Skip to main content
SpringerLink
Log in

Clinical analysis of 2790 children with retinoblastoma: a single-center experience in China

  • Original Article
  • Published:
World Journal of Pediatrics Aims and scope Submit manuscript

Abstract

Background

In this study, we aimed to analyze the clinical characteristics and prognosis of children with retinoblastoma (RB) in a single center in China with a large sample collection spanning 17 years.

Methods

The clinical data of 2790 children with RB treated in Beijing Tongren Hospital from 2005 to 2021 were collected, and a retrospective analysis was conducted.

Results

The median age of the participants was 28.3 months. There were 3624 affected eyes, 12.4% of which were in groups A–C, 67.1% in groups D–E and 16.2% were not specified. The primary symptom observed in most cases was a white pupil, accounting for 66.5%, followed by strabismus (12.8%). The median follow-up time was 59.7 months. The enucleation rate was 71.3% (703/986) in a single left eye and 72.5% (702/968) in a single right eye. The overall survival (OS) rate was 95.8% (2444/2552) because 237 patients dropped out, and 109 died. Kaplan‒Meier survival analysis showed that the median survival time (MST) was 125.92 months [95% confidence interval (CI) = 124.83–127.01]. Cox multivariate survival analysis showed that trilateral RB (P = 0.017), metastasis site (P = 0.001), and combined distant tissue metastasis (P = 0.001) were independent prognostic factors for RB. The OS of 44 cases of familial RB was 93.2% (41/44), with an MST of 80.62 months (95% CI = 67.70–93.54).

Conclusions

The timing of eye protection treatment and enucleation should be comprehensively judged to avoid worsening prognosis due to operation time delay. More importantly, the promotion and popularization of diagnosis and treatment technologies are necessary to further improve RB prognosis.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3
Fig. 4

Similar content being viewed by others

Data availability

The analyzed data in the current study are available from the corresponding author on reasonable request.

References

  1. AlAli A, Kletke S, Gallie B, Lam WC. Retinoblastoma for pediatric ophthalmologists. Asia Pac J Ophthalmol (Phila). 2018;7:160–8.

    PubMed  Google Scholar 

  2. Soliman SE, Racher H, Zhang C, MacDonald H, Gallie BL. Genetics and molecular diagnostics in retinoblastoma—an update. Asia Pac J Ophthalmol (Phila). 2017;6:197–207.

    CAS  PubMed  Google Scholar 

  3. Soliman SE, Dimaras H, Souka AA, Ashry MH, Gallie BL. Socioeconomic and psychological impact of treatment for unilateral intraocular retinoblastoma. J Fr Ophtalmol. 2015;38:550–8.

    Article  CAS  PubMed  Google Scholar 

  4. Dimaras H, Corson TW, Cobrinik D, White A, Zhao J, Munier FL, et al. Retinoblastoma. Nat Rev Dis Primers. 2015;1:15021.

    Article  PubMed  PubMed Central  Google Scholar 

  5. Dimaras H, Kimani K, Dimba EA, Gronsdahl P, White A, Chan HS, et al. Retinoblastoma. Lancet. 2012;379:1436–46.

    Article  PubMed  Google Scholar 

  6. Kivelä T. The epidemiological challenge of the most frequent eye cancer: retinoblastoma, an issue of birth and death. Br J Ophthalmol. 2009;93:1129–31.

    Article  PubMed  Google Scholar 

  7. Huang DS, Zhang Y. Retinoblastoma in children. Chin J Pract Pediatr. 2018;33:757–62 (in Chinese).

    Google Scholar 

  8. Bowman RJ, Mafwiri M, Luthert P, Luande J, Wood M. Outcome of retinoblastoma in east Africa. Pediatr Blood Cancer. 2008;50:160–2.

    Article  CAS  PubMed  Google Scholar 

  9. Lo JC, Rodriguez C, Monestel R, Zúñiga A. Clinical, epidemiological, and therapeutic profile of patients with a retinoblastoma diagnosis experience in the Costa Rica National Children’s Hospital Oncology Unit from January 2009 to December 2015. Ecancermedicalscience. 2017;11:754.

