Shields Jerry A, Eagle Ralph C, Shields Carol L, Brown Gary C, Lally Sara E
Ocular Oncology Service, Wills Eye Institute, Thomas Jefferson University, Philadelphia, Pennsylvania 19107, USA.
Ophthalmology. 2009 Nov;116(11):2213-6. doi: 10.1016/j.ophtha.2009.04.048. Epub 2009 Sep 10.
To report a clinicopathologic correlation of an adenocarcinoma that arose from solitary congenital hypertrophy of the retinal pigment epithelium (CHRPE).
Case report with clinicopathologic correlation.
A 56-year-old black woman was referred for an asymptomatic fundus lesion in her left eye. It had features typical of CHRPE, but there was a small elevated nodule within the flat component, and the diagnosis was adenoma of the retinal pigment epithelium (RPE) arising from CHRPE. The lesion was observed periodically. The nodule slowly enlarged, and increasing amounts of lipoproteinaceous exudation accumulated in the adjacent retina. Thirteen years after the initial diagnosis, the patient returned with severe visual loss and no view of the fundus resulting from cataract and posterior synechia. Ultrasonography revealed a total retinal detachment and a pedunculated tumor measuring 7.5 mm in thickness. The eye was enucleated and studied histopathologically.
Clinical evaluation and correlation of clinical findings with histopathologic results of the enucleated eye.
Histopathologically, the mass was composed of a proliferation of atypical RPE cells with a marked infiltration of benign plasma cells. Typical features of CHRPE were present at the base of the tumor. The final diagnosis was adenocarcinoma arising from CHRPE.
Congenital hypertrophy of the retinal pigment epithelium, once considered to be a benign and stationary lesion, may spawn a malignant neoplasm.
报告一例起源于孤立性先天性视网膜色素上皮肥大（CHRPE）的腺癌的临床病理相关性。
临床病理相关性病例报告。
一名56岁黑人女性因左眼无症状眼底病变前来就诊。其具有CHRPE的典型特征，但在扁平部分内有一个小的隆起结节，诊断为起源于CHRPE的视网膜色素上皮（RPE）腺瘤。对该病变进行定期观察。结节逐渐增大，相邻视网膜内积聚的脂蛋白性渗出物增多。初次诊断13年后，患者因白内障和虹膜后粘连导致严重视力丧失且无法观察眼底而复诊。超声检查显示完全性视网膜脱离和一个厚度为7.5 mm的带蒂肿瘤。该眼行眼球摘除术并进行组织病理学研究。
临床评估以及临床发现与眼球摘除组织病理学结果的相关性。
组织病理学检查显示，肿块由非典型RPE细胞增生构成，并伴有大量良性浆细胞浸润。肿瘤底部存在CHRPE的典型特征。最终诊断为起源于CHRPE的腺癌。
视网膜色素上皮先天性肥大，曾被认为是一种良性静止性病变，可能会产生恶性肿瘤。
