Global Retinoblastoma Presentation and Analysis by National Income Level

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 - Latinovic, 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 - Peric, 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

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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

AU - Global Retinoblastoma Study Group

PY - 2020/5/1

Y1 - 2020/5/1

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.

U2 - 10.1001/jamaoncol.2019.6716

DO - 10.1001/jamaoncol.2019.6716

M3 - SCORING: Journal article

C2 - 32105305

VL - 6

SP - 685

EP - 695

JO - JAMA ONCOL

JF - JAMA ONCOL

SN - 2374-2437

IS - 5

ER -