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Adenocarcinoma Arising From Congenital Hypertrophy of Retinal Pigment Epithelium
Jerry A. Shields, MD;
Carol L. Shields, MD;
Ralph C. Eagle, Jr, MD;
Arun D. Singh, MD
Arch Ophthalmol. 2001;119:597-602.
Congenital hypertrophy of the retinal pigment epithelium (CHRPE), traditionally
regarded as a benign stationary condition, has recently been shown in 5 cases
to give rise to an elevated, solid tumor. However, the histopathologic nature
of the tumor that arises from CHRPE has not been previously determined. A
65-year-old woman developed a progressively enlarging peripheral fundus tumor
that arose from a focus of classic CHRPE. The tumor produced a localized exudative
retinal detachment, cystoid macular edema, and surface-wrinkling retinopathy.
The mass was removed by local resection, and histopathologic examination revealed
a low-grade adenocarcinoma of the retinal pigment epithelium, apparently arising
from CHRPE. Although CHRPE is usually a benign nonprogressive lesion, it can
give rise to a malignant tumor. Congenital hypertrophy of the retinal pigment
epithelium should be observed periodically for development of a neoplasm.
From the Oncology Service (Drs J. Shields, C. Shields, and Singh),
Pathology Department (Dr Eagle), Wills Eye Hospital, Thomas Jefferson University,
Philadelphia, Pa.
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