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Global Retinoblastoma Presentation and Analysis by National Income Level

Global Retinoblastoma Study GroupInternational Centre for Eye Health, London School of Hygiene & Tropical Medicine, London, United KingdomIdo Didi FabianInternational Centre for Eye Health, London School of Hygiene & Tropical Medicine, London, United KingdomAbdallah ElhassanOphthalmology Department of Rabat, Mohammed V University, Rabat, MoroccoShehu U. AbdullahiBasra Children's Specialty Hospital, Basra, IraqRula Ahmed AbdulqaderBasra Children’s Specialty Hospital, Basra, IraqSahadatou Adamou BoubacarNational Hospital of Niamey, Niamey, NigerDupe Ademola-PopoolaUniversity of Ilorin Teaching Hospital, University of Ilorin, Ilorin, NigeriaAdedayo AdioDepartment of Ophthalmology, University of Port Harcourt Teaching Hospital, Port Harcourt, NigeriaArmin R. AfsharUniversity of California, San FranciscoPriyanka AggarwalDepartment of Pediatrics, Banaras Hindu University, Varanasi, IndiaAE AghajiDepartment of Ophthalmology, College of Medicine, University of Nigeria, Enugu, NigeriaAlia AhmadMarliyanti N. R. AkibRS Dr Wahidin Sudirohusodo, Makassar, IndonesiaLamis Al HarbyThe Royal London Hospital, Barts Health NHS Trust, and Moorfields Eye Hospital NHS Foundation Trust, London, United KingdomMouroge H. Al AniHawler Medical University, Erbil, IraqAygun AlakbarovaZarifa Aliyeva National Center of Ophthalmology, Baku, AzerbaijanS. Alarcón PortabellaDepartment of Pediatric Ophthalmology, Hospital Vall d’Hebron, Barcelona, SpainSafa A. FarajPediatric Oncology Unit, Children Welfare Teaching Hospital, College of Medicine, University of Baghdad, Baghdad, IraqAna Patricia AlcasabasPhilippine General Hospital, University of the Philippines, Manila, PhilippinesSaad Al-DahmashCollege of Medicine, King Saud University, Riyadh, Saudi ArabiaAmanda AlejosUnidad Nacional de Oncología Pediátrica, Guatemala City, GuatemalaErnesto Alemany-RubioInstituto Cubano de Oftalmología Ramón Pando Ferrer, Marianao, Havana, CubaAmadou I Alfa BioUniversity of Parakou, Parakou, BeninYvania Alfonso CarrerasSt Damien Pediatric Hospital, Port-au-Prince, HaitiChristiane Al‐HaddadDepartment of Ophthalmology, American University of Beirut Medical Center, Beirut, LebanonHamoud H. Y. Al-HussainiPediatric Oncology Department, National Oncology Center, Sana’a, YemenAmany AliPediatric Oncology Department, South Egypt Cancer Institute, Assiut University, Assiut, EgyptDonjeta B. AliaUniversity Hospital Center Mother Theresa, Tirana, AlbaniaMazin Faisal Al‐JadiryPediatric Oncology Unit, Children Welfare Teaching Hospital, College of Medicine, University of Baghdad, Baghdad, IraqUsama Al‐JumailyImam Hussein Cancer Center, Karbala, IraqHind M. AlkatanCollege of Medicine, King Saud University, Riyadh, Saudi ArabiaCharlotta All‐ErikssonSt Erik Eye Hospital, Stockholn, SwedenAli A. R. M. Al-MafrachiIbn Al Haitham Teaching Eye Hospital, Baghdad, IraqArgentino A AlmeidaBeira Central Hospital, Beira, MozambiqueKhalifa M AlsawidiTripoli Eye Hospital, University of Tripoli, Tripoli, LibyaAthar Abdul Samad Majeed Al-ShaheenBasra Children’s Specialty Hospital, Basra, IraqEntissar Hadi Al-ShammaryOncology Unit, Child’s Central Teaching Hospital, Baghdad, IraqPrimawita O. AmiruddinNational Eye Center, Cicendo Eye Hospital, Bandung, IndonesiaAntonino RomanzoBambino Gesù IRCCS Children’s Hospital, Rome, ItalyNick AstburyInternational Centre for Eye Health, London School of Hygiene & Tropical Medicine, London, United KingdomHatice Tuba AtalayDepartment of Ophthalmology, School of Medicine, Gazi University, Ankara, TurkeyL.