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感觉性视网膜和视网膜色素上皮联合错构瘤

Combined hamartoma of sensory retina and retinal pigment epithelium.

作者信息

Font R L, Moura R A, Shetlar D J, Martinez J A, McPherson A R

机构信息

Department of Ophthalmology, Cullen Eye Institute, Baylor College of Medicine, Houston, Texas 77030.

出版信息

Retina. 1989;9(4):302-11. doi: 10.1097/00006982-198909040-00011.

DOI:10.1097/00006982-198909040-00011
PMID:2697919
Abstract

We report two cases of combined hamartoma of the sensory retina and retinal pigment epithelium (CHR-RPE) in which apparent growth of the lesion was observed. In case 1, the eye was enucleated with a presumed diagnosis of juxtapapillary malignant melanoma. Histopathologically, the enucleated globe showed an elevated peripapillary mass containing disorganized retinal tissue intermixed with vascular and glial elements as well as tubules of proliferating retinal pigment epithelium. We have summarized the clinical features of 53 patients with CHR-RPE reported between 1952 and 1988 excluding the cases compiled by the Macular Society Collaborative Study. While the latter study found an equal sex predilection among their cases, we found a 70% male preponderance among the 53 patients. Of the 54 lesions observed in 53 patients, 76% were juxtapapillary, 17% were macular, and 7% were peripheral. Furthermore, periodic follow-up examination disclosed apparent enlargement of the mass in six patients, five of whom underwent enucleation of the globe for suspected melanoma.

摘要

我们报告了两例伴有视网膜感觉层和视网膜色素上皮联合错构瘤(CHR-RPE)的病例,其中观察到病变有明显生长。病例1中,患眼因疑似诊断为乳头旁恶性黑色素瘤而被摘除眼球。组织病理学检查显示,摘除的眼球出现一个乳头旁隆起性肿块,其中包含杂乱无章的视网膜组织,混杂有血管和神经胶质成分,以及增殖的视网膜色素上皮小管。我们总结了1952年至1988年间报告的53例CHR-RPE患者的临床特征(不包括黄斑学会协作研究汇编的病例)。虽然后者的研究发现其病例中男女发病率相当,但我们发现这53例患者中男性占比达70%。在53例患者中观察到的54个病变中,76%位于乳头旁,17%位于黄斑区,7%位于周边。此外,定期随访检查发现6例患者的肿块明显增大,其中5例因疑似黑色素瘤而接受了眼球摘除术。

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