Abstract
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.
| Original language | English |
|---|---|
| Pages (from-to) | 685-695 |
| Number of pages | 11 |
| Journal | JAMA Oncology |
| Volume | 6 |
| Issue number | 5 |
| DOIs | |
| Publication status | Published - May 2020 |
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In: JAMA Oncology, Vol. 6, No. 5, 05.2020, p. 685-695.
Research output: Contribution to journal › Article › peer-review
TY - JOUR
T1 - Global Retinoblastoma Presentation and Analysis by National Income Level
AU - Fabian, Ido Didi
AU - Abdallah, Elhassan
AU - Abdullahi, Shehu U.
AU - Abdulqader, Rula A.
AU - Adamou Boubacar, Sahadatou
AU - Ademola-Popoola, Dupe S.
AU - Adio, Adedayo
AU - Afshar, Armin R.
AU - Aggarwal, Priyanka
AU - Aghaji, Ada E.
AU - Ahmad, Alia
AU - Akib, Marliyanti N.R.
AU - Al Harby, Lamis
AU - Al Ani, Mouroge H.
AU - Alakbarova, Aygun
AU - Portabella, Silvia Alarcón
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
AU - Al-Hussaini, Hamoud H.Y.
AU - Ali, Amany M.
AU - Alia, Donjeta B.
AU - Al-Jadiry, Mazin F.
AU - Al-Jumaly, Usama
AU - Alkatan, Hind M.
AU - All-Eriksson, Charlotta
AU - Al-Mafrachi, Ali A.R.M.
AU - Almeida, Argentino A.
AU - Alsawidi, Khalifa M.
AU - Al-Shaheen, Athar A.S.M.
AU - Al-Shammary, Entissar H.
AU - Amiruddin, Primawita O.
AU - Antonino, Romanzo
AU - Astbury, Nicholas J.
AU - Atalay, Hatice T.
AU - Atchaneeyasakul, La Ongsri
AU - Atsiaya, Rose
AU - Attaseth, Taweevat
AU - Aung, Than H.
AU - Ayala, Silvia
AU - Baizakova, Baglan
AU - Balaguer, Julia
AU - Balayeva, Ruhengiz
AU - Balwierz, Walentyna
AU - Barranco, Honorio
AU - Bascaran, Covadonga
AU - Beck Popovic, Maja
AU - Benavides, Raquel
AU - Benmiloud, Sarra
AU - Bennani Guebessi, Nissrine
AU - Berete, Rokia C.
AU - Berry, Jesse L.
AU - Bhaduri, Anirban
AU - Bhat, Sunil
AU - Biddulph, Shelley J.
AU - Biewald, Eva M.
AU - Bobrova, Nadia
AU - Boehme, Marianna
AU - Boldt, H. C.
AU - Bonanomi, Maria Teresa B.C.
AU - Bornfeld, Norbert
AU - Bouda, Gabrielle C.
AU - Bouguila, Hédi
AU - Boumedane, Amaria
AU - Brennan, Rachel C.
AU - Brichard, Bénédicte G.
AU - Buaboonnam, Jassada
AU - Calderón-Sotelo, Patricia
AU - Calle Jara, Doris A.
AU - Camuglia, Jayne E.
AU - Cano, Miriam R.
AU - Capra, Michael
AU - Cassoux, Nathalie
AU - Castela, Guilherme
AU - Castillo, Luis
AU - Català-Mora, Jaume
AU - Chantada, Guillermo L.
AU - Chaudhry, Shabana
AU - Chaugule, Sonal S.
AU - Chauhan, Argudit
AU - Chawla, Bhavna
AU - Chernodrinska, Violeta S.
AU - Chiwanga, Faraja S.
AU - Chuluunbat, Tsengelmaa
AU - Cieslik, Krzysztof
AU - Cockcroft, Ruellyn L.
AU - Comsa, Codruta
AU - Correa, Zelia M.
AU - Correa Llano, Maria G.
AU - Corson, Timothy W.
AU - Cowan-Lyn, Kristin E.
AU - Csóka, Monika
AU - Cui, Xuehao
AU - Da Gama, Isac V.
AU - Dangboon, Wantanee
AU - Das, Anirban
AU - Das, Sima
AU - Davanzo, Jacquelyn M.
AU - Davidson, Alan
AU - De Potter, Patrick
AU - Delgado, Karina Q.
AU - Demirci, Hakan
AU - Desjardins, Laurence
AU - Diaz Coronado, Rosdali Y.
AU - Dimaras, Helen
AU - Dodgshun, Andrew J.
AU - Donaldson, Craig
AU - Donato Macedo, Carla R.
AU - Dragomir, Monica D.
