (8%), and 46 (43%) of pT1, pT2, pT3, and pT4 (P < 0.001) categories, respectively; tumor-related death was seen in 12 (2%), 7 (4%), 64 (13%), and 64 (59%) of pT1, pT2, pT3, and pT4 (P < 0.001) categories, respectively. Multivariate Cox proportional hazards analysis of outcomes revealed pT category and adjuvant therapy as independent predictors of outcomes. Categories pT3b (P = 0.005), pT3c (P < 0.001), pT3d (P < 0.001), and pT4 (P < 0.001) had a greater hazard for orbital recurrence; categories pT2a (P = 0.015), pT3a (P < 0.001), pT3b (P < 0.001), pT3c (P < 0.001), pT3d (P < 0.001), and pT4 (P < 0.001) had a greater hazard for tumor-related metastasis; and categories pT2a (P = 0.068), pT2b (P = 0.004), pT3a (P < 0.001), pT3b (P < 0.001), pT3c (P < 0.001), pT3d (P < 0.001), and pT4 (P < 0.001) had a greater hazard for tumor-related death when compared with the pT1 category. Patients who did not receive adjuvant therapy had greater hazards of orbital tumor recurrence in categories pT3b (P = 0.005), pT3c (P = 0.003), and pT4 (P = 0.002); greater hazards of tumor-related metastasis in categories pT3a (P = 0.001), pT3b (P = 0.01), pT3c (P = 0.001), and pT4 (P = 0.007); and tumor-related death in categories pT3a (P < 0.001), pT3b (P = 0.009), pT3c (P = 0.018), and pT4 (P < 0.001) when compared with those who received adjuvant therapy. Conclusions: The 8th edition AJCC pathological classification predicts outcomes in patients undergoing primary enucleation for RB, and adjuvant therapy is associated with a lower risk of orbital recurrence, tumor-related metastasis, and tumor-related death in the pT3 and pT4 categories. Financial Disclosure(s): The author(s) have no proprietary or commercial interest in any materials discussed in this article.
Treatment Outcomes and Definition Inconsistencies in High-Risk Unilateral Retinoblastoma
(Elsevier Inc., 2024) Arazi, M; Baum, A; Casavilca-Zambrano, S; Alarcon-Leon, S; Diaz-Coronado, R; Ahmad, Alia; Mushtaq, Asma; Hussain, M; Ushakova, T; Yuri, S; Vladimir, P; Shields, Cl; Eagle, RC; Berry, JL; Pike, S; Brown, B; Roy, Sr; Huque, F; Fabian, Ina; Frenkel, S; Eiger-Moscovich, Maya; Pe'er, J; Hubbard, GB; Olson, TA; Grossniklaus, H; Reddy, MA; Sagoo, MA; Staffieri, SE; Elder, JE; Mckenzie, JD; Tanabe, M; Kaliki, S; Fabian, ID
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 invasion, were included. Main outcome measures included orbital tumor recurrence, systemic metastasis, survival and number, and outcome of cases converted to standardized HRHF. Results: A total of 600 children presenting to 14 centers in 9 countries were included. Of these, 505 (84.2%) were considered locally as HRHF and received adjuvant chemotherapy. After a median follow-up period of 39.2 ± 1.6 months (range: 0.8-60.0 months), 36 (6.0%) had orbital tumor recurrence, 49 (8.2%) metastasis, and 72 (12.0%) children died. Children not receiving adjuvant chemotherapy were at significantly increased risk of orbital tumor recurrence, metastasis, and death (P ≤ .002). Of the study children, 63/600 (10.5%) were considered locally non-HRHF, but converted to standardized HRHF and included in the analysis. Of these, 6/63 (9.5%) had orbital tumor recurrence, 5/63 (7.9%) metastasis, and 6/63 (9.5%) children died. Isolated minor choroidal invasion with prelaminar/laminar optic nerve invasion was reported in 114 (19.0%) children, but considered locally as HRHF only in 68/114 (59.6%). Of these, 6/114 (5.3%) children developed metastasis and subsequently died, yielding a number needed to treat of 15. Conclusion: Based on this multinational cohort of children with Rb, we recommend the use of adjuvant chemotherapy following upfront enucleation and diagnosis of HRHF. Variation exists worldwide among centers when defining HRHF, resulting in adverse patient outcomes, warranting standardization.