Atypical intravitreal growth of retinoblastoma with a multi-branching configuration.
Atypical intravitreal growth of retinoblastoma with a multi-branching configuration.
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DOI：
10.1016/j.ajoc.2017.04.003
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发表时间：
2017-09
影响因子：
--
通讯作者：
Azuma N
中科院分区：
文献类型：
--
作者：
Nishina S;Katagiri S;Nakazawa A;Kiyotani C;Yokoi T;Azuma N
报告一个多分支结构的视网膜母细胞瘤的非典型玻璃体内生长的临床和组织病理学表现。一个7个月大的男孩被转介到我们的医院，由于白瞳孔的右眼。眼科检查发现右眼透镜后透明组织包围多分支血管。影像学检查显示小眼球、带状钙化和囊性病变。由于视网膜母细胞瘤的非典型临床特征，很难排除先天性异常，如持续性胎儿血管，我们采用角膜缘入路进行活检。术中快速病理检查导致视网膜母细胞瘤的明确诊断。右眼摘除，术后辅助化疗。免疫组化染色显示，肿瘤细胞和血管周围透明的肿瘤组织呈神经元特异性烯醇化酶和Ki-67阳性，胶质细胞酸性蛋白（GFAP）部分阳性。GFAP染色肿瘤周围透明组织血管，透镜后透明组织血管染色较淡。我们描述了一个不典型的视网膜母细胞瘤的假性持久胎儿血管与多分支的配置，扩大了视网膜母细胞瘤的临床谱。胚胎肿瘤的这种特定生长模式可能发生在视网膜发育、视网膜血管系统和玻璃体血管系统的组合中。
To report the clinical and histopathological findings of atypical intravitreal growth of a retinoblastoma with a multi-branching configuration. A 7-month-old boy was referred to our hospital due to leukocoria in the right eye. Ophthalmic examinations identified multi-branching vessels surrounded by diaphanous tissue behind the lens in the right eye. Imaging modalities showed microphthalmos, band-shaped calcification, and cystic lesions in that eye. Because it was difficult to rule out congenital anomalies such as persistent fetal vasculature due to the atypical clinical features of retinoblastoma, we performed a biopsy using a limbal approach. An intraoperative rapid pathological examination led to the definitive diagnosis of retinoblastoma. The right eye was enucleated and postoperative adjuvant chemotherapy was administered. Immunohistochemical staining of the enucleated eyeball showed that the tumoral cells and diaphanous tumoral tissue around the vessels were positive for neuron-specific enolase and Ki-67 and partially positive for glial fibrillary acidic protein (GFAP). The vessels of the diaphanous tissues near the tumoral mass were stained by GFAP and those behind the lens were stained faintly. We described an atypical retinoblastoma of pseudo-persistent fetal vasculature with a multi-branching configuration, which expanded the clinical spectrum of retinoblastoma. Such a specific growth pattern of the embryonic tumor might occur with a combination of retinal development, retinal vasculature, and hyaloid vascular system.