Houten J K, Weiner H L
Department of Neurosurgery, New York University Medical Center, New York 10016, USA.
J Neurooncol. 2000 May;47(3):225-30. doi: 10.1023/a:1006418506213.
Intramedullary spinal cord tumors (IMSCTs) of the pediatric population are rare and comprise thirty-five percent of intraspinal neoplasms. Low-grade astrocytomas predominate; ependymomas increase in frequency with ascending age and become the most frequent IMSCT in adults. Gangliogliomas are very rare in adults but comprise nearly thirty percent of tumors in children under three years of age. The cervical spine is the region of the spine most affected. Pain is the most common presenting symptom with weakness, gait deterioration, torticollis also frequently reported. Hydrocephalus occurs with greater frequency than in adult patients and often requires a shunt. Motor and sensory evoked potential monitoring is routinely utilized. Osteoplastic laminotomy is performed to forestall the development of progressive spinal deformity. Gross total resection is feasible in most ependymomas and results in surgical cure. Astrocytomas are infiltrating neoplasms and gross total resection is occasionally possible only in the pediatric population. However, the role of radical resection of low-grade fibrillary astrocytomas of the spinal cord in children has not been definitively demonstrated in the literature. Outcome for low-grade astrocytomas is better in children than adults, but not as favorable as that for ependymomas. Malignant tumors have dismal outcomes and surgery in these patients serves only to provide a diagnosis.
小儿脊髓髓内肿瘤(IMSCTs)较为罕见,占椎管内肿瘤的35%。低级别星形细胞瘤最为常见;室管膜瘤的发病率随年龄增长而增加,在成人中成为最常见的IMSCT。神经节胶质瘤在成人中非常罕见,但在3岁以下儿童的肿瘤中占近30%。颈椎是脊柱受影响最严重的部位。疼痛是最常见的症状,虚弱、步态恶化、斜颈也经常出现。脑积水的发生率高于成人患者,通常需要分流。常规使用运动和感觉诱发电位监测。进行骨成形性椎板切开术以防止进行性脊柱畸形的发展。大多数室管膜瘤可行全切除并实现手术治愈。星形细胞瘤是浸润性肿瘤,仅在小儿患者中偶尔可能实现全切除。然而,脊髓低级别纤维性星形细胞瘤在儿童中的根治性切除作用在文献中尚未得到明确证实。低级别星形细胞瘤患儿的预后比成人好,但不如室管膜瘤。恶性肿瘤的预后很差,这些患者的手术仅用于提供诊断。