AU - Du, Yi
AU - Du Bruyn, Magritha
AU - Edison, Kemala S.
AU - Eka Sutyawan, I. Wayan
AU - El Kettani, Asmaa
AU - Elbahi, Amal M.
AU - Elder, James E.
AU - Elgalaly, Dina
AU - Elhaddad, Alaa M.
AU - Elhassan, Moawia M.Ali
AU - Elzembely, Mahmoud M.
AU - Essuman, Vera A.
AU - Evina, Ted Grimbert A.
AU - Fadoo, Zehra
AU - Fandiño, Adriana C.
AU - Faranoush, Mohammad
AU - Fasina, Oluyemi
AU - Fernández, Delia D.P.G.
AU - Fernández-Teijeiro, Ana
AU - Foster, Allen
AU - Frenkel, Shahar
AU - Fu, Ligia D.
AU - Fuentes-Alabi, Soad L.
AU - Gallie, Brenda L.
AU - Gandiwa, Moira
AU - Garcia, Juan L.
AU - García Aldana, David
AU - Gassant, Pascale Y.
AU - Geel, Jennifer A.
AU - Ghassemi, Fariba
AU - Girón, Ana V.
AU - Gizachew, Zelalem
AU - Goenz, Marco A.
AU - Gold, Aaron S.
AU - Goldberg-Lavid, Maya
AU - Gole, Glen A.
AU - Gomel, Nir
AU - Gonzalez, Efren
AU - Gonzalez Perez, Graciela
AU - González-Rodríguez, Liudmira
AU - Garcia Pacheco, Henry N.
AU - Graells, Jaime
AU - Green, Liz
AU - Gregersen, Pernille A.
AU - Grigorovski, Nathalia D.A.K.
AU - Guedenon, Koffi M.
AU - Gunasekera, D. Sanjeeva
AU - Gündüz, Ahmet K.
AU - Gupta, Himika
AU - Gupta, Sanjiv
AU - Hadjistilianou, Theodora
AU - Hamel, Patrick
AU - Hamid, Syed A.
AU - Hamzah, Norhafizah
AU - Hansen, Eric D.
AU - Harbour, J. William
AU - Hartnett, M. Elizabeth
AU - Hasanreisoglu, Murat
AU - Hassan, Sadiq
AU - Hassan, Shadab
AU - Hederova, Stanislava
AU - Hernandez, Jose
AU - Hernandez, Lorelay Marie Carcamo
AU - Hessissen, Laila
AU - Hordofa, Diriba F.
AU - Huang, Laura C.
AU - Hubbard, G. B.
AU - Hummlen, Marlies
AU - Husakova, Kristina
AU - Hussein Al-Janabi, Allawi N.
AU - Ida, Russo
AU - Ilic, Vesna R.
AU - Jairaj, Vivekaraj
AU - Jeeva, Irfan
AU - Jenkinson, Helen
AU - Ji, Xunda
AU - Jo, Dong Hyun
AU - Johnson, Kenneth P.
AU - Johnson, William J.
AU - Jones, Michael M.
AU - Kabesha, Theophile B.Amani
AU - Kabore, Rolande L.
AU - Kaliki, Swathi
AU - Kalinaki, Abubakar
AU - Kantar, Mehmet
AU - Kao, Ling Yuh
AU - Kardava, Tamar
AU - Kebudi, Rejin
AU - Kepak, Tomas
AU - Keren-Froim, Naama
AU - Khan, Zohora J.
AU - Khaqan, Hussain A.
AU - Khauv, Phara
AU - Kheir, Wajiha J.
AU - Khetan, Vikas
AU - Khodabande, Alireza
AU - Khotenashvili, Zaza
AU - Kim, Jonathan W.
AU - Kim, Jeong Hun
AU - Kiratli, Hayyam
AU - Kivelä, Tero T.
AU - Klett, Artur
AU - Komba Palet, Jess Elio Kosh
AU - Krivaitiene, Dalia
AU - Kruger, Mariana
AU - Kulvichit, Kittisak
AU - Kuntorini, Mayasari W.
AU - Kyara, Alice
AU - Lachmann, Eva S.
AU - Lam, Carol P.S.
AU - Lam, Geoffrey C.
AU - Larson, Scott A.
AU - Latinović, Slobodanka
AU - Laurenti, Kelly D.
AU - Le, Bao Han A.
AU - Lecuona, Karin
AU - Leverant, Amy A.
AU - Li, Cairui
AU - Limbu, Ben
AU - Long, Quah Boon
AU - López, Juan P.
AU - Lukamba, Robert M.
AU - Lumbroso, Livia
AU - Luna-Fineman, Sandra
AU - Lutfi, Delfitri
AU - Lysytsia, Lesia
AU - Magrath, George N.
