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

Abstract

<jats:sec> <jats:title>Object</jats:title> <jats:p>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.</jats:p></jats:sec> <jats:sec> <jats:title>Methods</jats:title> <jats:p>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.</jats:p></jats:sec> <jats:sec> <jats:title>Results</jats:title> <jats:p>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, respectively (p = 0.02). Chemotherapy and radiation therapy showed similar efficacy, achieving sustained tumor control in most patients. However, this excellent survival rate was associated with an 83% rate of significant neurological and orthopedic sequelae.</jats:p></jats:sec> <jats:sec> <jats:title>Conclusions</jats:title> <jats:p>This study provides basic data on the incidence, clinical course, and outcome of spinal cord LGGs in pediatric patients. The similarities between spinal and intracranial LGGs in pediatric patients showing excellent survival but high morbidity suggest that a less aggressive approach may be the preferable treatment option for these patients.</jats:p></jats:sec>

Pediatric intramedullary spinal cord tumors: A single center experience

“…Age distribution of our cases is similar to that reported by O'Sullivan et al 10 Scheinemann et al 11 reported the same age with 5.8 years in their study on spinal glioma patients, but most of their patients (41%) were under 4 years. The male/ female ratio in their study was 2.6.…”

“…Intraspinal tumors in children are low-grade and progress slowly, and therefore their diagnosis can be delayed. The interval between symptom onset and diagnosis was approximately 10 months in the case of low-grade tumors 11,13 and 2e7 months in the case of high-grade tumors. 14e16 The high rate of severe deficit was probably due to delays in admission to our center.…”

“…Other studies have reported similar symptoms as in our study, but with a better neurological status (McCormick grade II). 9,11,13,17 Radiotherapy has been successfully used to treat both ependymomas and astrocytomas 10,18e21 in some series. O'Sullivan et al 10 used radiotherapy in cases who had uncomplete resection.…”

Long-term disease and neurological outcomes in patients with pediatric intramedullary spinal cord tumors

“…[41][42][43] The overall GTR rate in our series (38%) was lower than that previously reported by some authors 4 but in line with the rate reported by others. 44 In contrast to earlier studies, 2,33 our results support the beneficial role for GTR, where feasible, in long-term disease survival and prevention of disease progression over extended follow-up. 30 The predictive value of extent of resection was maintained across our cumulative case series.…”

“…2,9,22,26,32,33,36,44,46 The overall rate of tumor progression in our series was 47%, with a mean onset of 3 years postoperatively (mean [± SEM] 3.3 ± 1 years, range 0.1-20 years). Radiotherapy was used following disease progression or recurrence in 51% of the 55 patients, while chemotherapy was used in 29% of the patients.…”