TY - JOUR
T1 - The Global Retinoblastoma Outcome Study
T2 - a prospective, cluster-based analysis of 4064 patients from 149 countries
AU - The Global Retinoblastoma Study Group
AU - Fabian, Ido Didi
AU - Abdallah, Elhassan
AU - Abdullahi, Shehu U.
AU - Abdulqader, Rula A.
AU - Abdulrahaman, Aminatu A.
AU - Abouelnaga, Sherif
AU - Ademola-Popoola, Dupe S.
AU - Adio, Adedayo
AU - Afifi, Mahmoud A.
AU - Afshar, Armin R.
AU - Aggarwal, Priyanka
AU - Aghaji, Ada E.
AU - Ahmad, Alia
AU - Akib, Marliyanti N. R.
AU - Akinsete, Adeseye
AU - Al Harby, Lamis
AU - Al Mesfer, Saleh
AU - Al Ani, Mouroge H.
AU - Alarcón Portabella, Silvia
AU - Al-Badri, Safaa A. F.
AU - Alcasabas, Ana Patricia A.
AU - Al-Dahmash, Saad A.
AU - Alejos, Amanda
AU - Alemany-Rubio, Ernesto
AU - Alfa Bio, Amadou I.
AU - Alfonso Carreras, Yvania
AU - Al-Haddad, Christiane E.
AU - Al-Hussaini, Hamoud H. Y.
AU - Ali, Amany M.
AU - Alia, Donjeta B.
AU - Al-Jadiry, Mazin F.
AU - Al-Jumaily, Usama
AU - Alkatan, Hind M.
AU - All-Eriksson, Charlotta
AU - Al-Mafrachi, Ali A. RM
AU - Almeida, Argentino A.
AU - Alsawidi, Khalifa M.
AU - Al-Shaheen, Athar A. SM
AU - Al-Shammary, Entissar H.
AU - Amankwaa-Frempong, Doreen
AU - Amiruddin, Primawita O.
AU - Armytasari, Inggar
AU - Astbury, Nicholas J.
AU - Atalay, Hatice T.
AU - Ataseven, Eda
AU - Atchaneeyasakul, La-ongsri
AU - Atsiaya, Rose
AU - Autrata, Rudolf
AU - Moll, Annette C.
AU - van Hoefen Wijsard, Milo
N1 - Funding Information: This work was supported by a grant from the Queen Elizabeth Diamond Jubilee Trust (TG002). MJB is supported by grants from the Wellcome Trust (207472/Z/17/Z). Publisher Copyright: © 2022 The Author(s). Published by Elsevier Ltd. This is an Open Access article under the CC BY 4.0 license
PY - 2022/8/1
Y1 - 2022/8/1
N2 - Background: Retinoblastoma is the most common intraocular cancer worldwide. There is some evidence to suggest that major differences exist in treatment outcomes for children with retinoblastoma from different regions, but these differences have not been assessed on a global scale. We aimed to report 3-year outcomes for children with retinoblastoma globally and to investigate factors associated with survival. Methods: We did a prospective cluster-based analysis of treatment-naive patients with retinoblastoma who were diagnosed between Jan 1, 2017, and Dec 31, 2017, then treated and followed up for 3 years. Patients were recruited from 260 specialised treatment centres worldwide. Data were obtained from participating centres on primary and additional treatments, duration of follow-up, metastasis, eye globe salvage, and survival outcome. We analysed time to death and time to enucleation with Cox regression models. Findings: The cohort included 4064 children from 149 countries. The median age at diagnosis was 23·2 months (IQR 11·0–36·5). Extraocular tumour spread (cT4 of the cTNMH classification) at diagnosis was reported in five (0·8%) of 636 children from high-income countries, 55 (5·4%) of 1027 children from upper-middle-income countries, 342 (19·7%) of 1738 children from lower-middle-income countries, and 196 (42·9%) of 457 children from low-income countries. Enucleation surgery was available for all children and intravenous chemotherapy was available for 4014 (98·8%) of 4064 children. The 3-year survival rate was 99·5% (95% CI 98·8–100·0) for children from high-income countries, 91·2% (89·5–93·0) for children from upper-middle-income countries, 80·3% (78·3–82·3) for children from lower-middle-income countries, and 57·3% (52·1–63·0) for children from low-income countries. On analysis, independent factors for worse survival were residence in low-income countries compared to high-income countries (hazard ratio 16·67; 95% CI 4·76–50·00), cT4 advanced tumour compared to cT1 (8·98; 4·44–18·18), and older age at diagnosis in children up to 3 years (1·38 per year; 1·23–1·56). For children aged 3–7 years, the mortality risk decreased slightly (p=0·0104 for the change in slope). Interpretation: This study, estimated to include approximately half of all new retinoblastoma cases worldwide in 2017, shows profound inequity in survival of children depending on the national income level of their country of residence. In high-income countries, death from retinoblastoma is rare, whereas in low-income countries estimated 3-year survival is just over 50%. Although essential treatments are available in nearly all countries, early diagnosis and treatment in low-income countries are key to improving survival outcomes. Funding: Queen Elizabeth Diamond Jubilee Trust.
