Fang Xiaolian, Wang Huanmin, Ma Xiaoli, Guo Yongli, Yang Wei, Hu Shoulong, Qiu Yue, Zhao Junyang, Ni Xin
Department of Otolaryngology Head and Neck Surgery, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China.
Department of Surgical Oncology, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China.
J Ophthalmol. 2020 Jul 10;2020:9315784. doi: 10.1155/2020/9315784. eCollection 2020.
Retinoblastoma and neuroblastoma are the most common malignant extracranial solid tumors in children. This study aimed to summarize the clinical features, especially the delayed diagnosis in children with retinoblastoma and neuroblastoma.
In a single hospital-based case-control study, a retrospective cohort of 175 children with retinoblastoma and neuroblastoma diagnosed from January 2016 to January 2018 were reviewed. The state of enucleation in retinoblastomas and pathological prognosis in neuroblastomas were outcome indicators. Hereby, the patients were divided into two groups, and clinical features including age at presentation and delayed diagnosis were compared.
A total of 112 patients with retinoblastoma and 63 with neuroblastoma were included. In the retinoblastoma cohort, the median age at presentation was 17.2 months (0.3-110 months). The mean delay of diagnosis was 1.6 ± 2.3 months, and the rate of enucleation was 61.6%. Unilateral disease, the International Classification of Intraocular Retinoblastoma (IIRC) stage E, and delay of diagnosis over 2.5 months were independent risk factors of ocular outcomes. Notably, the risk of enucleation was increased by 474% when the delay was longer than 2.5 months. In the neuroblastoma cohort, the delay of diagnosis of the unfavorable histology (UH) group was longer than that of the favorable histology (FH) group (1.9 months vs. 1.4 months, =.487). The levels of serum ferritin and neuron-specific enolase were higher in the UH group than in the FH group ( < .05).
This study summarized the clinical features and diagnosis biomarkers of retinoblastoma and neuroblastoma patients in China. These results might help to focus on early detection and treatment in children with retinoblastoma and neuroblastoma.
视网膜母细胞瘤和神经母细胞瘤是儿童最常见的颅外恶性实体瘤。本研究旨在总结视网膜母细胞瘤和神经母细胞瘤患儿的临床特征,尤其是延迟诊断情况。
在一项基于单一医院的病例对照研究中,回顾性分析了2016年1月至2018年1月期间诊断为视网膜母细胞瘤和神经母细胞瘤的175例患儿。视网膜母细胞瘤的眼球摘除情况和神经母细胞瘤的病理预后为结局指标。据此,将患者分为两组,比较包括就诊年龄和延迟诊断在内的临床特征。
共纳入112例视网膜母细胞瘤患者和63例神经母细胞瘤患者。在视网膜母细胞瘤队列中,就诊时的中位年龄为17.2个月(0.3 - 110个月)。诊断的平均延迟时间为1.6±2.3个月,眼球摘除率为61.6%。单眼病变、国际眼内视网膜母细胞瘤分类(IIRC)E期以及诊断延迟超过2.5个月是眼部结局的独立危险因素。值得注意的是,当延迟时间超过2.5个月时,眼球摘除的风险增加了474%。在神经母细胞瘤队列中,不良组织学(UH)组的诊断延迟时间长于良好组织学(FH)组(1.9个月对1.4个月,P = 0.487)。UH组血清铁蛋白和神经元特异性烯醇化酶水平高于FH组(P < 0.05)。
本研究总结了中国视网膜母细胞瘤和神经母细胞瘤患者的临床特征及诊断生物标志物。这些结果可能有助于关注视网膜母细胞瘤和神经母细胞瘤患儿的早期发现和治疗。