Ido Didi FabianElhassan AbdallahShehu U. AbdullahiRula A. AbdulqaderSahadatou Adamou BoubacarDupe S. Ademola-PopoolaAdedayo AdioArmin R. AfsharPriyanka AggarwalAda E. AghajiAlia AhmadMarliyanti N.R. AkibLamis Al HarbyMouroge H. Al AniAygun AlakbarovaSilvia Alarcón PortabellaSafaa A.F. Al-BadriAna Patricia A. AlcasabasSaad A. Al-DahmashAmanda AlejosErnesto Alemany-RubioAmadou I. Alfa BioYvania Alfonso CarrerasChristiane Al-HaddadHamoud H.Y. Al-HussainiAmany M. AliDonjeta B. AliaMazin F. Al-JadiryUsama Al-JumalyHind M. AlkatanCharlotta All-ErikssonAli A.R.M. Al-MafrachiArgentino A. AlmeidaKhalifa M. AlsawidiAthar A.S.M. Al-ShaheenEntissar H. Al-ShammaryPrimawita O. AmiruddinRomanzo AntoninoNicholas J. AstburyHatice T. AtalayLa Ongsri AtchaneeyasakulRose AtsiayaTaweevat AttasethThan H. AungSilvia AyalaBaglan BaizakovaJulia BalaguerRuhengiz BalayevaWalentyna BalwierzHonorio BarrancoCovadonga BascaranMaja Beck PopovicRaquel BenavidesSarra BenmiloudNissrine Bennani GuebessiRokia C. BereteJesse L. BerryAnirban BhaduriSunil BhatShelley J. BiddulphEva M. BiewaldNadia BobrovaMarianna BoehmeH. C. BoldtMaria Teresa B.C. BonanomiNorbert BornfeldGabrielle C. BoudaHédi BouguilaAmaria BoumedaneRachel C. BrennanBénédicte G. BrichardJassada BuaboonnamPatricia Calderón-SoteloDoris A. Calle JaraJayne E. CamugliaMiriam R. CanoMichael CapraNathalie CassouxGuilherme CastelaLuis CastilloJaume Català-MoraGuillermo L. ChantadaShabana ChaudhrySonal S. ChauguleArgudit ChauhanBhavna ChawlaVioleta S. ChernodrinskaFaraja S. ChiwangaTsengelmaa ChuluunbatKrzysztof CieslikRuellyn L. CockcroftCodruta ComsaZelia M. CorreaMaria G. Correa LlanoTimothy W. CorsonKristin E. Cowan-LynMonika CsókaXuehao CuiIsac V. Da GamaWantanee DangboonHawler Medical UniversityDr. Rajendra Prasad Centre for Ophthalmic SciencesFilatov Institute of Eye Diseases and Tissue TherapyInstytut "Pomnik - Centrum Zdrowia Dziecka"University of TripoliUniversity Hospital Centre TiranaCentre Hospitalier Universitaire Yalgado OuedraogoHospital Nacional de Niños Dr. Carlos Sáenz HerreraCentro Hospitalario Pereira RossellMuhimbili National HospitalUniversity of the Philippines ManilaBaghdad Teaching HospitalUniversity of BaghdadChildren's Hospital LahoreUniversité de Tunis El Manar, Institut Hedi Raies d'ophtalmologie de TunisCalcutta Medical Research InstituteAmerican University of Beirut Medical CenterKing Edward Medical University LahoreHospital Universitari i Politècnic La FeLondon School of Hygiene & Tropical MedicineUniversity of Port HarcourtUniversity of NigeriaShanghai Jiao Tong UniversitySemmelweis EgyetemIndiana University-Purdue University IndianapolisUniversity of IowaUniversity of California, San FranciscoCentre Hospitalier Universitaire VaudoisUniwersytet Jagielloński Collegium MedicumUniversity of LouisvilleStarship Children's HealthOur Lady's Hospital for Sick ChildrenCentre Hospitalier Universitaire Ibn-RochdSt. Jude Children's Research HospitalCentro Hospitalar e Universitário de CoimbraIRCCS Ospedale Pediatrico Bambino GesùUniversity of Cincinnati College of MedicineMoorfields Eye Hospital NHS Foundation TrustUniversity of WitwatersrandFaculty of Medicine, Ramathibodi Hospital, Mahidol UniversityCentre Hospitalier Universitaire Hassan IIKeck School of Medicine of USCGazi ÜniversitesiKing Saud UniversityCliniques Universitaires Saint-LucHospital Universitari Vall d'HebronInstitutul Oncologic Prof. Dr. Alexandru Trestioreanu BucurestiUniversity Hospital AlexandrovskaFaculty of Medicine, Siriraj Hospital, Mahidol UniversityUniversity of IlorinBanaras Hindu UniversityUniversidade de Sao Paulo - USPUniversitäts Klinikum Essen und Medizinische FakultätPrince of Songkla UniversityTel Aviv UniversityConsejo Nacional de Investigaciones Científicas y TécnicasBayero UniversityFundacion Hospital de Pediatria Professor Dr. Juan P. GarrahanAssiut UniversityImam Hussein Cancer CenterEtablissement Hospitaliere Specialise Emir Abdelkader CEA Service d'Oncologie PediatriqueScientific Center of Pediatrics and Pediatric SurgeryMohammed v UniversityUniversité Paris DescartesQueensland Children's HospitalBasra Specialty HospitalNational Center for Maternal and Child HealthUniversity of Medicine 1Central Hospital QuelimaneIbn Al-Haitham Teaching Eye HospitalUniversity of ParakouPBMA's H. V. Desai Eye HospitalSt. Damien Pediatric HospitalTeaching Hospital of TreichvilleNational Centre of Ophthalmology Named after Acad. Zarifa AliyevaBeira Central HospitalInstituto Cubano de Oftalmología Ramón Pando FerrerHospital Sant Joan de DéuMinisterio de Salud PúblicaSt Erik Eye HospitalNational Oncology CentreHospital del Niño Dr. Francisco de Icaza BustamanteRetina Consultants of HoustonUnidad Nacional de Oncología PediátricaLighthouse for Christ Eye CentreCicendo Eye HospitalNational Hospital of NiameyBustamante Hospital for ChildrenHospital Infantil Manuel de Jesus Rivera2020-03-262020-03-262020-01-01JAMA Oncology. (2020)23742445237424372-s2.0-85081545321https://repository.li.mahidol.ac.th/handle/20.500.14594/53618© 2020 Cambridge University Press. All rights reserved. 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- A nd 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.Mahidol UniversityBiochemistry, Genetics and Molecular BiologyMedicineArticleSCOPUS10.1001/jamaoncol.2019.6716