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Pediatric central nervous system germ cell tumors: a review.
TLDR
Central nervous system (CNS) germ cell tumors (GCTs) represent approximately 3% of primary pediatric brain tumors and encompass a wide pathologic spectrum and treatment and prognosis differ greatly between groups. Expand
Genomic analysis of diffuse intrinsic pontine gliomas identifies three molecular subgroups and recurrent activating ACVR1 mutations
TLDR
This work integrated whole-genome sequencing with methylation, expression and copy number profiling, discovering that DIPGs comprise three molecularly distinct subgroups (H3-K27M, silent and MYCN) and uncovering a new recurrent activating mutation affecting the activin receptor gene ACVR1 in 20% of DIPG. Expand
Markers of survival and metastatic potential in childhood CNS primitive neuro-ectodermal brain tumours: an integrative genomic analysis.
TLDR
Three molecular subgroups of CNS PNETs that were distinguished by primitive neural, oligoneural, and mesenchymal lineage gene-expression signatures with differential expression of cell-lineage markers LIN28 and OLIG2 are identified. Expand
Pediatric High Grade Glioma: a Review and Update on Tumor Clinical Characteristics and Biology
TLDR
A review of pediatric non-brainstem HGG, including epidemiology, presentation, histology, imaging characteristics, treatments, survival outcomes, and an overview of both basic and translational research is provided. Expand
REST Is a Novel Prognostic Factor and Therapeutic Target for Medulloblastoma
TLDR
Two studies suggest that HDACIs may have therapeutic potential for patients with REST-positive tumors and a panel of HDAC inhibitors (HDACI) for their effects on growth and differentiation of established and primary REST- positive cell lines. Expand
Selumetinib in paediatric patients with BRAF-aberrant or neurofibromatosis type 1-associated recurrent, refractory, or progressive low-grade glioma: a multicentre, phase 2 trial.
TLDR
Results show that selumetinib could be an alternative to standard chemotherapy for these subgroups of patients, and have directly led to the development of two Children's Oncology Group phase 3 studies comparing standard chemotherapy to selumsentinib in patients with newly diagnosed paediatric low-grade glioma both with and without NF1. Expand
CNS-PNETs with C19MC amplification and/or LIN28 expression comprise a distinct histogenetic diagnostic and therapeutic entity
TLDR
Current known histologic categories of CNS-PNETs, which include ETANTRs, medulloepitheliomas, ependymoblastomas in various CNS locations, comprise a common molecular and diagnostic entity and identify inhibitors of the LIN28/let7/PI3K/mTOR axis and DNMT3B as promising therapeutics for this distinct histogenetic entity. Expand
Intensive chemotherapy followed by consolidative myeloablative chemotherapy with autologous hematopoietic cell rescue (AuHCR) in young children with newly diagnosed supratentorial primitive
TLDR
Head Start protocols treated children with newly diagnosed sPNET utilizing intensified induction chemotherapy (ICHT) followed by consolidation with myeloablative chemotherapy and autologous hematopoietic cell rescue (AuHCR) to improve overall survival and decrease radiation exposure. Expand
Aggressive variant of a papillary glioneuronal tumor. Report of 2 cases.
TLDR
The authors conclude that papillary glioneuronal tumors do not always behave in a strictly benign fashion. Expand
Survivin splice variants regulate the balance between proliferation and cell death
TLDR
It is shown that the diverse functional roles of survivin can be explained, in part, by its heterodimerization with survivin splice variants in tumor cells, which is likely to have a significant impact in the design of biologic agents for clinical therapy. Expand
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