Merchant T E, Kiehna E N, Thompson S J, Heideman R, Sanford R A, Kun L E
Department of Radiation Oncology, St. Jude Children's Research Hospital, Memphis, TN 38105, USA.
Pediatr Neurosurg. 2000 Jan;32(1):30-6. doi: 10.1159/000028894.
Our institutional experience with pediatric spinal cord tumors includes 25 patients with the diagnosis of ependymoma (EP; n = 4), myxopapillary ependymoma (MPEP; n = 4), juvenile pilocytic astrocytoma (JPA; n = 5), nonpilocytic astrocytoma (WHO grade I or II, n = 6), and other nonastrocytic spinal cord tumors (n = 6) treated during the period 1974-1999. Nineteen patients required radiation therapy (RT). The median progression-free survival following RT was 65 months (range 1-206 months). Seven patients recurred at an average of 22 months. The EP patients recurred at an average of 8.5 months, while the patients with low-grade astrocytoma recurred at an average of 42 months. Including the 6 nonsurviving patients, the median overall survival was 96 months. Two EP patients died with a progression-free survival of 9 months. One patient with MPEP died of other causes at 7 months. The treatment of pediatric spinal cord tumors should be individualized based on the histologic type. Radical surgery is indicated for nonmyxopapillary EP and low-grade astrocytic tumors. The need for adjuvant therapy most often depends on the extent of resection as well as the tumor type. Patients with disseminated EP, MPEP, JPA and nonpilocytic astrocytoma may achieve long-term progression-free survival with craniospinal irradiation.
我们机构在小儿脊髓肿瘤方面的经验包括1974年至1999年期间治疗的25例诊断为室管膜瘤（EP；n = 4）、黏液乳头型室管膜瘤（MPEP；n = 4）、青少年毛细胞型星形细胞瘤（JPA；n = 5）、非毛细胞型星形细胞瘤（世界卫生组织I级或II级，n = 6）以及其他非星形细胞脊髓肿瘤（n = 6）的患者。19例患者需要放疗（RT）。放疗后的无进展生存期中位数为65个月（范围1 - 206个月）。7例患者复发，平均复发时间为22个月。EP患者平均8.5个月复发，而低级别星形细胞瘤患者平均42个月复发。包括6例未存活患者，总生存期中位数为96个月。2例EP患者在无进展生存期9个月时死亡。1例MPEP患者在7个月时死于其他原因。小儿脊髓肿瘤的治疗应根据组织学类型个体化。对于非黏液乳头型EP和低级别星形细胞瘤，建议行根治性手术。辅助治疗的必要性通常取决于切除范围以及肿瘤类型。患有播散性EP、MPEP、JPA和非毛细胞型星形细胞瘤的患者通过全脑全脊髓照射可能实现长期无进展生存。
