Atwine Raymond, Laffita Damaris, Birungi Abraham, Kiconco Ritah, Waddell Keith
Pathology, Mbarara University of Science and Technology, Mbarara, UGA.
Pathology, Angel Arturo Aballi Hospital, Havana, CUB.
Cureus. 2023 Dec 15;15(12):e50605. doi: 10.7759/cureus.50605. eCollection 2023 Dec.
Retinoblastoma (RB) is a malignant tumour that develops from the immature cells of the retina. It is the most frequent type of paediatric intraocular cancer and is curable. Clinical and histological findings after enucleation of the affected eye dictate not only the patient's secondary care but also their prognosis. We assessed the clinical and histopathologic predictors of survival among children with RB from two tertiary health facilities in Uganda.
This retrospective research utilized archived formalin fixed and paraffin-embedded blocks of eye specimens enucleated between 2014 and 2016 at Mbarara University of Science and Technology (MUST) Pathology Department and Ruharo Eye Centre (REC) in Mbarara, Uganda. The specimens were then processed and stained with haematoxylin and eosin. The confirmation of RB was made to include the histologic stage and features of the tumor. Biographic data of the patients and clinical features, such as leukocoria, proptosis, phthisis, staphyloma and buphthalmos, were retrieved from the records.
Males (55.1%, n=43) dominated the study population (N=78). The median age was 31 months. The most common clinical sign was leukocoria (69.2%, n=52), and the most predominant histopathological stage was stage 1 (41%, n=32). Optic nerve (ON) invasion was seen in 38.5% (n=30), choroidal invasion in 29.5% (n=23), scleral invasion in 7.7% (n=6) and orbital extension in 16.7% (n=13) of the cases. Flexner-Wintersteiner rosettes were seen in 34.6% (n=27). Necrosis was a prominent feature (71.8%, n=56). The two-year survival was estimated to be 61.5% (n=48). Leukocoria (risk ratio (RR) 1.1), female gender (RR 1.4), intralaminar ON invasion (RR 7.6) and a lack of orbital extension (RR 7) were significant predictors of survival.
Leukocoria and proptosis are noticeable clinical signs of RB. Most patients present while in stage one although stage four presentation is also common. Leukocoria, ON invasion, orbital extension and gender are significant factors predictive of survival in patients with RB.
视网膜母细胞瘤（RB）是一种起源于视网膜未成熟细胞的恶性肿瘤。它是最常见的小儿眼内癌类型，且可治愈。患眼摘除后的临床和组织学检查结果不仅决定患者的二级护理，还决定其预后。我们评估了乌干达两家三级医疗机构中RB患儿生存的临床和组织病理学预测因素。
这项回顾性研究利用了2014年至2016年期间在乌干达姆巴拉拉科学技术大学（MUST）病理科和姆巴拉拉鲁哈罗眼科中心（REC）存档的经福尔马林固定和石蜡包埋的眼标本块。然后对标本进行处理，并用苏木精和伊红染色。RB的确诊包括肿瘤的组织学分期和特征。从记录中检索患者的传记资料以及临床特征，如白瞳症、眼球突出、眼球痨、巩膜葡萄肿和牛眼。
男性（55.1%，n = 43）在研究人群（N = 78）中占主导。中位年龄为31个月。最常见的临床体征是白瞳症（69.2%，n = 52），最主要的组织病理学分期是1期（41%，n = 32）。38.5%（n = 30）的病例可见视神经（ON）侵犯，29.5%（n = 23）可见脉络膜侵犯，7.7%（n = 6）可见巩膜侵犯，16.7%（n = 13）可见眶内扩展。34.6%（n = 27）的病例可见Flexner-Wintersteiner菊形团。坏死是一个突出特征（71.8%，n = 56）。两年生存率估计为61.5%（n = 48）。白瞳症（风险比（RR）1.1）、女性（RR 1.4）、层内ON侵犯（RR 7.6）和无眶内扩展（RR 7）是生存的重要预测因素。
白瞳症和眼球突出是RB明显的临床体征。大多数患者就诊时处于1期，不过4期就诊也很常见。白瞳症、ON侵犯、眶内扩展和性别是RB患者生存的重要预测因素。
