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Retinoblastoma: Epidemiologic Aspects

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Clinical Ophthalmic Oncology

Abstract

Retinoblastoma is the paradigm for the two-hit model of carcinogenesis [1]. From a genetic standpoint, three forms may be considered: familial, sporadic heritable, and nonheritable retinoblastoma (Fig. 4.1). These three forms are thought to account for most instances of retinoblastoma. However, findings on imprinting and mosaicism indicate that our understanding of the genetics of this disease is still evolving and that the genetics are more complex than indicated by our discussion of the three main forms [2–5]. In the discussion that follows, we use the proportions of retinoblastomas of each form seen in industrialized countries. In developing countries, nonheritable retinoblastoma accounts for a larger proportion.

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Author information

Authors and Affiliations

  1. Division of Oncology, Department of Pediatrics, Children’s Hospital of Philadelphia, 3535 Market St., Rm 1472, Philadelphia, PA, 19027, USA

    Greta R. Bunin PhD

  2. Department of Clinical Public Health (Environmental Health Sciences) and Clinical Pediatrics (Pediatric Oncology), Columbia University and The Children’s Hospital of New York-Presbyterian, Columbia University Medical Center, New York, NY, USA

    Manuela Orjuela MD, ScM

  3. Environmental Health Sciences, Mailman School of Public Health, 60 Haven Avenue, Rm B 106, New York, NY, 10032, USA

    Manuela Orjuela MD, ScM

Authors
  1. Greta R. Bunin PhD
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  2. Manuela Orjuela MD, ScM
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Corresponding author

Correspondence to Greta R. Bunin PhD .

Editor information

Editors and Affiliations

  1. Dept. of Ophthalmic Oncology, Cole Eye Institute Cleveland Clinic Foundation, Cleveland, Ohio, USA

    Arun D. Singh

  2. Department of Ophthalmology, Children's Hospital Los Angeles, Los Angeles, USA

    A. Linn Murphree

  3. University of California, San Francisco, California, USA

    Bertil E. Damato

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Bunin, G.R., Orjuela, M. (2015). Retinoblastoma: Epidemiologic Aspects. In: Singh, A., Murphree, A., Damato, B. (eds) Clinical Ophthalmic Oncology. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-43451-2_4

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  • DOI: https://doi.org/10.1007/978-3-662-43451-2_4

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