Latimer Elizabeth, Anderson Glenn, Sebire Neil James
Department of Histopathology, UCL, London, UK.
Institute of Child Health, UCL, London, UK ; Department of Histopathology, UCL, London, UK ; Great Ormond Street Hospital for Children Foundation Trust, London WC1N 3JH, UK.
BMC Clin Pathol. 2014 Mar 31;14:13. doi: 10.1186/1472-6890-14-13. eCollection 2014.
Neuroblastoma is the most common solid tumour of infancy and is responsible for 15% of childhood cancer deaths. Presence of amplified MYCN in neuroblastoma is associated with poor prognosis and rapid tumour progression. The aim of this study was to examine and compare the ultrastructural features of high-risk MYCN amplified neuroblastomas, with lower-risk non-MYCN amplified tumours.
This was a retrospective study evaluating archival diagnostic tissue samples, in which Fluorescence in-situ hybridisation (FISH) had been used at diagnosis to establish MYCN status. 22 (11 MYCN amplified tumours and 11 non-MYCN amplified) tumours of similar light microscopic appearance (poorly differentiated neuroblastoma) were then selected for ultrastructural examination.
There is a relationship between ultrastructural features in neuroblastoma and MYCN status, although with marked overlap between groups. MYCN amplified tumours generally exhibited a 'less differentiated' ultrastructural phenotype, with significantly smaller neurotubules (NT) in the cell body (p < 0.002). Non-MYCN amplified tumours show increased features of neuronal differentiation, with fewer neurosecretory granules (NSG) and NT in the cytoplasm.
MYCN amplification is associated with a less differentiated ultrastructural phenotype, and lack of MYCN amplification with relative ultrastructural neuronal differentiation.
神经母细胞瘤是婴儿期最常见的实体瘤,占儿童癌症死亡病例的15%。神经母细胞瘤中MYCN基因扩增与预后不良及肿瘤快速进展相关。本研究旨在检查并比较高危MYCN基因扩增的神经母细胞瘤与低危非MYCN基因扩增肿瘤的超微结构特征。
这是一项回顾性研究,评估存档的诊断组织样本,这些样本在诊断时已使用荧光原位杂交(FISH)来确定MYCN状态。然后选择22例光镜表现相似(低分化神经母细胞瘤)的肿瘤(11例MYCN基因扩增肿瘤和11例非MYCN基因扩增肿瘤)进行超微结构检查。
神经母细胞瘤的超微结构特征与MYCN状态之间存在关联,尽管两组之间有明显重叠。MYCN基因扩增的肿瘤通常表现出“分化程度较低”的超微结构表型,细胞体内的神经微管(NT)明显更小(p < 0.002)。非MYCN基因扩增的肿瘤显示出神经分化特征增加,细胞质中的神经分泌颗粒(NSG)和NT较少。
MYCN基因扩增与分化程度较低的超微结构表型相关,而缺乏MYCN基因扩增则与相对的超微结构神经分化相关。