AB - Background: Retinoblastoma is the most common intraocular cancer worldwide. There is some evidence to suggest that major differences exist in treatment outcomes for children with retinoblastoma from different regions, but these differences have not been assessed on a global scale. We aimed to report 3-year outcomes for children with retinoblastoma globally and to investigate factors associated with survival. Methods: We did a prospective cluster-based analysis of treatment-naive patients with retinoblastoma who were diagnosed between Jan 1, 2017, and Dec 31, 2017, then treated and followed up for 3 years. Patients were recruited from 260 specialised treatment centres worldwide. Data were obtained from participating centres on primary and additional treatments, duration of follow-up, metastasis, eye globe salvage, and survival outcome. We analysed time to death and time to enucleation with Cox regression models. Findings: The cohort included 4064 children from 149 countries. The median age at diagnosis was 23·2 months (IQR 11·0–36·5). Extraocular tumour spread (cT4 of the cTNMH classification) at diagnosis was reported in five (0·8%) of 636 children from high-income countries, 55 (5·4%) of 1027 children from upper-middle-income countries, 342 (19·7%) of 1738 children from lower-middle-income countries, and 196 (42·9%) of 457 children from low-income countries. Enucleation surgery was available for all children and intravenous chemotherapy was available for 4014 (98·8%) of 4064 children. The 3-year survival rate was 99·5% (95% CI 98·8–100·0) for children from high-income countries, 91·2% (89·5–93·0) for children from upper-middle-income countries, 80·3% (78·3–82·3) for children from lower-middle-income countries, and 57·3% (52·1–63·0) for children from low-income countries. On analysis, independent factors for worse survival were residence in low-income countries compared to high-income countries (hazard ratio 16·67; 95% CI 4·76–50·00), cT4 advanced tumour compared to cT1 (8·98; 4·44–18·18), and older age at diagnosis in children up to 3 years (1·38 per year; 1·23–1·56). For children aged 3–7 years, the mortality risk decreased slightly (p=0·0104 for the change in slope). Interpretation: This study, estimated to include approximately half of all new retinoblastoma cases worldwide in 2017, shows profound inequity in survival of children depending on the national income level of their country of residence. In high-income countries, death from retinoblastoma is rare, whereas in low-income countries estimated 3-year survival is just over 50%. Although essential treatments are available in nearly all countries, early diagnosis and treatment in low-income countries are key to improving survival outcomes. Funding: Queen Elizabeth Diamond Jubilee Trust.
UR - http://www.scopus.com/inward/record.url?scp=85133828984&partnerID=8YFLogxK
U2 - https://doi.org/10.1016/S2214-109X(22)00250-9
DO - https://doi.org/10.1016/S2214-109X(22)00250-9
M3 - Article
C2 - 35839812
SN - 2214-109X
VL - 10
SP - e1128-e1140
JO - The Lancet Global Health
JF - The Lancet Global Health
IS - 8
ER -