Adenocarcinoma arising from congenital hypertrophy of the retinal pigment epithelium.
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.
Department of Ophthalmology, University of Cincinnati College of Medicine, Cincinnati, OH 45267-0527, USA. ntrichope@hotmail.com
Nikolaos
N
Trichopoulos
James J
JJ
Augsburger
Susan
S
Schneider
D000230:Adenocarcinoma / Q000000981:diagnostic imaging / Q000473:pathology*; D000328:Adult; D005260:Female; D006801:Humans; D006984:Hypertrophy / Q000151:congenital; D010857:Pigment Epithelium of Eye / Q000000981:diagnostic imaging / Q000473:pathology*; D019572:Retinal Neoplasms / Q000000981:diagnostic imaging / Q000473:pathology*; D014463:Ultrasonography