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Trilateral Retinoblastoma

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Clinical Ophthalmic Oncology

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Abstract

The term trilateral retinoblastoma (TRB) classically refers to the association of bilateral intraocular retinoblastoma with a pineoblastoma, a primitive neuroectodermal tumor that arises in the pineal gland. There is some dispute regarding the cell of origin for TRB, and more recent studies suggest that the tumor may arise from the germinal layer of primitive cells (subependymal plate) rather than the pineal gland. For that reason, some authors refer to the intracranial tumor in retinoblastoma patients as a pineal neuroblastic tumor (PNT) rather than a pineoblastoma. The overall incidence of TRB is approximately 3% for all patients with RB, up to 5–6% for patients with bilateral retinoblastoma, and as high as 10–15% for patients with familial retinoblastoma. The average age at diagnosis of TRB is between 26 and 40 months, with a range of 1–142 months. Screening recommendations for trilateral retinoblastoma are somewhat controversial, mainly because of the low incidence of TRB and the need for anesthesia for performing magnetic resonance imaging (MRI) in this population. Screening programs should be directed to children at highest risk for TRB (bilateral disease, positive family history, germline mutation present). The overall prognosis for TRB is poor even with aggressive treatment, as patients usually die of disseminated neuraxis disease within the first year after diagnosis. Surgical resection may play a role in certain cases if the intracranial disease is not disseminated.

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Authors and Affiliations

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

    Jonathan W. Kim

  2. Department of Pediatrics, Memorial Sloan Kettering Cancer Center, New York, NY, USA

    Ira J. Dunkel

Authors
  1. Jonathan W. Kim
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  2. Ira J. Dunkel
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Correspondence to Jonathan W. Kim .

Editor information

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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Kim, J.W., Dunkel, I.J. (2019). Trilateral Retinoblastoma. 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_23

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  • DOI: https://doi.org/10.1007/978-3-030-11123-6_23

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-030-11122-9

  • Online ISBN: 978-3-030-11123-6

  • eBook Packages: MedicineMedicine (R0)

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