Changes to this Summary (05/17/2012)

The PDQ cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.

General Information

Added Margraf et al. as reference 7.

Revised text about the genomic alterations involving BRAF, including the BRAF-KIAA1549 fusion in pilocytic astrocytomas and BRAF point mutations (V600E) in nonpilocytic pediatric low-grade gliomas (cited Lin et al. as reference 20, Hawkins et al. as reference 21, Janzarik et al. as reference 22, Dougherty et al. as reference 23, Dias-Santagata et al. as reference 24, and Schindler et al. as reference 25).

Added text about the incidence of IDH1 and histone H3.3 (H3F3A) mutations in pediatric high-grade astrocytomas. Pediatric high-grade astrocytomas with H3F3A mutations often additionally have somatic mutations in ATRX, a gene coding for a protein involved in chromatin remodeling, which suggests that a substantial proportion of these astrocytomas are associated with processes required for establishing normal chromatin architecture (cited Schwartzentruber et al. and Wu et al. as references 30 and 31, respectively).

Added text to state that the molecular profile of pediatric patients with oligodendroglioma does not demonstrate deletions of 1p or 19q as found in 40% to 80% of adult cases. Pediatric oligodendroglioma harbors MGMT gene promoter methylation in the majority of tumors (cited Suri et al. as reference 32).

Added Wisoff et al. as reference 40.

Added text to state that elevated MIB-1 labelling index is associated with shortened progression-free survival (PFS) in patients with pilocytic astrocytoma; a BRAF-KIAA fusion, found in pilocytic tumors, confers a better clinical outcome.

Treatment of Childhood Low-Grade Astrocytomas

Added Kramm et al. as reference 4 and Wisoff et al. as reference 14.

Added text about how factors related to outcome for children with low-grade gliomas treated with surgery followed by observation were identified in a Children’s Oncology Group study that included 518 evaluable patients. Overall outcome for the entire group was 78% PFS at 8 years and 96% overall survival (OS) at 8 years. The prognostic factors included histology, extent of resection, age, and tumor location.

Added text to state that approximately 50% of patients with less-than-gross total resection may have disease that remains progression free at 5 to 8 years, supporting the observation strategy in selected patients.

Added text to state that most patients ultimately require further treatment; this is particularly true for children who initially present with hypothalamic/chiasmatic gliomas that have neuraxis dissemination (cited von Hornstein et al. as reference 46 and level of evidence 3iiiDiii).

Treatment of Recurrent Childhood Low-Grade Astrocytomas

Added Scheinemann et al. as reference 5 and level of evidence 3iA.

Treatment of Childhood High-Grade Astrocytomas

Revised text to state that similarly poor survival is seen in children with spinal cord primaries and children with thalamic high-grade gliomas (cited Kramm et al. as reference 3).