
Contents
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Introduction Introduction
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Epidemiology and etiology Epidemiology and etiology
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Histopathology and molecular pathogenesis Histopathology and molecular pathogenesis
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Proliferation Proliferation
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Grading Grading
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Immunophenotype and molecular profile Immunophenotype and molecular profile
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Clinical presentation Clinical presentation
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Imaging Imaging
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Management Management
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Surgery Surgery
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Radiotherapy Radiotherapy
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Grade, target volume, and dosage Grade, target volume, and dosage
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Perspectives on proton therapy Perspectives on proton therapy
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Systemic pharmacotherapy Systemic pharmacotherapy
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IDH inhibitors IDH inhibitors
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Other agents Other agents
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Recurrent tumors Recurrent tumors
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Future directions Future directions
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Prognosis, quality of life, survivorship, palliative care Prognosis, quality of life, survivorship, palliative care
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Surveillance recommendations Surveillance recommendations
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Commentary on EANO and NCCN guidelines Commentary on EANO and NCCN guidelines
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References References
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Cite
Abstract
Astrocytoma, isocitrate dehydrogenase (IDH)-mutant is diagnosed by the presence of an IDH mutation and no 1p/19q codeletion, usually with loss of ATRX expression. Based on histological criteria three grades are distinguished, the presence of homozygous deletion of the CDKN2A gene in the updated results in the World Health Organization 2021 central nervous system tumor classification also is a grade 4 diagnosis. Grade 2 and most grade 3 IDH-mutant astrocytomas are slowly growing tumors, with a median survival of 9–11 years. Surgery is an essential element of treatment. Especially in grade 2 tumors, tumor volumes before and after surgery are major predictive factors for outcome. IDH-mutant astrocytomas are sensitive to radiotherapy and chemotherapy; the optimal treatment for patients requiring treatment after surgery is radiotherapy followed by chemotherapy. Recent trials have shown that IDH inhibitors modify the growth of grade 2 non-enhancing astrocytoma, this is likely to become part of standard of care after surgery and prior to further treatments.
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