Pediatric high grade glioma of the spinal cord: results of the HIT-GBM database

Abstract

Little is known about pediatric spinal cord high grade gliomas (SCHGG) beyond their dismal prognosis. Here, we analyzed the HIT-GBM(®) database for the influence of surgical resection on survival. Between 1991 and 2010 the HIT-GBM group collected data from European children diagnosed with high grade glioma. Patients with the following inclusion criteria were analyzed in this study: astrocytic histology, WHO grade III or IV, age at diagnosis <18 years, and tumor localized to the spinal cord. 28 patients (mean age 11.28 years, 14 male) with primary SCHGG were identified. The tumor sizes were measured by the span across adjacent vertebrae and varied greatly (range: 1-20, median: 4). Histology was classified as WHO grade III in 15 and grade IV in 13 tumors. Of note, the four largest tumors identified were WHO grade III. Surgery was classified as complete resection (n = 6), subtotal resection (STR) (n = 7), partial resection (n = 12) or biopsy only (n = 3). 27 patients received chemotherapy, 22 of which also received radiation. With the mean follow-up time of 2.88 (SD ± 2.95) years, 14 patients were still alive resulting in a median overall survival of 2.5 years (SE ± 1.6). The positive prognostic indicators for overall survival were: age younger than 5 years (P = 0.047), WHO grade III (P = 0.046), absence of necrosis (P = 0.025) and gross total resection (GTR) (P = 0.012). The prognosis of SCHGG might not be as miserable as generally assumed. GTR is of benefit. Larger data sets and meta-analysis are necessary to identify patient sub-groups.

Analysis of Risk Factors and Survival in Pediatric High-Grade Spinal Cord Astrocytoma: A Population-Based Study

“…Median survival for GBM was 7 months and for AA it was 12 months. This finding reflects what has been published in the literature about cranial and spinal high-grade gliomas: correlation between higher grade of tumor and mortality is also reported by other groups [6,9,10]. Merchant et al [11] report otherwise in one retrospective study of 11 pediatric HGSCA patients where no difference in survival was found between patients with grade III and IV tumors; however, multiple other reports corroborate a worse prognosis with grade IV tumors.…”

“…Although pediatric HGSCAs are very rare and few published studies specifically focus on this group, there is supportive evidence that younger age may be associated with better outcomes: Wolff et al [6 ]found that age <5 years was a positive prognostic indicator for overall survival in pediatric high-grade spinal cord glioma [6]. Santi et al [7] examined 36 patients with malignant spinal cord astrocytomas with a mean age of 32.4 years, and also report that patients >40 years of age had a shorter survival period compared to younger patients.…”

“…Merchant et al [11] report otherwise in one retrospective study of 11 pediatric HGSCA patients where no difference in survival was found between patients with grade III and IV tumors; however, multiple other reports corroborate a worse prognosis with grade IV tumors. For instance, Wolff et al [6] noted lower tumor grade to be a statistically significant prognostic indicator of longer overall survival. Tendulkar et al [9 ]found that GBM had higher rates of local progression than AA.…”

“…Minehan et al [13] studied a spectrum of spinal cord astrocytomas and report postoperative radiation therapy (with a majority of surgical interventions being biopsies and a minority being subtotal or gross total resections) significantly improved survival for patients with infiltrative astrocytomas. Other published studies have not shown significant improvements in survival among those treated with radiation [6,7,14]. A SEER population-based study examining a range of low-grade and high-grade pediatric spinal cord tumors including gliomas and ependymomas described decreased use of radiation over time from 1975 to 2007, though the impact of radiation was not addressed in their methodology [1].…”

Pediatric Glioblastoma

“…p-GBMs are most commonly reported in the second decade of life although their occurrence have been reported even in utero (2,4,5,9,20,21). The highest incidence of p-GBM is seen between ages 15 and 19.…”