    PubMed  PubMed Central  Google Scholar 

  10. Maccarthy A, Draper GJ, Steliarova-Foucher E, Kingston JE. Retinoblastoma incidence and survival in European children (1978–1997). Report from the Automated Childhood Cancer Information System project. Eur J Cancer. 2006;42:2092–102.

    Article  CAS  PubMed  Google Scholar 

  11. Nyamori JM, Kimani K, Njuguna MW, Dimaras H. The incidence and distribution of retinoblastoma in Kenya. Br J Ophthalmol. 2012;96:141–3.

    Article  PubMed  Google Scholar 

  12. Leal-Leal C, Flores-Rojo M, Medina-Sansón A, Cerecedo-Díaz F, Sánchez-Félix S, González-Ramella O, et al. A multicentre report from the Mexican Retinoblastoma Group. Br J Ophthalmol. 2004;88:1074–7.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  13. Rodrigues KE, Latorre Mdo R, de Camargo B. Delayed diagnosis in retinoblastoma. J Pediatr (Rio J). 2004;80:511–6 (in Portuguese).

    Article  PubMed  Google Scholar 

  14. Canadian Retinoblastoma Society. National retinoblastoma strategy Canadian guidelines for care: strategie therapeutique du retinoblastome guide clinique canadien. Can J Ophthalmol. 2009;44(Suppl 2):S1–88.

    Google Scholar 

  15. Menon BS, Alagaratnam J, Juraida E, Mohamed M, Ibrahim H, Naing NN. Late presentation of retinoblastoma in Malaysia. Pediatr Blood Cancer. 2009;52:215–7.

    Article  PubMed  Google Scholar 

  16. Zhang S, Xin H, Li Y, Zhang D, Shi J, Yang J, et al. Skimmin, a coumarin from hydrangea paniculata, slows down the progression of membranous glomerulonephritis by anti-inflammatory effects and inhibiting immune complex deposition. Evid Based Complement Alternat Med. 2013;2013:819296.

    PubMed  PubMed Central  Google Scholar 

  17. Zhang Y, Huang X, Liu J, Chen G, Liu C, Zhang S, et al. New insight into long non-coding RNAs associated with bone metastasis of breast cancer based on an integrated analysis. Cancer Cell Int. 2021;21:372.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  18. Luo Y, Zhou C, He F, Fan J, Wen X, Ding Y, et al. Contemporary update of retinoblastoma in China: three-decade changes in epidemiology, clinical features, treatments, and outcomes. Am J Ophthalmol. 2022;236:193–203.

    Article  PubMed  Google Scholar 

  19. Zhang Y, Wang Y, Huang D, Ma J, Zhang W, Gu H, et al. Correlation between family RB1 gene pathogenic variant with clinical features and prognosis of retinoblastoma under 5 years old. Dis Markers. 2021;2021:9981028.

    PubMed  PubMed Central  Google Scholar 

  20. Wang YZ, Zhang Y, Huang DS, Shi JT, Ma JM, Li B, et al. Clinical characteristics, treatment and prognosis of children with unilateral retinoblastoma and intracranial segment of Retrobulbar optic nerve invasion. BMC Ophthalmol. 2021;21:38.

    Article  PubMed  PubMed Central  Google Scholar 

  21. Knudson AG Jr. Mutation and cancer: statistical study of retinoblastoma. Proc Natl Acad Sci U S A. 1971;68:820–3.

    Article  PubMed  PubMed Central  Google Scholar 

  22. Rushlow D, Piovesan B, Zhang K, Prigoda-Lee NL, Marchong MN, Clark RD, et al. Detection of mosaic RB1 mutations in families with retinoblastoma. Hum Mutat. 2009;30:842–51.

    Article  CAS  PubMed  Google Scholar 

  23. Roelofs K, Shaikh F, Astle W, Gallie BL, Soliman SE. Incidental neuroblastoma with bilateral retinoblastoma: what are the chances? Ophthalmic Genet. 2018;39:410–3.

    Article  PubMed  Google Scholar 

  24. Global Retinoblastoma Study Group, Fabian ID, Abdallah E, Abdullahi SU, Abdulqader RA, Adamou Boubacar S, et al. Global retinoblastoma presentation and analysis by national income level. JAMA Oncol. 2020;6:685–95.

