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Retinoblastoma survival and enucleation outcomes in 41 countries from the African continent
  1. Thamanna Nishath1,
  2. Andrew W Stacey2,
  3. David Steinberg3,
  4. Allen Foster4,
  5. Richard Bowman4,5,
  6. Vera Essuman6,
  7. Ido Didi Fabian4,7
  8. The Global Retinoblastoma Study Group
    1. 1University of Washington School of Medicine, Seattle, Washington, USA
    2. 2Department of Ophthalmology, University of Washington, Seattle, Washington, USA
    3. 3Department of Statistics and Operations Research, School of Mathematical Sciences, Raymond and Beverly Sackler Faculty of Exact Sciences, Tel Aviv University, Tel Aviv, Israel
    4. 4International Centre for Eye Health, London School of Hygiene and Tropical Medicine, London, UK
    5. 5Ophthalmology Department, Great Ormond Street Children's Hospital, London, UK
    6. 6Korle Bu Teaching Hospital, Accra, Ghana
    7. 7Goldschleger Eye Institute, Sheba Medical Center, Tel Hashomer, Tel-Aviv University, Tel-Aviv, Israel
    1. Correspondence to Thamanna Nishath; tnishath{at}uw.edu

    Abstract

    Background Retinoblastoma is the most common intraocular malignancy in childhood. Despite one-third of cases occurring in Africa, little is known of the outcomes on the continent. This study aims to explore survival and globe salvage outcomes and identify their risk factors across a large cohort of patients from the African continent.

    Methods A 3-year prospective, observational study was conducted. Kaplan-Meier survival analysis was used to investigate the risk of globe loss and death from retinoblastoma in Africa. Cox regression was used to identify risk factors associated with these outcomes.

    Results A total of 958 patients from 41 African countries and 66 participating centres were enrolled in the study. The survival rate was 78.2% at 1 year and 66.2% at 3 years after diagnosis. Cox regression showed a higher risk of death with the most advanced clinical stage (cT4, HR=6.29 vs cT2, p<0.001). The risk of losing at least one eye after diagnosis was 50% within 4 months and 72.6% within 3 years. Higher risk of enucleation was associated with a higher clinical stage compared with cT1 (cT3, HR=4.11, p=0.001; cT4, HR=3.77, p=0.005).

    Conclusion Nearly one in every four children diagnosed with retinoblastoma in African participating centres succumb to retinoblastoma within 1 year. There is also high morbidity associated with the diagnosis as a large majority of patients require eye removal surgery. The outcome of disease in children with retinoblastoma in Africa is poor compared with other continents and requires prompt intervention by increasing efforts to improve survival and eye salvage outcomes.

    • Neoplasia
    • Prognosis
    • Public health
    • Retina
    • Risk Factors

    Data availability statement

    All data relevant to the study are included in the article or uploaded as online supplemental information.

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    Data availability statement

    All data relevant to the study are included in the article or uploaded as online supplemental information.

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    Footnotes

    • Correction notice This paper has been corrected since it was first published. The affiliations have been updated.

    • Collaborators The Global Retinoblastoma Study Group: Elhassan Abdallah, Shehu U Abdullahi, Rula A Abdulqader, Aminatu A Abdulrahaman, Sherif Abouelnaga, Dupe S Ademola-Popoola, Adedayo Adio, Mahmoud A Afifi, Ada E Aghaji, Adeseye M Akinsete, Amadou I Alfa Bio, Amany M Ali, Argentino A Almeida, Khalifa M Alsawidi, Doreen Amankwaa-Frempong, Nicholas J Astbury, Rose Atsiaya, Covadonga Bascaran, Sarra Benmiloud, Rokia C Berete, Sharon Blum, Gabrielle C Bouda, Hédi Bouguila, Aléine Budiongo, Matthew J Burton, Faraja S Chiwanga, Line Couitchere, Alan Davidson, Magritha D Bruyn, Johannes P Du Plessis, Asmaa El Kettani, Amal M Elbahi, Alaa M Elhaddad, Moawia MA Elhassan, Mahmoud M Elzembely, Ted Grimbert A Evina, Ifeoma R Ezegwui, Oluyemi Fasina, Jennifer A Geel, Nir Gomel, Koffi M Guedenon, Sadiq Hassan, Laila Hessissen, Diriba F Hordofa, Shilo Horev, Affiong A Ibanga, Theophile BA Kabesha, Rolande L Kabore, Abubakar Kalinaki, Pius Kamsang, Noa Kapelushnik, Jess Elio Kosh Komba Palet, Mariana Kruger, Alice Kyara, Robert M Lukamba, Ibrahim O Matende, Marchelo Matua, Ismail Mayet, Freddy B Mbumba, Aemero A Mengesha, Furahini G Mndeme, Ahmed A Mohamedani, Claude Moreira, Mchikirwa S Msina, Gerald Msukwa, Ichengelo Muma, Kareem O Musa, Anne A Musika, Hamzah Mustak, Tajudeen Mustapha, Okwen M Muyen, Khumo Myezo, Gita Naidu, Natasha Naidu, Paule Aïda Ndoye Roth, Elizabeth D Nkanga, Henry Nkumbe, Marcel N Numbi, Mutale Nyaywa, Chinsisi Nyirenda, Ghislaine Obono-Obiang, Vivian Paintsil, Luisa Paiva, Remezo Philbert, Hoby L Randrianarisoa, Léa Raobela, Lorna A Renner, David Reynders, Dahiru Ribadu, Azza MA Said, Trish A Scanlan, Sadik T Sherief, Sidi Sidi cheikh, Grace Ssali, David K Stones, Fatoumata Sylla, Tuyisabe Theophile, Fousseyni Traoré, Harba Tyau-Tyau, Ali B Umar, Keith Waddell, Amina H Wali, Julie Wetter, Amelia DC Wime, Jenny M Yanga, Ekhtelbenina Zein, Othman AO Ziko, Marcia Zondervan.

    • Contributors Conception and design of study: AS and IDF. Statistical analysis: DS and SH. Data interpretation: TN, AS, DS, SH and IDF. Initial drafting of the manuscript: TN and AS. Critical revisions of article and important intellectual content: all authors. Final approval of manuscript: TN, AS and IDF. TN and AS are guarantors.

    • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

    • Competing interests None declared.

    • Provenance and peer review Not commissioned; externally peer reviewed.

    • Supplemental material This content has been supplied by the author(s). It has not been vetted by BMJ Publishing Group Limited (BMJ) and may not have been peer-reviewed. Any opinions or recommendations discussed are solely those of the author(s) and are not endorsed by BMJ. BMJ disclaims all liability and responsibility arising from any reliance placed on the content. Where the content includes any translated material, BMJ does not warrant the accuracy and reliability of the translations (including but not limited to local regulations, clinical guidelines, terminology, drug names and drug dosages), and is not responsible for any error and/or omissions arising from translation and adaptation or otherwise.