Survival and functional outcome of childhood spinal cord low-grade gliomas

作者：Katrin Scheinemann, Ute Katharina Bartels, Annie Huang, Cynthia E Hawkins, Abhaya Vivek Kulkarni, Éric Bouffet, Uri Tabori · 发表于：Journal of Neurosurgery Pediatrics · 年份：2009 · DOI：10.3171/2009.4.peds08411 · 被引用次数：49 · 研究领域：Glioma Diagnosis and Treatment、Neurofibromatosis and Schwannoma Cases、Meningioma and schwannoma management

OBJECT: Intramedullary spinal cord low-grade gliomas (LGGs) are rare CNS neoplasms in pediatric patients, and there is little information on therapy for and outcome of these tumors in this population. Furthermore, most patient series combine adult and pediatric patients or high- and low-grade tumors, resulting in controversial data regarding optimal treatment of these children. To clarify these issues, the authors performed a regional population-based study of spinal cord LGGs in pediatric patients. METHODS: All pediatric patients with LGGs treated during the MR imaging era (1985-2007) were identified in the comprehensive database of the Hospital for Sick Children in Toronto. Data on demographics, pathology, treatment details, and outcomes were collected. RESULTS: Spinal cord LGGs in pediatric patients constituted 29 (4.6%) of 635 LGGs. Epidemiological and clinical data in this cohort were different than in patients with other spinal tumors and strikingly similar to data from pediatric patients with intracranial LGGs. The authors observed an age peak at 2 years and a male predominance in patients with these tumors. Histological testing revealed a Grade I astrocytoma in 86% of tumors. Although 5-year progression-free survival for the entire group was 48 +/- 9%, all patients were alive at a median follow-up of 8.2 years. Five-year progression-free survival was 88 +/- 13% for patients undergoing gross-total resection and 34 +/- 11% for those undergoing all other therapies, respe...