Cohn S L, Salwen H, Herst C V, Maurer H S, Nieder M L, Morgan E R, Rosen S T
Division of Hematology/Oncology, Children's Memorial Hospital, Chicago, IL 60614.
Cancer. 1988 Aug 15;62(4):723-6. doi: 10.1002/1097-0142(19880815)62:4<723::aid-cncr2820620413>3.0.co;2-u.
Patients with neuroblastoma who present with the syndrome of opsoclonus and myoclonus enjoy a remarkably good prognosis independent of their stage of disease or their age at diagnosis. The presence of N-myc amplification also has been found to be an independent prognostic factor in neuroblastoma. Patients with multicopy N-myc tumors have rapid tumor progression whereas those with single-copy tumors have a significantly better progression-free survival. The authors examined four primary, untreated neuroblastomas for the N-myc copy number from patients who presented with opsoclonus and myoclonus. All four tumors had single copies of N-myc, and all four patients are alive with no evidence of recurrent disease with 6+ to 54+ months' follow-up. This appears to be the only report of N-myc analysis in this group of children. It would be interesting to analyze more neuroblastomas from patients who present with opsoclonus and myoclonus to determine how many of these patients have single N-myc copy tumors.
患有成神经细胞瘤且出现眼阵挛和肌阵挛综合征的患者，无论其疾病分期或诊断时的年龄如何，预后都非常好。N - myc基因扩增的存在也被发现是成神经细胞瘤的一个独立预后因素。多拷贝N - myc肿瘤的患者肿瘤进展迅速，而单拷贝肿瘤的患者无进展生存期明显更长。作者检测了4例未经治疗的原发性成神经细胞瘤患者的N - myc拷贝数，这些患者均表现为眼阵挛和肌阵挛。所有4个肿瘤均为N - myc单拷贝，所有4例患者均存活，随访6 +至54 +个月，无疾病复发迹象。这似乎是关于这组儿童N - myc分析的唯一报告。分析更多表现为眼阵挛和肌阵挛的成神经细胞瘤患者，以确定这些患者中有多少患有N - myc单拷贝肿瘤，将会很有意思。
