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 (US) |
---|---|
Pages (from-to) | 685-695 |
Number of pages | 11 |
Journal | JAMA oncology |
Volume | 6 |
Issue number | 5 |
DOIs | |
State | Published - May 2020 |
Keywords
- Child, Preschool
- Female
- Humans
- Infant
- Male
- Retinoblastoma/economics
ASJC Scopus subject areas
- Oncology
- Cancer Research
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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.
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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
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AU - Bennani Guebessi, Nissrine
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AU - Matende, Ibrahim O.
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AU - Zhang, Yi
AU - Zhao, Junyang
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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.
KW - Child, Preschool
KW - Female
KW - Humans
KW - Infant
KW - Male
KW - Retinoblastoma/economics
UR - http://www.scopus.com/inward/record.url?scp=85081545321&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.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 -