Karim M M, Yamamoto M, Itoh H
Department of Ophthalmology, Kobe University School of Medicine.
Kobe J Med Sci. 1996 Jun;42(3):163-72.
A male child of 1-year, 6-month-old had a history of leukocoria of the left eye for approximately three months prior to admission into our facility. No abnormality was found in the right eye. There was negative family history of retinoblastoma. Ophthalmoscopy revealed a white mass extending from the supero-posterior part of the left globe to the posterior surface of the lens. Orbital tomograms showed no intraocular tissue densities in the mass but a B-scan ultrasound showed an echo dense area of the mass. Histopathologically areas of photoreceptor differentiation and Flexner-Wintersteiner rosettes were observed in the tumor mass by light microscope. Transmission electron microscopy disclosed the presence of prominent mitochondria on the luminal side of the cells forming rosettes and these were believed to be the component parts of the inner-segments of the photoreceptor cells. The results of this study indicate a predominant neuronal nature of the neoplastic cells with photoreceptor-like differentiation.
一名1岁6个月大的男童在入院前约三个月有左眼白瞳症病史。右眼未发现异常。视网膜母细胞瘤家族史为阴性。检眼镜检查发现一个白色肿物，从左眼眼球的后上部分延伸至晶状体后表面。眼眶断层扫描显示肿物内无眼内组织密度影，但B超显示肿物有回声密集区。组织病理学检查显示，在肿瘤块中，光镜下观察到光感受器分化区域和Flexner-Wintersteiner菊形团。透射电子显微镜显示，在形成菊形团的细胞腔面存在突出的线粒体，这些线粒体被认为是光感受器细胞内段的组成部分。本研究结果表明，肿瘤细胞具有主要的神经元性质，并伴有光感受器样分化。
