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Retinal Pigment Epithelial Tumors

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Clinical Ophthalmic Oncology

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Abstract

Tumors of the retinal pigment epithelium (RPE) can be congenital or acquired. They may also be classified as reactive, hypertrophic, hamartomatous, or neoplastic. Those present at birth can be associated with systemic conditions such as familial adenomatous polyposis (FAP) or neurofibromatosis 2 (NF2). While most congenital tumors have quite characteristic clinical features that allow their accurate diagnosis, acquired RPE tumors are sometimes difficult to differentiate from choroidal neoplasms were it not for ancillary tests such as ultrasonography, optical coherence tomography, and fluorescein angiography. This chapter reviews the clinical features of congenital and acquired tumors of the RPE and their systemic associations.

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Author information

Authors and Affiliations

  1. Department of Pediatric Ophthalmology and Strabismus, Center for Genetic Eye Diseases, Cole Eye Institute (i-32), Cleveland Clinic Foundation, Cleveland, OH, USA

    Elias I. Traboulsi

  2. Department of Pediatric Ophthalmology and Strabismus, Center for Genetic Eye Diseases, Cole Eye Institute, Cleveland Clinic Foundation, Cleveland, OH, USA

    Matteo Scaramuzzi

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

    Arun D. Singh

Authors
  1. Elias I. Traboulsi
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  2. Matteo Scaramuzzi
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  3. Arun D. Singh
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Corresponding author

Correspondence to Elias I. Traboulsi .

Editor information

Editors and Affiliations

  1. Cleveland Clinic Foundation, Cole Eye Institute, Cleveland Clinic Foundation, Cleveland, OH, USA

    Arun D. Singh

  2. University of Oxford, Oxford, UK

    Bertil E. Damato

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Traboulsi, E.I., Scaramuzzi, M., Singh, A.D. (2019). Retinal Pigment Epithelial Tumors. In: Singh, A., Damato, B. (eds) Clinical Ophthalmic Oncology. Springer, Cham. https://doi.org/10.1007/978-3-030-04113-7_5

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  • DOI: https://doi.org/10.1007/978-3-030-04113-7_5

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