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Glioblastoma multiforme (GBM) is a complex and aggressive brain tumor that presents significant diagnostic and therapeutic challenges in both adults and children. Understanding the pathogenesis, molecular biology, symptom presentation, and imaging features of GBM is vital for effective therapy. This review summarizes current knowledge on pediatric GBM, specifically Pediatric Diffuse High-Grade Gliomas (pHGG), focusing on diagnosis and treatment. GBM typically arises from the cerebral hemispheres, with gross features marked by heterogeneous morphology and aggressive cell populations. Recent advances in genomic research have shed light on distinct molecular pathways associated with primary and secondary GBMs. Clinical symptoms vary widely but commonly include neurological deficits and increased intracranial pressure. Magnetic resonance imaging (MRI), with its excellent soft tissue contrast, is crucial for diagnosing and monitoring GBM. Emerging techniques, such as diffusion-weighted imaging (DWI) and perfusion-weighted imaging (PWI), provide insights into the tumor's microstructure and vascularity, assisting in the development of therapeutic strategies and response assessment. Despite advances in imaging, challenges remain in accurately diagnosing and managing pediatric GBM due to its molecular heterogeneity and unique biological behavior. New therapeutic approaches, including targeted therapies and immunotherapy, offer hope for improving outcomes in children with GBM. Clinical trials are ongoing to evaluate these treatments alongside standard options, such as surgery, radiotherapy, and chemotherapy, to meet the unmet needs of pediatric oncology. A multidisciplinary approach, tailored to the individual characteristics of both the patient and the tumor, is essential to optimize treatment and outcomes for pediatric GBM patients. This review highlights the role of advanced MRI techniques in diagnosis, treatment, and monitoring while emphasizing the need for further research and clinical trials to develop more effective therapies for this devastating disease. Recent studies indicate a median survival rate of 12-18 months for pediatric GBM, with treatment response varying based on molecular subtypes. Clinical trials show that IDH-wild-type tumors exhibit poorer prognosis, whereas targeted therapies are improving outcomes in select patient groups.
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