Johnsen John Inge, Dyberg Cecilia, Wickström Malin
Childhood Cancer Research Unit, Department of Women's and Children's Health, Karolinska Institutet (KI), Stockholm, Sweden.
Front Mol Neurosci. 2019 Jan 29;12:9. doi: 10.3389/fnmol.2019.00009. eCollection 2019.
Neuroblastoma is a neural crest derived malignancy of the peripheral nervous system and is the most common and deadliest tumor of infancy. It is characterized by clinical heterogeneity with a disease spectrum ranging from spontaneous regression without any medical intervention to treatment resistant tumors with metastatic spread and poor patient survival. The events that lead to the development of neuroblastoma from the neural crest have not been fully elucidated. Here we discuss factors and processes within the neural crest that when dysregulated have the potential to be initiators or drivers of neuroblastoma development. A more precise biological understanding of neuroblastoma causes and cell of origin is highly warranted. This will give valuable information for the development of medicines that specifically target molecules within neuroblastoma cells and also give hint about the mechanisms behind treatment resistance that is frequently seen in neuroblastoma.
神经母细胞瘤是一种起源于神经嵴的外周神经系统恶性肿瘤,是婴儿期最常见且最致命的肿瘤。其临床特征具有异质性,疾病谱涵盖从无需任何医学干预即可自发消退到具有转移扩散且患者生存率低的治疗抵抗性肿瘤。导致神经母细胞瘤从神经嵴发展而来的事件尚未完全阐明。在此,我们讨论神经嵴内的因素和过程,这些因素和过程一旦失调,就有可能成为神经母细胞瘤发展的起始因素或驱动因素。对神经母细胞瘤的病因和起源细胞进行更精确的生物学理解非常必要。这将为开发专门针对神经母细胞瘤细胞内分子的药物提供有价值的信息,也能为神经母细胞瘤中常见的治疗抵抗背后的机制提供线索。