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Management and Outcomes of Retinoblastoma Cases Presenting to Children’s Hospital Westmead, Sydney Between 2008 and 2018

Received: 30 November 2021     Accepted: 17 December 2021     Published: 29 December 2021
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Abstract

This retrospective review reports on the management and outcomes of retinoblastoma in children treated at Children’s Hospital Westmead (CHW), Sydney. Results were compared to those of a previous retrospective review of RB cases presenting between 1974 and 2005 at the same centre, which was published in this journal. A retrospective review of all cases of retinoblastoma presenting to the Children’s Hospital Westmead Medical between 2008 and 2018 was conducted. 67 patients were included in the study with a mean age at presentation of 23.5-months and 9.2-months for unilateral and bilateral disease respectively. All patients in our cohort were offered genetic testing. The rate of germline RB1 mutation in our cohort was 29% for unilateral disease and 86% for bilateral disease. Mean follow-up period was 48 months. Globe salvage rates in patients with bilateral disease was 57%, compared to the previous study which was 47%. The most common treatment-related ocular complication was strabismus. Our cohort had only one patient develop metastatic disease and one patient who presented with trilateral disease, which was a case of delayed presentation and was the only mortality in the study. Morbidity and mortality rates in our cohort are on par with other tertiary centres internationally. There has been a significant improvement in globe salvage rate with our current management protocol. As intra-arterial chemotherapy is implemented into the treatment regime at CHW, these results will provide a benchmark to ensure that the excellent standards of care and outcomes are maintained.

Published in International Journal of Ophthalmology & Visual Science (Volume 6, Issue 4)
DOI 10.11648/j.ijovs.20210604.24
Page(s) 273-280
Creative Commons

This is an Open Access article, distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution and reproduction in any medium or format, provided the original work is properly cited.

Copyright

Copyright © The Author(s), 2021. Published by Science Publishing Group

Keywords

Retinoblastoma, Chemotherapy, Enucleation

References
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Cite This Article
  • APA Style

    Julia Mary Starte, Amir Taher, Madeleine Powys, Craig Donaldson, Luciano Dalla-Pozza, et al. (2021). Management and Outcomes of Retinoblastoma Cases Presenting to Children’s Hospital Westmead, Sydney Between 2008 and 2018. International Journal of Ophthalmology & Visual Science, 6(4), 273-280. https://doi.org/10.11648/j.ijovs.20210604.24

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    ACS Style

    Julia Mary Starte; Amir Taher; Madeleine Powys; Craig Donaldson; Luciano Dalla-Pozza, et al. Management and Outcomes of Retinoblastoma Cases Presenting to Children’s Hospital Westmead, Sydney Between 2008 and 2018. Int. J. Ophthalmol. Vis. Sci. 2021, 6(4), 273-280. doi: 10.11648/j.ijovs.20210604.24

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    AMA Style

    Julia Mary Starte, Amir Taher, Madeleine Powys, Craig Donaldson, Luciano Dalla-Pozza, et al. Management and Outcomes of Retinoblastoma Cases Presenting to Children’s Hospital Westmead, Sydney Between 2008 and 2018. Int J Ophthalmol Vis Sci. 2021;6(4):273-280. doi: 10.11648/j.ijovs.20210604.24

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  • @article{10.11648/j.ijovs.20210604.24,
      author = {Julia Mary Starte and Amir Taher and Madeleine Powys and Craig Donaldson and Luciano Dalla-Pozza and Bhavna Padhye and Robyn Jamieson and Michael Murray Jones},
      title = {Management and Outcomes of Retinoblastoma Cases Presenting to Children’s Hospital Westmead, Sydney Between 2008 and 2018},
      journal = {International Journal of Ophthalmology & Visual Science},
      volume = {6},
      number = {4},
      pages = {273-280},
      doi = {10.11648/j.ijovs.20210604.24},
      url = {https://doi.org/10.11648/j.ijovs.20210604.24},
      eprint = {https://article.sciencepublishinggroup.com/pdf/10.11648.j.ijovs.20210604.24},
      abstract = {This retrospective review reports on the management and outcomes of retinoblastoma in children treated at Children’s Hospital Westmead (CHW), Sydney. Results were compared to those of a previous retrospective review of RB cases presenting between 1974 and 2005 at the same centre, which was published in this journal. A retrospective review of all cases of retinoblastoma presenting to the Children’s Hospital Westmead Medical between 2008 and 2018 was conducted. 67 patients were included in the study with a mean age at presentation of 23.5-months and 9.2-months for unilateral and bilateral disease respectively. All patients in our cohort were offered genetic testing. The rate of germline RB1 mutation in our cohort was 29% for unilateral disease and 86% for bilateral disease. Mean follow-up period was 48 months. Globe salvage rates in patients with bilateral disease was 57%, compared to the previous study which was 47%. The most common treatment-related ocular complication was strabismus. Our cohort had only one patient develop metastatic disease and one patient who presented with trilateral disease, which was a case of delayed presentation and was the only mortality in the study. Morbidity and mortality rates in our cohort are on par with other tertiary centres internationally. There has been a significant improvement in globe salvage rate with our current management protocol. As intra-arterial chemotherapy is implemented into the treatment regime at CHW, these results will provide a benchmark to ensure that the excellent standards of care and outcomes are maintained.},
     year = {2021}
    }
    

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  • TY  - JOUR
    T1  - Management and Outcomes of Retinoblastoma Cases Presenting to Children’s Hospital Westmead, Sydney Between 2008 and 2018
    AU  - Julia Mary Starte
    AU  - Amir Taher
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    AB  - This retrospective review reports on the management and outcomes of retinoblastoma in children treated at Children’s Hospital Westmead (CHW), Sydney. Results were compared to those of a previous retrospective review of RB cases presenting between 1974 and 2005 at the same centre, which was published in this journal. A retrospective review of all cases of retinoblastoma presenting to the Children’s Hospital Westmead Medical between 2008 and 2018 was conducted. 67 patients were included in the study with a mean age at presentation of 23.5-months and 9.2-months for unilateral and bilateral disease respectively. All patients in our cohort were offered genetic testing. The rate of germline RB1 mutation in our cohort was 29% for unilateral disease and 86% for bilateral disease. Mean follow-up period was 48 months. Globe salvage rates in patients with bilateral disease was 57%, compared to the previous study which was 47%. The most common treatment-related ocular complication was strabismus. Our cohort had only one patient develop metastatic disease and one patient who presented with trilateral disease, which was a case of delayed presentation and was the only mortality in the study. Morbidity and mortality rates in our cohort are on par with other tertiary centres internationally. There has been a significant improvement in globe salvage rate with our current management protocol. As intra-arterial chemotherapy is implemented into the treatment regime at CHW, these results will provide a benchmark to ensure that the excellent standards of care and outcomes are maintained.
    VL  - 6
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Author Information
  • Department of Ophthalmology, Children’s Hospital Westmead, Sydney, Australia

  • Department of Ophthalmology, Children’s Hospital Westmead, Sydney, Australia

  • Department of Oncology, Children’s Hospital Westmead, Sydney, Australia

  • Department of Ophthalmology, Children’s Hospital Westmead, Sydney, Australia

  • Department of Oncology, Children’s Hospital Westmead, Sydney, Australia

  • Department of Oncology, Children’s Hospital Westmead, Sydney, Australia

  • Department of Genetics, Children’s Hospital Westmead, Sydney, Australia

  • Department of Ophthalmology, Children’s Hospital Westmead, Sydney, Australia

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