Department of Biochemistry and Molecular Biology, College of Medicine, Pennsylvania State University, Hershey, PA 17033, USA.
Int J Mol Sci. 2020 Jul 27;21(15):5313. doi: 10.3390/ijms21155313.
The focus of this review is the ganglio-series of glycosphingolipids found in neuroblastoma (NB) and the myriad of unanswered questions associated with their possible role(s) in this cancer. NB is one of the more common solid malignancies of children. Five-year survival for those diagnosed with low risk NB is 90-95%, while that for children with high-risk NB is around 40-50%. Much of the survival rate reflects age of diagnosis with children under a year having a much better prognosis than those over two. Identification of expression of GD2 on the surface of most NB cells led to studies of the effectiveness and subsequent approval of anti-GD2 antibodies as a treatment modality. Despite much success, a subset of patients, possibly those whose tumors fail to express concentrations of gangliosides such as GD1b and GT1b found in tumors from patients with a good prognosis, have tumors refractory to treatment. These observations support discussion of what is known about control of ganglioside synthesis, and their actual functions in NB, as well as their possible relationship to treatment response.
本综述的重点是神经母细胞瘤(NB)中发现的神经节苷脂系列糖脂,以及它们在这种癌症中可能发挥的多种作用相关的诸多未解问题。NB 是儿童中较为常见的实体恶性肿瘤之一。低危 NB 患儿的五年生存率为 90-95%,而高危 NB 患儿的生存率约为 40-50%。高生存率在很大程度上反映了诊断时的年龄,1 岁以下患儿的预后明显好于 2 岁以上患儿。大多数 NB 细胞表面表达 GD2,这促使人们研究抗 GD2 抗体的有效性,并随后批准其作为一种治疗方法。尽管取得了很大的成功,但仍有一部分患者,可能是那些肿瘤未能表达 GD1b 和 GT1b 等神经节苷脂浓度的患者,他们的肿瘤对治疗有抗性。这些观察结果支持对神经节苷脂合成的控制及其在 NB 中的实际功能进行讨论,以及它们与治疗反应的可能关系。