Shochat S J, Corbelletta N L, Repman M A, Schengrund C L
J Pediatr Surg. 1987 Jul;22(7):660-4. doi: 10.1016/s0022-3468(87)80122-7.
A biochemical analysis was performed on tumor tissues from 20 patients who presented with thoracic neuroblastomas. Nine patients were under 1 year of age at the time of diagnosis, and 12 patients had stage A disease. Eighteen of the 20 patients are disease free with a mean follow-up of 5 1/2 years. The ganglioside composition of the tumor tissue was investigated, since these cell membrane components have been proposed to play a role in cell to cell interaction and may be altered on cell transformation. In addition, the ganglioside composition of the central nervous system changes with maturation. Previous studies in children with neuroblastoma have shown that tumor tissue containing more complex gangliosides is associated with a better prognosis. Neuroblastomas from patients with thoracic primaries were found to contain more complex gangliosides of the b series (GD1b, GT1b) and fewer monosialogangliosides, suggesting a more differentiated cellular composition. Tissue from one of the thoracic patients who died lacked GT1b. The absence of this ganglioside has proven to be an indicator of a poor prognosis. Four specimens contained no detectable GD2, which is thought to be a specific marker for neuroblastomas. These data suggest that the improved prognosis seen with thoracic neuroblastomas is due to a basic biologic difference within these tumors, and this finding should be taken into consideration when planning therapy.
对20例患有胸段神经母细胞瘤患者的肿瘤组织进行了生化分析。9例患者在诊断时年龄小于1岁,12例患者患有A期疾病。20例患者中有18例无疾病,平均随访时间为5年半。对肿瘤组织的神经节苷脂组成进行了研究,因为这些细胞膜成分被认为在细胞间相互作用中起作用,并且在细胞转化时可能会发生改变。此外,中枢神经系统的神经节苷脂组成会随着成熟而变化。先前对神经母细胞瘤患儿的研究表明,含有更复杂神经节苷脂的肿瘤组织与更好的预后相关。发现来自胸段原发患者的神经母细胞瘤含有更多的b系列复杂神经节苷脂(GD1b、GT1b)和更少的单唾液酸神经节苷脂,这表明细胞组成更具分化性。一名死亡的胸段患者的组织中缺乏GT1b。已证明这种神经节苷脂的缺失是预后不良的一个指标。4个标本中未检测到GD2,GD2被认为是神经母细胞瘤的一种特异性标志物。这些数据表明,胸段神经母细胞瘤预后的改善是由于这些肿瘤内部存在基本的生物学差异,在制定治疗方案时应考虑到这一发现。