–O. AtchaneeyasakulSiriraj Hospital, Mahidol University, Bangkok, ThailandRose AtsiayaLighthouse For Christ Eye Centre, Mombasa, KenyaTaweevat AttasethDepartment of Ophthalmology, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, ThailandThan AungYangon Eye Hospital, University of Medicine 1, Yangon, MyanmarSilvia González AyalaRetina Consultants of Houston, Houston, TexasBaglan BaizakovaScientific Center of Pediatrics and Pediatric Surgery, Almaty, KazakhstanJulia BalaguerPediatric Oncology Unit, Hospital Universitario y Politécnico La Fe, Valencia, SpainRuhengiz BalayevaZarifa Aliyeva National Center of Ophthalmology, Baku, AzerbaijanWalentyna BalwierzInstitute of Pediatrics, Jagiellonian University Medical College, Children’s University Hospital of Krakow, Krakow, PolandHonorio BarrancoPediatric Oncology Unit, Hospital Universitario y Politécnico La Fe, Valencia, SpainCovadonga BascaránInternational Centre for Eye Health, London School of Hygiene & Tropical Medicine, London, United KingdomMaja PopovićPediatric Hematology-Oncology Unit, Lausanne University Hospital, Lausanne, SwitzerlandRaquel BenavidesHospital Nacional de Niños Dr Carlos Sáenz Herrera, San Jose, Costa RicaSarra BenmiloudDepartment of Pediatric Oncology, University Hassan II Fès, Fez, MoroccoNissrine Bennani GuebessiCenter Hospitalier et Universitaire Ibn Rochd, Casablanca, MoroccoR. BérétéOphthalmologic Department of the Teaching Hospital of Treichville, Abidjan, Côte d’IvoireJesse L. BerryChildren’s Hospital Los Angeles, Keck School of Medicine, University of Southern California, Los AngelesAnirban BhaduriThe Calcutta Medical Research Institute, Kolkata, IndiaSunil BhatDepartment of Pediatric Hematology and Oncology, Narayana Health City, Bangalore, IndiaS J BiddulphUniversity of the Witwatersrand, Johannesburg, South AfricaEva BiewaldDepartment of Ophthalmology, Essen University Hospital, University Duisburg-Essen, Essen, GermanyNadia BobrovaThe Filatov Institute of Eye Diseases and Tissue Therapy, Odessa, UkraineMarianna BoehmeDepartment of Ophthalmology, Essen University Hospital, University Duisburg-Essen, Essen, GermanyH. Culver BoldtDepartment of Ophthalmology and Visual Sciences, University of Iowa, Iowa CityMaria Teresa B.C. BonanomiHospital das Clínicas da FMUSP, São Paulo, BrazilNorbert BornfeldDepartment of Ophthalmology, Essen University Hospital, University Duisburg-Essen, Essen, GermanyGabrielle Chantal BoudaCentre Hospitalier Universitaire Yalgado Ouédraogo de Ouagadougou, Ouagadougou, Burkina FasoH. BouguilaInstitut Hédi Raïs d’Ophtalmologie, Faculté de Médecine, Université Tunis El Manar, Tunis, TunisiaAmaria BoumedaneEtablissement Hospitalière Spécialise Emir Abdelkader CEA Service d’Oncologie Pédiatrique, Oran, AlgeriaRachel C. BrennanSolid Tumor Division, Department of Oncology, St. Jude Children’s Research Hospital, Memphis, TennesseeBénédicte G. BrichardCliniques Universitaires Saint-Luc, Brussel, BelgiumJassada BuaboonnamSiriraj Hospital, Mahidol University, Bangkok, ThailandPatricia Calderón-SoteloHospital Infantil Manuel de Jesús Rivera, Managua, NicaraguaDoris A. Calle JaraHospital del Niño Dr. Francisco De Icaza Bustamante, Guayaquil, EcuadorJayne E CamugliaDepartment of Ophthalmology, Queensland Children's Hospital, Brisbane, Queensland, AustraliaMiriam R. CanoSalud Ocular, Ministerio de Salud Publica, Asuncion, ParaguayMichael CapraOur Lady’s Children’s Hospital, Dublin, IrelandNathalie CassouxInstitut CurieGuilherme CastelaCentro Hospital Universitário de Coimbra, University of Coimbra, Coimbra, PortugalLuis CastilloHospital Pereira Rossell, Montevideo, UruguayJaume Catalá‐MoraHospital Sant Joan de Déu, Barcelona, SpainGuillermo ChantadaHospital Garrahan, Buenos Aires, ArgentinaShabana ChaudhryPaediatric Ophthalmology Department, Mayo Hospital and College of Allied Visual Sciences, King Edward Medical University, Lahore, PakistanSonal S. ChauguleDepartment of Ophthalmic Plastic Surgery, Orbit and Ocular Oncology, PBMA’s H. V. Desai Eye Hospital, Pune, Maharashtra, IndiaArgudit ChauhanUniversity of Louisville, Louisville, KentuckyBhavna ChawlaOcular Oncology Service, Dr Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi, IndiaVioleta ChernodrinskaEye Clinic, Department of Ophthalmology, University Hospital Alexandrovska, Medical University, Sofia, Sofia, BulgariaFaraja