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.