Low-grade glioma in children

Low-grade gliomas are common tumors of the central nervous system in children.
Kelly L. Collins and Ian F. Pollack of pediatric neurosurgery at the University of Pittsburgh review the classification, pathological and imaging manifestations of low-grade gliomas in children, new advances in surgery and complementary therapies, and new therapies, published online May 2020 in Cancers.
the results of the study, adult gliomas are mostly highly malignant, while children's gliomas are mainly low-level malignant.
low-level gliomas in adults are mostly evolving to a high level, while the incidence of malignant tumors in children is low, which is related to different genetic mutations in adults and children.
are common in adult glioma IDH mutants and TP53 mutants, while gliomas in children are rare and at ATRX and 1p19q code loss are common.
In-depth understanding of the molecular and genetic characteristics of glioma in children, to further improve the classification and treatment of tumors, such as the identification of the main cause of glioma in children is the silk rift active protein kinase (MAPK) signaling path path of genetic mutations, braF gene can be used as a therapeutic target.
, Tumor Pathology Classification According to the WHO Classification of Central Nervous System Tumors 2016, children's gliomas are divided into the following categories: 1. diffuse astrocytoma (diffuse astrocytoma): is a WHO II.level;
2. hairy cellular astrocytoma (pilocytic astrocytoma): is a WHO I.class, characterized by GFAP-positive mutations in dense bipolar astrocyte regions, alternating with sparsely sparse sacs of cells with clear boundaries, which can be seen in other gene mutations in the BRAF gene or MAPK signaling pathracies.
3. Polymorphic yellow cytoma (pleomorphic xanthoastrocytoma): is of WHO II.class;
4. Subependymal giant cell cell astrocytoma (subependymal giant cell astrocytoma): a WHO level;
histologically, these tumors contain large obese cells, shuttle cells, and neurocytocyte-like astrological glial cells, and the structure of the false fence around the blood vessels is visible in the mirror, but there is no silk division.
may be related to the imbalance of the mTOR signal.
5. Ganglioglioma: is a WHO I.class and consists of highly differentiated bicyte nerve cells in the context of astrological glial cells or occasionally less protrusive glial cells.
visible BRAF gene mutations, especially brafV600E mutations.
6. Fibrogenic infant astroblastoma and neurotic glioblastoma (desmoplas infantile astrocytoma and ganglioglioma): who belongs to THE I. level;
7. Embryonic dysembryoplasty neuroepithelial tumor: a WHO I. grade;
2. Clinical symptoms and imaging The common symptoms of low-level gliomas are seizures or degenerative neurofunctional deficiencies, such as weakness, sensory loss, language difficulties, visual impairment, cognitive difficulties, personality changes or decreased academic or motor performance;
symptoms progress slowly or disappear.
intracranial pressure boost can occur after hydrocephaly occurs.
ct scan tumors are of equal density or low density, often without reinforcement.
mri-T1 weighted imaging as an equivalent or low signal, T2 is weighted as a high signal and FLARE imaging as a suppressed signal.
some tumors are cystic and some are real (Figure 1).
functional magnetic resonance imaging (fMRI), dispersion imaging, body induced power generation (SSEP), and preoperative epidural subcrystrial raster electrodes or stereotactic electroencephalograms (sEEG) help functional areas locate and design surgical paths and surgical methods.
1. Typical images of low-grade gliomas in common children.
, the timing of surgery depends on tumor imaging characteristics and clinical conditions of children.
small lesions with no occupying effect can be selected for surgery or follow-up.
asymptomatic large lesions can be operated on for an optional period.
can be used for brain-outdoor draination and tumor removal when a tumor causes hydrocephaly, and in many cases, both tumor removal and brain-outdoor draination can be performed.
the tumor should be removed as a result of severe symptoms due to the placeholding effect.
principle, on-screen brain essential glioma preoperative use of anticonvulsant drugs, such as left ethyl laxitan, to prevent seizures.
children with severe cerebral edema were given a certain amount of corticosteroids before and after surgery, as appropriate.
the goal of this surgery is to obtain tumor tissue, make a clear pathological diagnosis, and remove as many tumors as possible without causing new neurological defects.
85% of children with low-grade gliomas can achieve 10 years of progression-free survival (PFS) after a full excision, while less than 50% of children with a total tumor removal achieve 10 years of PFS, so a full excision is required as much as possible.
, however, children with low-grade gliomas who have not been completely removed also have a 90% five-year survival rate;
For non-hairy cell-type gliomas with unclear boundaries, deep-soaked nuclear groups or other important areas or across the median line, it is not suitable for full excision, and should be stereotactic biopsy or partial excision of the skull.
4. Complementary treatment Children with low-grade gliomas have a good prognosis after total excision, and deep immersive tumors or shallow tumors that are not fully removed can be resection and complementary treatments, such as molecular targeted drugs, traditional chemotherapy and radiation therapy.
, the overall benefits and timing of radiation therapy for residual gliomas are not yet known.
radiation therapy appears to increase the incidence of malignant changes in residual gliomas, which can also lead to cognitive retardation, endocrine diseases and vascular lesions in children.
current radiotherapy technology allows children aged 10-12 and older with larger non-removable gliomas to safely use conventional radiotherapy.
for young children, the use of advanced radiotherapy programs can reduce the incidence of radiotherapy complications.
When young children need to avoid radiotherapy, routine chemotherapy, such as carbapus, chlorpyridoxine, 6-sulfur ostrich, prucabatin or lomostin, is not available for sustainable long-term tumor control.
, the latest progress of hairy cellular ascytoblastoma is often manifested in the BRAF gene's alternation or activation mutation, or fissurer activation protein kinase (MAPK) signaling path path changes.
is currently developing biological agents that inhibit mapK signaling pathlines, such as celesine, dabrafinib, qumeitinil, single-use or in a combined treatment with carpentry and chrysanthelio in children with the newly diagnosed BRAFV600E mutation of low-grade gliomas.
anti-angiogenesic drugs, beva bead monoantin and lanadamine have also been clinically trial.
clinical trials have found that MAPK inhibitors have some effect on diffuse asyteblastoma and polysophageal yellow asaidoma.
-targeted mTOR signal molecular preparations, such as ivemos, have good short-term tumor control effects.
the above treatment is currently in the clinical phase I. or II. trial stage.
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