Acta neuropathologica communications, 2020-03, Vol.8 (1), p.30-30, Article 30
2020
Details
- Autor(en) / Beteiligte
- Titel
- Pediatric low-grade glioma in the era of molecular diagnostics
- Ist Teil von
- Acta neuropathologica communications, 2020-03, Vol.8 (1), p.30-30, Article 30
- Ort / Verlag
- England: BioMed Central Ltd
- Erscheinungsjahr
- 2020
- Link zum Volltext
- Quelle
- MEDLINE
- Beschreibungen/Notizen
- Low grade gliomas are the most frequent brain tumors in children and encompass a spectrum of histologic entities which are currently assigned World Health Organisation grades I and II. They differ substantially from their adult counterparts in both their underlying genetic alterations and in the infrequency with which they transform to higher grade tumors. Nonetheless, children with low grade glioma are a therapeutic challenge due to the heterogeneity in their clinical behavior - in particular, those with incomplete surgical resection often suffer repeat progressions with resultant morbidity and, in some cases, mortality. The identification of up-regulation of the RAS-mitogen-activated protein kinase (RAS/MAPK) pathway as a near universal feature of these tumors has led to the development of targeted therapeutics aimed at improving responses while mitigating patient morbidity. Here, we review how molecular information can help to further define the entities which fall under the umbrella of pediatric-type low-grade glioma. In doing so we discuss the specific molecular drivers of pediatric low grade glioma and how to effectively test for them, review the newest therapeutic agents and their utility in treating this disease, and propose a risk-based stratification system that considers both clinical and molecular parameters to aid clinicians in making treatment decisions.
- Sprache
- Englisch
- Identifikatoren
- ISSN: 2051-5960eISSN: 2051-5960DOI: 10.1186/s40478-020-00902-zTitel-ID: cdi_doaj_primary_oai_doaj_org_article_1440370edd8245568bce0a1aa72f70b4
- Format
- –
- Schlagworte
- Adults, Astrocytoma - diagnosis, Astrocytoma - drug therapy, Astrocytoma - genetics, Astrocytoma - pathology, Brain cancer, Brain Neoplasms - diagnosis, Brain Neoplasms - drug therapy, Brain Neoplasms - genetics, Brain Neoplasms - pathology, brain tumor, Brain tumors, Chemotherapy, Child, Classification, Clinical trials, Cytotoxicity, Development and progression, Diseases, Ganglioglioma - diagnosis, Ganglioglioma - drug therapy, Ganglioglioma - genetics, Ganglioglioma - pathology, glioma, Glioma - diagnosis, Glioma - drug therapy, Glioma - genetics, Glioma - pathology, Gliomas, Health, High-Throughput Nucleotide Sequencing, Homeopathy, Humans, Immunohistochemistry, In Situ Hybridization, Fluorescence, Kinases, MAP Kinase Signaling System - genetics, Materia medica and therapeutics, Medical prognosis, Membrane Proteins - genetics, Mitogen-Activated Protein Kinase Kinases - antagonists & inhibitors, Mitogens, Molecular Diagnostic Techniques, Molecular Targeted Therapy, Morbidity, Morphology, Mortality, Mutation, Neoplasm Grading, Neoplasms, Neuroepithelial - diagnosis, Neoplasms, Neuroepithelial - drug therapy, Neoplasms, Neuroepithelial - genetics, Neoplasms, Neuroepithelial - pathology, neuro-oncology, Neurofibromatosis, neurofibromatosis type I, Pathology, Molecular, pediatric, Pediatric low-grade glioma, Pediatric tumors, Pediatrics, Polymerase Chain Reaction, Polymorphism, Single Nucleotide, Protein Kinase Inhibitors - therapeutic use, Protein kinases, Proto-Oncogene Proteins B-raf - antagonists & inhibitors, Proto-Oncogene Proteins B-raf - genetics, Public health, ras Proteins - genetics, Receptor, Fibroblast Growth Factor, Type 1 - antagonists & inhibitors, Receptor, Fibroblast Growth Factor, Type 1 - genetics, Review, Therapeutics, Tuberous Sclerosis Complex 1 Protein - genetics, Tuberous Sclerosis Complex 2 Protein - genetics, Tumors, Up-Regulation, World health, World Health Organization