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双侧视网膜母细胞瘤:临床表现、管理与治疗

Bilateral retinoblastoma: clinical presentation, management and treatment.

作者信息

Pichi Francesco, Lembo Andrea, De Luca Mariacarla, Hadjistilianou Theodora, Nucci Paolo

机构信息

San Giuseppe Hospital, University Eye Clinic, Via San Vittore 12, 20123, Milan, Italy,

出版信息

Int Ophthalmol. 2013 Oct;33(5):589-93. doi: 10.1007/s10792-012-9703-5. Epub 2013 Jan 1.

Abstract

Management of retinoblastoma (Rb), the most common intraocular malignant tumor in childhood, is tailored to each individual case and based on the overall situation. We present a case of bilateral Rbs in a 4-month-old girl, referred to our center for bilateral leukocoria. In the right eye, the optic disc was partially visible, and three large foci of retinoblastoma were noted adjective in the vitreous cavity with satellite retinal detachment. The macula was obscured by the tumors. The tumor was therefore classified as group D (International classification of retinoblastoma), and thus underwent intravenous chemotherapy with the standard three-agent protocol of ifosfamide, carboplatin and etoposide (ICE protocol) delivered monthly for six cycles, regressing with a type I calcified pattern. The left eye presented three multifocal yellow-white retinal masses, with a total retinal detachment, and secondary glaucoma. The lesions were classified as group E and, therefore, taking the results obtained by Shields et al. in group E tumors into consideration, underwent enucleation. This case clearly shows that this cancer is curable if detected at a stage in which it is still contained within the retina, subretinal space or vitreous, and that the management of Rb relies on an experienced team of ocular oncologists, pediatricians and pediatric ophthalmologists working together for the single goal of saving the child's life.

摘要

视网膜母细胞瘤(Rb)是儿童最常见的眼内恶性肿瘤,其治疗方案需根据每个病例的具体情况并基于整体状况进行定制。我们报告一例4个月大双侧Rb的女童病例,该患儿因双侧白瞳症转诊至我们中心。右眼视盘部分可见,在玻璃体腔中发现三个大的视网膜母细胞瘤病灶,伴有卫星状视网膜脱离。黄斑被肿瘤遮挡。因此,该肿瘤被分类为D组(视网膜母细胞瘤国际分类),并采用异环磷酰胺、卡铂和依托泊苷的标准三联化疗方案(ICE方案)进行静脉化疗,每月一次,共六个周期,肿瘤呈I型钙化模式消退。左眼有三个多灶性黄白色视网膜肿物,伴有完全性视网膜脱离和继发性青光眼。这些病变被分类为E组,因此,考虑到Shields等人在E组肿瘤中获得的结果,该眼接受了眼球摘除术。该病例清楚地表明,如果在肿瘤仍局限于视网膜、视网膜下间隙或玻璃体的阶段被发现,这种癌症是可以治愈的,并且Rb的治疗依赖于由经验丰富的眼肿瘤学家、儿科医生和小儿眼科医生组成的团队共同努力,以实现挽救患儿生命这一单一目标。

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