Choi Gwi Hyun, Oh Jae Keun, Kim Tae Yup, You Nam Kyu, Lee Hyo Sang, Yoon Do Heum, Ha Yoon, Yi Seong, Kim Dong Seok, Choi Joong Uhn, Kim Keung Nyun
Department of Spinal Neurosurgery, Guro TnTn Hospital, 547 Siheung-main road, Guro-gu, Seoul, South Korea.
Childs Nerv Syst. 2012 Jun;28(6):897-904. doi: 10.1007/s00381-012-1718-8. Epub 2012 Mar 25.
Primary spinal cord tumors (PSCTs) in pediatric patients are rare, with a reported overall incidence rate of 1-2.6 per one million children. We reviewed our experience of surgically treated 27 pediatric patients with PSCT and discussed the clinical features, radiological findings, surgical outcomes, and prognostic factors.
Between March 1999 and March 2010, a total of 27 pediatric patients with PSCT were surgically treated in a single institution. We retrospectively analyzed their data.
There were 13 females and 14 males, and their ages ranged from 6 months to 19 years (mean age, 12.1 years). The most common presenting symptom was motor weakness, and the histologic type of the tumors were mainly schwannoma, astrocytoma, and ependymoma. The tumor was completely resected in 17 patients, subtotally resected in 7 patients, and partial resection or biopsy was performed in 3 patients. Adjuvant chemotherapy was performed in 9 patients, and radiotherapy in 12 patients, respectively. The average follow-up period was 33.5 months (1.17-129). Five patients experienced the progression of disease, and three of them expired. The mean time for disease progression was 19.0 months (4.5-48.7).
PSCT in pediatric patients can be surgically removed with an acceptable low surgical morbidity. Progression-free survival was found to be related to the grade of tumor and the extent of tumor resection. Early diagnosis and treatment anticipate good functional neurologic outcome.
小儿原发性脊髓肿瘤(PSCT)较为罕见,据报道其总体发病率为每百万儿童1 - 2.6例。我们回顾了27例接受手术治疗的小儿PSCT患者的经验,并讨论了其临床特征、影像学表现、手术结果及预后因素。
1999年3月至2010年3月期间,在单一机构对27例小儿PSCT患者进行了手术治疗。我们对他们的数据进行了回顾性分析。
患者中女性13例,男性14例,年龄范围为6个月至19岁(平均年龄12.1岁)。最常见的首发症状是运动无力,肿瘤的组织学类型主要为神经鞘瘤、星形细胞瘤和室管膜瘤。17例患者肿瘤完全切除,7例次全切除,3例行部分切除或活检。分别有9例患者接受辅助化疗,12例患者接受放疗。平均随访期为33.5个月(1.17 - 129个月)。5例患者病情进展,其中3例死亡。疾病进展的平均时间为19.0个月(4.5 - 48.7个月)。
小儿PSCT可以通过手术切除,手术并发症发生率较低。无进展生存期与肿瘤分级和肿瘤切除范围有关。早期诊断和治疗有望获得良好的神经功能预后。