Adenocarcinoma arising from congenital hypertrophy of retinal pigment epithelium.
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.
Shields
J A
JA
Oncology Service, Wills Eye Hospital, 900 Walnut St, Philadelphia, PA 19107, USA.
Shields
C L
CL
Eagle
R C
RC
Singh
A D
AD
D000230:Adenocarcinoma / Q000000981:diagnostic imaging / Q000209:etiology / Q000473:pathology* / Q000601:surgery; D000368:Aged; D005260:Female; D005451:Fluorescein Angiography; D005654:Fundus Oculi; D006801:Humans; D006984:Hypertrophy; D010857:Pigment Epithelium of Eye / Q000473:pathology*; D019572:Retinal Neoplasms / Q000000981:diagnostic imaging / Q000209:etiology / Q000473:pathology* / Q000601:surgery; D014463:Ultrasonography