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Multicenter Study
. 2010 Dec;12(12):1257-68.
doi: 10.1093/neuonc/noq092. Epub 2010 Sep 22.

A multivariate analysis of factors determining tumor progression in childhood low-grade glioma: a population-based cohort study (CCLG CNS9702)

Tore Stokland  1 , Jo-Fen Liu, James W Ironside, David W Ellison, Roger Taylor, Kathryn J Robinson, Susan V Picton, David A Walker
Affiliations
Multicenter Study

A multivariate analysis of factors determining tumor progression in childhood low-grade glioma: a population-based cohort study (CCLG CNS9702)

Tore Stokland et al. Neuro Oncol. 2010 Dec.

Abstract

The purpose of this study was to identify risk factors for the progression of low-grade glioma in children from a large population-based cohort. Patient and tumor details of a national cohort of children with low-grade glioma, recruited into an international multidisciplinary clinical strategy, were subjected to univariate and multivariate analyses of progression-free survival and overall survival. From the cohort of 798 patients, 639 patients were eligible, with a median age 6.71 years (0.26-16.75 years); 49% were males; 15.9% had neurofibromatosis type 1, 63.7% pilocytic astrocytoma, 5.9% fibrillary astrocytoma, 4.2% mixed neuronal-glial tumors, and 3.6% others; 21.1% were diagnosed clinically. Anatomically implicated were 31.6% cerebellum, 24.6% chiasma/hypothalamus, 16.0% cerebral hemispheres, 9.9% brain stem, 6.1% other supratentorial midline structures, 5.9% optic nerve only, 4.5% spinal cord, and 1.4% others. The 5-year overall survival and progression-free survival in the whole cohort were 94.6% and 69.4%, respectively. There was a significant association between age and site (P < .001) and extent of tumor resection and site (P < .001). Multivariate analysis identified young age, fibrillary astrocytoma, and extent of surgical resection as significant independent risk factors for progression. Hypothalamic/chiasmatic tumors demonstrated the most sustained tendency to progress. In conclusion, the influence of age and anatomical site upon the risk of tumor progression suggests that these factors strongly influence tumor behavior for the majority of pilocytic tumors. Age <1 year and 1-5 years, fibrillary histology, completeness of resection, and chiasmatic location are candidates for stratification in future studies.

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Figures

Fig. 1.
Fig. 1.
Study population and treatment strategy.
Fig. 2.
Fig. 2.
Age and site distribution.  (A)  Age distribution, all; (B) age distribution, chiasma/hypothalamus; (C) age distribution, cerebral hemisphere; (D) age distribution, cerebellum; (E) median age by site; (F) extent of tumor resection by site.
Fig. 3.
Fig. 3.
The Kaplan–Meier curves of PFS. (A) PFS, all; (B) by pathology; (C) by age; (D) by age at nonoptic pathway glioma (non-OPG) sites; (E) by NF1 status; (F) by NF1 status at chiasma/hypothalamus; (G) by site; (H) by extent of tumor resection.
Fig. 4.
Fig. 4.
The Kaplan–Meier curves of OS. (A) OS, all; (B) OS by age group.
See this image and copyright information in PMC

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