Clinicopathologic study of glioblastoma in children with neurofibromatosis type 1.

摘要: Background Neurofibromatosis type 1 (NF1) is characterized by low-grade tumors of the central and peripheral nervous system. There also an increased risk developing malignant tumors. Glioblastoma uncommon, tumor children that even less frequently observed in with NF1. Procedure We performed a retrospective review patients NF1 glioblastoma to determine specific clinical pathologic indicators overall prognosis. Results Five were identified from CHB/DFCI database for whom imaging studies available. All specimens demonstrated vascular proliferation necrosis. samples stained positively p53. Chromogenic situ hybridization (CISH) epidermal growth factor receptor (EGFR) copy numbers was increased, PTEN normal promoter O(6)-methylguanine-DNA methyltransferase (MGMT) gene unmethylated one patient evaluated. In same time period, there 56 without diagnosed who treated at our institution. Although small sample size precludes formal statistical analysis, 2-year survival 60% median 9.25 years compared non-NF1 25% 1.08 years. Conclusions This study provides preliminary evidence may be glioblastoma, but these have NF1. Additional molecular will required if pathogenesis differs Pediatr Blood Cancer 2010;54:890–896 © 2010 Wiley-Liss, Inc.