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Adenocarcinoma arising from congenital hypertrophy of the retinal pigment epithelium

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Abstract

Background

Adenocarcinoma of the retinal pigment epithelium (RPE) is a rare primary malignant intraocular neoplasm. We report a histopathologically confirmed case of adenocarcinoma of the retinal pigment epithelium arising from congenital hypertrophy of the retinal pigment epithelium (CHRPE).

Methods

The clinical features, surgical management, and histopathological features of a melanotic tumor arising from CHRPE are presented. The tumor was excised by transcleral resection.

Results

Histopathological and immunohistochemical study of the tumor showed it to be an adenocarcinoma of the RPE.

Conclusions

Adenocarcinoma of the RPE arising from CHRPE is extremely rare. Such a tumor can resemble a choroidal melanoma. In spite of the rarity of this association, periodic monitoring of CHRPE for development of a nodular tumor of the RPE is probably warranted.

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Acknowledgements

This work was supported in part by an Institutional Challenge Grant from Research to Prevent Blindness, Inc., New York, N.Y., to the Department of Ophthalmology of the University of Cincinnati College of Medicine (James J. Augsburger MD, Chairman) and the Department's Ophthalmology Research and Education Fund.

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Author notes

  1. Susan Schneider

    Present address: Department of Ophthalmology, The New York Eye and Ear Infirmary, New York, NY, USA

Authors and Affiliations

  1. Department of Ophthalmology, University of Cincinnati College of Medicine, Health Professions Building, Suite #350, 3223 Eden Avenue, Cincinnati, OH, 45267-0527, USA

    Nikolaos Trichopoulos, James J. Augsburger & Susan Schneider

  2. Department of Pathology, University of Cincinnati College of Medicine, Cincinnati, OH, USA

    Susan Schneider

Authors
  1. Nikolaos Trichopoulos
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  2. James J. Augsburger
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  3. Susan Schneider
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Correspondence to Nikolaos Trichopoulos.

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Trichopoulos, N., Augsburger, J.J. & Schneider, S. Adenocarcinoma arising from congenital hypertrophy of the retinal pigment epithelium. Graefe's Arch Clin Exp Ophthalmo 244, 125–128 (2006). https://doi.org/10.1007/s00417-005-0011-x

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  • DOI: https://doi.org/10.1007/s00417-005-0011-x

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