The understanding of brainstem gliomas and diffuse midline gliomas has significantly increased in the last decade. However, the management paradigm remains a dilemma. The critical location is the foremost factor dictating the outcome. Recent advancements in the field of neuro-oncology are pushing the boundaries of optimal care in the developed world nevertheless, the strategies in low- and middle-income countries (LMICs) need to be tailored according to the resources to improve outcome. The objective of these guidelines is to provide an algorithm-based management plan to cater challenges for healthcare providers in LMICs.
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The role of brainstem biopsy and targeted therapies in pediatric diffuse midline glioma/diffuse intrinsic pontine glioma.
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Department of Pediatric Hematology-Oncology and Blood & Marrow Transplant, Cleveland Clinic, Cleveland, OH, United States.
Pediatric diffuse midline glioma (DMG), including diffuse intrinsic pontine glioma (DIPG), are aggressive brainstem tumors with a dire prognosis, traditionally diagnosed based on MRI characteristics. The recognition that molecular characteristics may determine prognosis and response to therapy has led to a reevaluation of biopsy necessity. This comprehensive review addresses the evolving role of brainstem biopsies in diagnosing and managing these tumors - both within the context of a clinical trial and in routine clinical care.
View Article and Find Full Text PDFA single-center experience of central nervous system tumors in children under three years old.
Front Pediatr
December 2024
Department of Neurosurgery, Tsinghua University Yuquan Hospital (Tsinghua University Hospital of Integrated Traditional Chinese and Western Medicine), Beijing, China.
Purpose: This study aims to summarize the characteristics of children under three years old (≤3 years) with central nervous system (CNS) tumors and to investigate the factors that influence their overall survival (OS) time.
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Introduction: Diffuse intrinsic pontine glioma (DIPG) in children comprises 80% of brainstem gliomas. In 2021, 5th edition of WHO CNS tumor classification defined H3K27M altered diffuse midline gliomas (DMGs) which replaced this entity. Lesion location precludes resection and the only current option available is radiotherapy.
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Serine 31 is a phospho-site unique to the histone H3.3 variant; mitotic phospho-Ser31 is restricted to pericentromeric heterochromatin, and disruption of phospho-Ser31 results in chromosome segregation defects and loss of p53-dependant G cell-cycle arrest. Ser31 is proximal to the H3.
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bioRxiv
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Yale University, Department of Molecular, Cellular and Developmental Biology, Faculty of Arts and Sciences; 260 Whitney Avenue, New Haven, Connecticut 06511, USA.
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