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Retinoblastoma: Incidence and Etiologic Factors

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Clinical Ophthalmic Oncology

Abstract

Although the molecular etiology of retinoblastoma is well understood, our knowledge of the role of the underlying risk and origin of the events leading to lack of functional pRb remains limited though more recent findings suggest intriguing aspects to examine further. Our understanding of the role of environmental exposures during retinal development, though preliminary, appears to suggest a greater role during early eye development. We present global incidence organized by UN-defined regions using data from the recent IARC International Incidence of Childhood Cancer. The international variation in incidence suggests that nongenetic risk factors for the development of retinoblastoma may exist. Recent studies have focused on exposures occurring during critical time periods (before or after conception) and individual at risk (father vs. mother or child) for initiation of sporadic heritable and nonheritable retinoblastoma. Other studies have used cross-linked pollution indices, cancer registry, and birth registry data to examine the role of environmental exposures. Epidemiologic studies are now increasingly accounting for molecular differences in order to investigate events that surround the critical time period in the individuals at risk. Such studies will improve our understanding of disease development, risk factors, and potential strategies for prevention or for more effective treatment of retinoblastoma.

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Author information

Authors and Affiliations

  1. Department of Epidemiology, Mailman School of Public Health, Columbia University Medical Center, New York, NY, USA

    Manuela Orjuela-Grimm & Silvia Bhatt-Carreño

  2. School of Medicine, Case Western University, Cleveland, OH, USA

    Nakul Singh

  3. Department of Ophthalmic Oncology, Cole Eye Institute, Cleveland Clinic, Cleveland, OH, USA

    Arun D. Singh

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  1. Manuela Orjuela-Grimm
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  2. Nakul Singh
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  3. Silvia Bhatt-Carreño
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  4. Arun D. Singh
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Correspondence to Manuela Orjuela-Grimm .

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Editors and Affiliations

  1. Associate Professor of Ophthalmology, Children’s Hospital Los Angeles, The USC Roski Eye Institute Keck School of Medicine, Los Angeles, CA, USA

    Jesse L. Berry

  2. Children’s Hospital Los Angeles, The USC Roski Eye Institute, Keck School of Medicine, Los Angeles, CA, USA

    Jonathan W. Kim

  3. University of Oxford, Nuffield Department of Clinical Neurosciences, John Radcliffe Hospital, Oxford, UK

    Bertil E. Damato

  4. Department of Ophthalmic Oncology, Cole Eye Institute, Cleveland Clinic, Cleveland, OH, USA

    Arun D. Singh

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Orjuela-Grimm, M., Singh, N., Bhatt-Carreño, S., Singh, A.D. (2019). Retinoblastoma: Incidence and Etiologic Factors. In: Berry, J., Kim, J., Damato, B., Singh, A. (eds) Clinical Ophthalmic Oncology. Springer, Cham. https://doi.org/10.1007/978-3-030-11123-6_4

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  • DOI: https://doi.org/10.1007/978-3-030-11123-6_4

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