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Pediatric Malignancies: Pathology and Imaging pp 383–398Cite as

Intraocular Tumors

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Abstract

Intraocular tumors of childhood are rare but have great clinical importance. The commonest pediatric intraocular tumor, the retinoblastoma, has provided pivotal insights into our understanding of tumor genesis. Retinoblastoma arises in the context of multiple genetic abnormalities, including two “hits” to the RB1 gene. In inherited cases, one of these hits is carried in the germ line; such patients often have multiple tumors. Correct handling of the enucleated eye is important in providing optimal information for further management. Massive choroidal involvement and extension into the optic nerve head, especially to the surgical resection margin in the case of the latter, are adverse prognostic features. Other intraocular tumors of childhood such as astrocytic hamartoma and medulloepithelioma are even rarer than retinoblastoma but should be considered in the differential diagnosis.

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Authors and Affiliations

  1. Cellular Pathology, The Royal London Hospital, Pathology and Pharmacy Building, 80 Newark Street, London, E1 2ES, UK

    Irene Scheimberg M.D., F.R.C.Path.

  2. Radiological Sciences, St. Jude Children’s Research Hospital, 262 Danny Thomas Place, Memphis, TN, 38105, USA

    M. Beth McCarville M.D.

  3. Department of Eye Pathology, UCL Institute of Ophthalmology and Moorfields Eye Hospital, 11-43 Bath Street, London, EC1V 9EL, UK

    Philip Luthert B.Sc., M.B.B.S.

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  1. Irene Scheimberg M.D., F.R.C.Path.
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  2. M. Beth McCarville M.D.
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  3. Philip Luthert B.Sc., M.B.B.S.
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Correspondence to Irene Scheimberg M.D., F.R.C.Path. .

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Editors and Affiliations

  1. Department of Pathology and Laboratory Medicine, Children’s Hospital Los Angeles, University of Southern California, Los Angeles, California, USA

    David M. Parham

  2. Associate Professor of Pathology and Laboratory Medicine, The University of Texas M.D. Anderson Cancer Center, Houston, Texas, USA

    Joseph D. Khoury

  3. Department of Radiological Sciences, St. Jude Children's Research Hospital, Memphis, Tennessee, USA

    M. Beth McCarville

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Scheimberg, I., McCarville, M.B., Luthert, P. (2015). Intraocular Tumors. In: Parham, D., Khoury, J., McCarville, M. (eds) Pediatric Malignancies: Pathology and Imaging. Springer, New York, NY. https://doi.org/10.1007/978-1-4939-1729-7_14

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