117例纵隔肿瘤并椎管内侵犯患儿的临床特征及疗效分析

Clinical characteristics and therapeutic outcomes of mediastinal tumor with intraspinal extension:a report of 117 cases

目的评估纵隔肿瘤可侵犯椎管导致脊髓压迫综合征患儿的临床特征及治疗方案。方法2011年1月至2020年5月期间,广州妇女儿童医疗中心共收治纵隔肿瘤并椎管内侵犯患儿117例,回顾性分析其人口特征、神经症状恢复、生存率等临床资料。结果117例患儿中神经母细胞瘤46例,神经节神经母细胞瘤21例,神经节细胞瘤10例,原始神经外胚层肿瘤(PNET)5例,淋巴瘤8例,生殖细胞肿瘤5例,横纹肌样瘤5例,横纹肌肉瘤3例,其他14例。63例患儿就诊时出现神经症状,化疗组23例治疗后症状改善13例(13/23,56.5%),神经外科手术组40例,治疗后改善30例(30/40,75.0%)。中重度神经功能障碍患儿中,脊髓压迫症状持续时间≤4周患儿治疗后其神经功能障碍完全康复率明显高于持续时间>4周的患儿(66.7%比8.7%,P<0.001)。结论后纵隔肿瘤并椎管内侵犯患儿缺乏特异性临床症状,部分患儿伴随脊髓压迫综合征,其神经症状功能障碍的恢复与首次治疗方案及神经症状持续时间相关。

Objective Mediastinal tumor invades spinal canal and results in spinal cord compression.Some patients develop severe spinal deformities after a decompression of spinal cord.The optimal therapeutic strategy for mediastinal tumor with intraspinal extension remains elusive.Our study was to assess the clinical characteristics and therapeutic strategies for mediastinal tumor with intraspinal extension.Methods From January 2011 to May 2020,117 children of mediastinal tumors with intraspinal extension were recruited.Clinical features,personal demographics,neurologic recovery and survival rate were assessed.Results Among 117 children with mediastinal tumors with intraspinal extension,there were neuroblastoma(n=46),ganglioneuroblastoma(n=21),gangliobastoma(n=10),primitive neuroectodermal tumor(PNET,n=5),lymphoma(n=8),teratoma(n=5),rhabdomyoid tumor(n=5),rhabdomyosarcoma(n=3)and miscellaneous(n=14).Initial neurological symptoms were present in 63 children.In chemotherapy group,neurological deficits improved(13/23,56.5%);in those undergoing neurosurgical intervention,neurological deficits improved(30/40,75%).In children with moderate-to-severe neurological deficits,compression manifestations of≤4 weeks duration showed an improved outcome compared to a longer compression time,with complete recovery of neurological function in 66.7%of children versus 8.7%for those with a longer symptom duration(P<0.001).Conclusions Symptoms of posterior mediastinal tumors are nonspecific and spinal cord compression is evident in some patients.Neurological recovery is correlated with the type of initial treatment and duration of neurological symptoms.