AU - Mahajan, Amita
AU - Majeed, Abdul Rahim
AU - Maka, Erika
AU - Makan, Mayuri
AU - Makimbetov, Emil K.
AU - Manda, Chatonda
AU - Martín Begue, Nieves
AU - Mason, Lauren
AU - Mason, John O.
AU - Matende, Ibrahim O.
AU - Materin, Miguel
AU - Mattosinho, Clarissa C.D.S.
AU - Matua, Marchelo
AU - Mayet, Ismail
AU - Mbumba, Freddy B.
AU - McKenzie, John D.
AU - Medina-Sanson, Aurora
AU - Mehrvar, Azim
AU - Mengesha, Aemero A.
AU - Menon, Vikas
AU - Mercado, Gary John V.D.
AU - Mets, Marilyn B.
AU - Midena, Edoardo
AU - Mishra, Divyansh K.C.
AU - Mndeme, Furahini G.
AU - Mohamedani, Ahmed A.
AU - Mohammad, Mona T.
AU - Moll, Annette C.
AU - Montero, Margarita M.
AU - Morales, Rosa A.
AU - Moreira, Claude
AU - Mruthyunjaya, Prithvi
AU - Msina, McHikirwa S.
AU - Msukwa, Gerald
AU - Mudaliar, Sangeeta S.
AU - Muma, Kangwa I.
AU - Munier, Francis L.
AU - Murgoi, Gabriela
AU - Murray, Timothy G.
AU - Musa, Kareem O.
AU - Mushtaq, Asma
AU - Mustak, Hamzah
AU - Muyen, Okwen M.
AU - Naidu, Gita
AU - Nair, Akshay Gopinathan
AU - Naumenko, Larisa
AU - Ndoye Roth, Paule Aïda
AU - Nency, Yetty M.
AU - Neroev, Vladimir
AU - Ngo, Hang
AU - Nieves, Rosa M.
AU - Nikitovic, Marina
AU - Nkanga, Elizabeth D.
AU - Nkumbe, Henry
AU - Nuruddin, Murtuza
AU - Nyaywa, Mutale
AU - Obono-Obiang, Ghislaine
AU - Oguego, Ngozi C.
AU - Olechowski, Andrzej
AU - Oliver, Scott C.N.
AU - Osei-Bonsu, Peter
AU - Ossandon, Diego
AU - Paez-Escamilla, Manuel A.
AU - Pagarra, Halimah
AU - Painter, Sally L.
AU - Paintsil, Vivian
AU - Paiva, Luisa
AU - Pal, Bikramjit P.
AU - Palanivelu, Mahesh Shanmugam
AU - Papyan, Ruzanna
AU - Parrozzani, Raffaele
AU - Parulekar, Manoj
AU - Pascual Morales, Claudia R.
AU - Paton, Katherine E.
AU - Pawinska-Wasikowska, Katarzyna
AU - Pe'Er, Jacob
AU - Peña, Armando
AU - Perić, Sanja
AU - Pham, Chau T.M.
AU - Philbert, Remezo
AU - Plager, David A.
AU - Pochop, Pavel
AU - Polania, Rodrigo A.
AU - Polyakov, Vladimir G.
AU - Pompe, Manca T.
AU - Pons, Jonathan J.
AU - Prat, Daphna
AU - Prom, Vireak
AU - Purwanto, Ignatius
AU - Qadir, Ali O.
AU - Qayyum, Seema
AU - Qian, Jiang
AU - Rahman, Ardizal
AU - Rahman, Salman
AU - Rahmat, Jamalia
AU - Rajkarnikar, Purnima
AU - Ramanjulu, Rajesh
AU - Ramasubramanian, Aparna
AU - Ramirez-Ortiz, Marco A.
AU - Raobela, Léa
AU - Rashid, Riffat
AU - Reddy, M. Ashwin
AU - Reich, Ehud
AU - Renner, Lorna A.
AU - Reynders, David
AU - Ribadu, Dahiru
AU - Riheia, Mussagy M.
AU - Ritter-Sovinz, Petra
AU - Rojanaporn, Duangnate
AU - Romero, Livia
AU - Roy, Soma R.
AU - Saab, Raya H.
AU - Saakyan, Svetlana
AU - Sabhan, Ahmed H.
AU - Sagoo, Mandeep S.
AU - Said, Azza M.A.
AU - Saiju, Rohit
AU - Salas, Beatriz
AU - San Román Pacheco, Sonsoles
AU - Sánchez, Gissela L.
AU - Sayalith, Phayvanh
AU - Scanlan, Trish A.
AU - Schefler, Amy C.
