Diffuse Anterior Retinoblastoma with Sarcoidosis-Like Nodule.
Diffuse Anterior Retinoblastoma with Sarcoidosis-Like Nodule.
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弥漫性前视网膜母细胞瘤，带有类似类似的结节。
DOI：
10.1159/000442744
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发表时间：
2015-09
影响因子：
0.4
通讯作者：
Sotozono C
中科院分区：
文献类型：
--
作者：
Kitazawa K;Nagata K;Yamanaka Y;Kuwahara Y;Iehara T;Kinoshita S;Sotozono C
视网膜母细胞瘤占儿童恶性肿瘤的4%，其中1-2%为弥漫性浸润性视网膜母细胞瘤。弥漫性前视网膜母细胞瘤是罕见的，不涉及视网膜。在这里，我们报告了一个弥漫性前部视网膜母细胞瘤，虹膜上有大的结节病样结节，对抗炎治疗有反应。我们报告一位六岁女童，患有前葡萄膜炎，右眼虹膜及角膜后表面有白色结节。结节最初对抗炎治疗反应良好。然而，眼前节光学相干断层扫描（AS-OCT）显示结节逐渐生长，使虹膜缩小。然后，我们收集了用于诊断的房水。活检显示小细胞簇，核质比高，部分形成玫瑰花结。因此，我们诊断为弥漫性前部视网膜母细胞瘤，没有视网膜受累，并进行了右眼眼球摘除术。组织病理学显示未分化细胞与活检中所见相似，肿瘤细胞侵入虹膜基质、角膜后表面、睫状体和巩膜。摘除术后，她接受了化疗，仍然活着。当眼内出现抗炎治疗无效的白色结节时，即使没有明显的视网膜肿块，也应考虑视网膜母细胞瘤的鉴别诊断。AS-OCT结果可用于评估视网膜母细胞瘤。
Retinoblastomas account for 4% of malignancies in children, 1-2% of which are diffuse infiltrating retinoblastomas. Diffuse anterior retinoblastoma is rare and does not involve the retina. Here, we report on a diffuse anterior retinoblastoma with large sarcoidosis-like nodules on the iris that were responsive to anti-inflammatory therapy. We present a 6-year-old girl who had anterior uveitis with white nodules on the iris and posterior surface of the cornea in her right eye. The nodules initially responded well to anti-inflammatory treatment. However, anterior segment optical coherence tomography (AS-OCT) showed that the nodules gradually grew, shrinking the iris. We then collected the aqueous humor for diagnosis. A biopsy revealed clusters of small cells with a high nuclear-to-cytoplasm ratio with partial rosette formation. Therefore, we diagnosed diffuse anterior retinoblastoma without retinal involvement and performed enucleation of the right eye. The histopathology demonstrated undifferentiated cells similar to those seen on the biopsy, and tumor cells invaded the iris stroma, posterior surface of the cornea, ciliary body, and sclera. After the enucleation, she underwent chemotherapy and remains alive. A differential diagnosis of retinoblastoma should be considered when white nodules refractory to anti-inflammatory therapy occur in the eye, even in the absence of obvious retinal masses. AS-OCT findings are useful in assessing retinoblastoma.