Wirz K, Lee W R, Coaker T
Graefes Arch Clin Exp Ophthalmol. 1982;219(5):214-21. doi: 10.1007/BF00231238.
A disc-shaped flat, heavily pigmented tumour (5 mm diameter) was discovered at the retinal periphery in a glutaraldehyde fixed eye which was enucleated in treatment of a peripapillary melanoma in a 60 year old man. The macroscopic appearances of the peripheral lesion were identical to those described in clinical reports as "Congenital Hypertrophy of the RPE". By light microscopy and transmission electron microscopy the hamartoma consisted of a hypertrophic and hyperplastic layer of RPE cells beneath an atrophic outer retina. The hyperplastic RPE cells did not contain lipofuscin and the content and size of the abnormal melanin granules increased towards the retinal aspect. The cells were linked by complex process interdigitations and attachments; the intercellular spaces contained basement membrane material and 100 nm banded collagen. Large vacuolar structures were prominent in the cells adjacent to the normal choriocapillaris. In the RPE cells at the periphery there was an excess lipofuscin and here there were recognisable outer segments. The morphological pattern suggested that this lesion should be considered as a congenital hypertrophy of the RPE which has progressed to focal segmental hyperplasia and focal atrophy.
在一名60岁男性因视乳头周围黑色素瘤而摘除的经戊二醛固定的眼球中，在视网膜周边发现了一个盘状扁平、色素沉着严重的肿瘤（直径5毫米）。周边病变的宏观表现与临床报告中描述的“视网膜色素上皮先天性肥大”相同。通过光学显微镜和透射电子显微镜观察，错构瘤由萎缩的外层视网膜下方一层肥厚和增生的视网膜色素上皮（RPE）细胞组成。增生的RPE细胞不含脂褐素，异常黑色素颗粒的含量和大小向视网膜方向增加。细胞通过复杂的突起相互交错和连接；细胞间隙含有基底膜物质和100纳米带状胶原。在与正常脉络膜毛细血管相邻的细胞中，大的空泡结构很突出。在周边的RPE细胞中有过量的脂褐素，并且在这里可以识别出外层节段。形态学模式表明，该病变应被视为已发展为局灶性节段性增生和局灶性萎缩的RPE先天性肥大。