AU - Schoeman, Judy
AU - Sedaghat, Ahad
AU - Seregard, Stefan
AU - Seth, Rachna
AU - Shah, Ankoor S.
AU - Shakoor, Shawkat A.
AU - Sharma, Manoj K.
AU - Sherief, Sadik T.
AU - Shetye, Nandan G.
AU - Shields, Carol L.
AU - Siddiqui, Sorath Noorani
AU - Sidi Cheikh, Sidi
AU - Silva, Sónia
AU - Singh, Arun D.
AU - Singh, Niharika
AU - Singh, Usha
AU - Singha, Penny
AU - Sitorus, Rita S.
AU - Skalet, Alison H.
AU - Soebagjo, Hendrian D.
AU - Sorochynska, Tetyana
AU - Ssali, Grace
AU - Stacey, Andrew W.
AU - Staffieri, Sandra E.
AU - Stahl, Erin D.
AU - Stathopoulos, Christina
AU - Stirn Kranjc, Branka
AU - Stones, David K.
AU - Strahlendorf, Caron
AU - Suarez, Maria Estela Coleoni
AU - Sultana, Sadia
AU - Sun, Xiantao
AU - Sundy, Meryl
AU - Superstein, Rosanne
AU - Supriyadi, Eddy
AU - Surukrattanaskul, Supawan
AU - Suzuki, Shigenobu
AU - Svojgr, Karel
AU - Sylla, Fatoumata
AU - Tamamyan, Gevorg
AU - Tan, Deborah
AU - Tandili, Alketa
AU - Tarrillo Leiva, Fanny F.
AU - Tashvighi, Maryam
AU - Tateshi, Bekim
AU - Tehuteru, Edi S.
AU - Teixeira, Luiz F.
AU - Teh, Kok Hoi
AU - Theophile, Tuyisabe
AU - Toledano, Helen
AU - Trang, Doan L.
AU - Traoré, Fousseyni
AU - Trichaiyaporn, Sumalin
AU - Tuncer, Samuray
AU - Tyau-Tyau, Harba
AU - Umar, Ali B.
AU - Unal, Emel
AU - Uner, Ogul E.
AU - Urbak, Steen F.
AU - Ushakova, Tatiana L.
AU - Usmanov, Rustam H.
AU - Valeina, Sandra
AU - Van Hoefen Wijsard, Milo
AU - Varadisai, Adisai
AU - Vasquez, Liliana
AU - Vaughan, Leon O.
AU - Veleva-Krasteva, Nevyana V.
AU - Verma, Nishant
AU - Victor, Andi A.
AU - Viksnins, Maris
AU - Villacís Chafla, Edwin G.
AU - Vishnevskia-Dai, Vicktoria
AU - Vora, Tushar
AU - Wachtel, Antonio E.
AU - Wackernagel, Werner
AU - Waddell, Keith
AU - Wade, Patricia D.
AU - Wali, Amina H.
AU - Wang, Yi Zhuo
AU - Weiss, Avery
AU - Wilson, Matthew W.
AU - Wime, Amelia D.C.
AU - Wiwatwongwana, Atchareeya
AU - Wiwatwongwana, Damrong
AU - Wolley Dod, Charlotte
AU - Wongwai, Phanthipha
AU - Xiang, Daoman
AU - Xiao, Yishuang
AU - Yam, Jason C.
AU - Yang, Huasheng
AU - Yanga, Jenny M.
AU - Yaqub, Muhammad A.
AU - Yarovaya, Vera A.
AU - Yarovoy, Andrey A.
AU - Ye, Huijing
AU - Yousef, Yacoub A.
AU - Yuliawati, Putu
AU - Zapata López, Arturo M.
AU - Zein, Ekhtelbenina
AU - Zhang, Chengyue
AU - Zhang, Yi
AU - Zhao, Junyang
AU - Zheng, Xiaoyu
AU - Zhilyaeva, Katsiaryna
AU - Zia, Nida
AU - Ziko, Othman A.O.
AU - Zondervan, Marcia
AU - Bowman, Richard
N1 - Publisher Copyright: © 2020 American Medical Association. All rights reserved.
PY - 2020/5
Y1 - 2020/5
N2 - 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.
AB - 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.
UR - http://www.scopus.com/inward/record.url?scp=85081545321&partnerID=8YFLogxK
U2 - 10.1001/jamaoncol.2019.6716
DO - 10.1001/jamaoncol.2019.6716
M3 - Article
C2 - 32105305
AN - SCOPUS:85081545321
SN - 2374-2437
VL - 6
SP - 685
EP - 695
JO - JAMA Oncology
JF - JAMA Oncology
IS - 5
ER -