Retinoblastoma: Epidemiologic Aspects

  • Greta R. BuninEmail author
  • Manuela Orjuela


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.


Cancer Survivor Germ Line Mutation Atomic Bomb Survivor Unilateral Disease Bilateral Retinoblastoma 
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Copyright information

© Springer-Verlag Berlin Heidelberg 2015

Authors and Affiliations

  1. 1.Division of Oncology, Department of PediatricsChildren’s Hospital of PhiladelphiaPhiladelphiaUSA
  2. 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 CenterNew YorkUSA
  3. 3.Environmental Health SciencesMailman School of Public HealthNew YorkUSA

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