Hartmann O, Favrot M C
Département de pédiatrie, Institut Gustave-Roussy, Villejuif.
Rev Prat. 1993 Nov 1;43(17):2182-6.
Neuroblastoma is a malignant tumour of the sympathetic tissue observed in children, generally before 6 years and before 1 year in one-third of the cases. In most cases the primary tumour lies in the retroperitoneal region, and in particular in the adrenal gland. Metastases affect the bones and bone-marrow after the age of 1 year and the liver in infants. Children whose metastases are present at the time of diagnosis are the most numerous. The therapeutic problems posed by non-metastatic forms are almost exclusively due to the difficulties encountered in complete excision of the tumour. For this reason, tumour reduction by preoperative chemotherapy plays a large role in the therapeutic approach. The prognosis of metastatic forms is totally different in infants and in older children: in infants, hepatic metastases without bone lesions have a favourable prognosis as a rule and sometimes regress without treatment. In older children, the usual osteomedullary damage still has a very sombre prognosis despite the recent therapeutic advances due to high-dose chemotherapy with bone-marrow transplantation. Cytogenetic studies and molecular biology have improved our understanding of these differences in the outcome of the disease. Amplification of Nmy cellular oncogene, ploidy and chromosome 1 short-arm deletion are essential prognostic factors in this particular tumour.
神经母细胞瘤是一种发生于儿童交感组织的恶性肿瘤，通常在6岁前发病，三分之一的病例在1岁前发病。大多数情况下，原发肿瘤位于腹膜后区域，尤其是肾上腺。1岁后转移至骨骼和骨髓，婴儿期转移至肝脏。诊断时已有转移的儿童最为常见。非转移性神经母细胞瘤的治疗问题几乎完全是由于肿瘤完整切除困难所致。因此，术前化疗缩小肿瘤在治疗方案中起着重要作用。转移性神经母细胞瘤在婴儿和大龄儿童中的预后完全不同：在婴儿中，无骨病变的肝转移通常预后良好，有时不经治疗即可消退。在大龄儿童中，尽管最近采用大剂量化疗联合骨髓移植取得了治疗进展，但常见的骨髓损害预后仍然很差。细胞遗传学研究和分子生物学提高了我们对该疾病不同预后的认识。Nmy细胞癌基因扩增、倍性和1号染色体短臂缺失是这种特殊肿瘤的重要预后因素。