ChiwangaMuhimbili National Hospital, Dar es Salaam, TanzaniaTsengelmaa ChuluunbatNational Center for Maternal and Children Health of Mongolia, Ulaanbaatar, MongoliaKrzysztof CieślikDepartment of Ophthalmology, The Children’s Memorial Health Institute, Warsaw, PolandRuellyn L. CockcroftStarship Children’s Health, Auckland, New ZealandCodruta ComsaInstitute of Oncology, Prof. Dr Al. Trestioreanu, Bucharest, RomaniaZélia M. CorrêaWilmer Eye Institute, Johns Hopkins Medicine, Baltimore, Maryland, and University of Cincinnati College of Medicine, Cincinnati, OhioMaria G. Correa LlanoHospital Sant Joan de Déu, Barcelona, SpainTimothy W. CorsonIndiana University Medical Center, IndianapolisKristin E. Cowan-LynBustamante Hospital for Children, Kingston, JamaicaMonika CsókaSemmelweis University, Budapest, HungaryXuehao CuiDepartment of Ophthalmology, Xinhua Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, ChinaIsac V. Da GamaQuelimane Central Hospital, Quelimane, Mozambique
JAMA Oncologyjournal2020en
ABI

Аннотация

Importance: Early diagnosis of retinoblastoma, the most common intraocular cancer, can save both a child's life and vision. However, anecdotal evidence suggests that many children across the world are diagnosed late. To our knowledge, the clinical presentation of retinoblastoma has never been assessed on a global scale. Objectives: To report the retinoblastoma stage at diagnosis in patients across the world during a single year, to investigate associations between clinical variables and national income level, and to investigate risk factors for advanced disease at diagnosis. Design, Setting, and Participants: A total of 278 retinoblastoma treatment centers were recruited from June 2017 through December 2018 to participate in a cross-sectional analysis of treatment-naive patients with retinoblastoma who were diagnosed in 2017. Main Outcomes and Measures: Age at presentation, proportion of familial history of retinoblastoma, and tumor stage and metastasis. Results: The cohort included 4351 new patients from 153 countries; the median age at diagnosis was 30.5 (interquartile range, 18.3-45.9) months, and 1976 patients (45.4%) were female. Most patients (n = 3685 [84.7%]) were from low- and middle-income countries (LMICs). Globally, the most common indication for referral was leukocoria (n = 2638 [62.8%]), followed by strabismus (n = 429 [10.2%]) and proptosis (n = 309 [7.4%]). Patients from high-income countries (HICs) were diagnosed at a median age of 14.1 months, with 656 of 666 (98.5%) patients having intraocular retinoblastoma and 2 (0.3%) having metastasis. Patients from low-income countries were diagnosed at a median age of 30.5 months, with 256 of 521 (49.1%) having extraocular retinoblastoma and 94 of 498 (18.9%) having metastasis. Lower national income level was associated with older presentation age, higher proportion of locally advanced disease and distant metastasis, and smaller proportion of familial history of retinoblastoma. Advanced disease at diagnosis was more common in LMICs even after adjusting for age (odds ratio for low-income countries vs upper-middle-income countries and HICs, 17.92 [95% CI, 12.94-24.80], and for lower-middle-income countries vs upper-middle-income countries and HICs, 5.74 [95% CI, 4.30-7.68]). Conclusions and Relevance: This study is estimated to have included more than half of all new retinoblastoma cases worldwide in 2017. Children from LMICs, where the main global retinoblastoma burden lies, presented at an older age with more advanced disease and demonstrated a smaller proportion of familial history of retinoblastoma, likely because many do not reach a childbearing age. Given that retinoblastoma is curable, these data are concerning and mandate intervention at national and international levels. Further studies are needed to investigate factors, other than age at presentation, that may be associated with advanced disease in LMICs.

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