    Article  Google Scholar 

  25. Zhang Y, Zhang WL, Huang DS, Wang YZ, Meng X. Diagnosis, treatment and prognosis of secondary leukemia in children with malignant solid tumor. Chin J Obset Gynecol Pediatr. 2020;16:67–73 (in Chinese).

    CAS  Google Scholar 

  26. Morton LM, Dores GM, Schonfeld SJ, Linet MS, Sigel BS, Lam CJK, et al. Association of chemotherapy for solid tumors with development of therapy-related myelodysplastic syndrome or acute myeloid leukemia in the modern era. JAMA Oncol. 2019;5:318–25.

    Article  PubMed  Google Scholar 

Download references

Funding

None.

Author information

Authors and Affiliations

  1. Department of Pediatrics, Beijing Tongren Hospital, Capital Medical University, Beijing 100176, China

    Yi Zhang, Yi-Zhuo Wang, Wei-Ling Zhang, Hua-Li Gu, Yan Zhou, Yan-Yan Mei, Song Li, Ting-Ting Liu, Hui-Min Hu, Tian Zhi & Dong-Sheng Huang

  2. Department of Ophthalmic Oncology, Beijing Tongren Hospital, Capital Medical University, Beijing 100176, China

    Ji-Tong Shi, Jian-Min Ma, Li-Bin Jiang, Hong-Shu Zhao & Xin Ge

  3. Department of Ophthalmic Pathology, Beijing Tongren Hospital, Capital Medical University, Beijing 100176, China

    Bin Li

Authors
  1. Yi Zhang
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Yi-Zhuo Wang
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Ji-Tong Shi
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Jian-Min Ma
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Bin Li
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Wei-Ling Zhang
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Hua-Li Gu
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Yan Zhou
    View author publications

    You can also search for this author in PubMed Google Scholar

  9. Yan-Yan Mei
    View author publications

    You can also search for this author in PubMed Google Scholar

  10. Song Li
    View author publications

    You can also search for this author in PubMed Google Scholar

  11. Ting-Ting Liu
    View author publications

    You can also search for this author in PubMed Google Scholar

  12. Li-Bin Jiang
    View author publications

    You can also search for this author in PubMed Google Scholar

  13. Hong-Shu Zhao
    View author publications

    You can also search for this author in PubMed Google Scholar

  14. Xin Ge
    View author publications

    You can also search for this author in PubMed Google Scholar

  15. Hui-Min Hu
    View author publications

    You can also search for this author in PubMed Google Scholar

  16. Tian Zhi
    View author publications

    You can also search for this author in PubMed Google Scholar

  17. Dong-Sheng Huang
    View author publications

    You can also search for this author in PubMed Google Scholar

Contributions

ZY, WYZ, SJT and MJM contributed equally to this work. ZY contributed to the conception and design of the study, the acquisition and analysis of data, and drafted the manuscript. WYZ, SJT, MJM, LB, ZWL, GLH, ZY, and MYY were responsible for the acquisition and analysis of data. LS, LTT, JLB, ZHS, GX, HHM, and ZT conducted data check and statistical analysis. HDS contributed to the conception and design of the study, and revised and commented on the draft. This study was completed with the assistance and approval of all authors.

Corresponding author

Correspondence to Dong-Sheng Huang.

Ethics declarations

Ethical approval

This study was approved by the Medical Ethics Committee of Beijing Tongren Hospital (approved number: 2020-06-BJRRB-ZY).

Conflict of interest

No financial or non-financial benefits have been received or will be received from any party related directly or indirectly to the subject of this article. The authors have no conflict of interest to declare.

Additional information

Publisher's Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Supplementary Information

Below is the link to the electronic supplementary material.

Supplementary file 2 (MP4 13058 KB)

Supplementary file 3 (PDF 440 KB)

Rights and permissions

Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law.

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Zhang, Y., Wang, YZ., Shi, JT. et al. Clinical analysis of 2790 children with retinoblastoma: a single-center experience in China. World J Pediatr 19, 1169–1180 (2023). https://doi.org/10.1007/s12519-023-00719-5

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s12519-023-00719-5

Keywords

Search

Navigation