小儿弥漫性高级别胶质瘤:诊断与治疗先进方法的全面综述

Pediatric Diffuse High-Grade Gliomas: A Comprehensive Review Of Ad-vanced Methods Of Diagnosis And Treatment.

作者信息

Budha Roja Rani, Khan Saba Wahid, Sharma Deep Chandraprakash, Kulchandani Yash, Gsn Koteswara Rao

机构信息

H.K. College of Pharmacy, Oshiwara, Jogeshwari (W), Mumbai-400102, Maharashtra, India.

Shobhaben Pratapbhai Patel School of Pharmacy and Technology Management, SVKM's, NMIMS, Vile Parle (W), Mumbai, 400056, Maharashtra, India.

出版信息

Curr Cancer Drug Targets. 2025 Jun 30. doi: 10.2174/0115680096365252250618115641.

Abstract

Glioblastoma multiforme (GBM) is an aggressive brain tumor, posing significant challenges in diagnosis and treatment, particularly in 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, 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.Glioblastoma multiforme (GBM) is a complex and aggressive brain tumor that presents significant diagnostic and therapeutic challenges in both adults and children. Un-derstanding the pathogenesis, molecular biology, symptom presentation, and imaging fea-tures 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 ad-vances in genomic research have shed light on distinct molecular pathways associated with primary and secondary GBMs. Clinical symptoms vary widely but commonly include neu-rological deficits and increased intracranial pressure. Magnetic resonance imaging (MRI), with its excellent soft tissue contrast, is crucial for diagnosing and monitoring GBM. Emerg-ing techniques, such as diffusion-weighted imaging (DWI) and perfusion-weighted imaging (PWI), provide insights into the tumor's microstructure and vascularity, assisting in the de-velopment of therapeutic strategies and response assessment. Despite advances in imaging, challenges remain in accurately diagnosing and managing pediatric GBM due to its molec-ular 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 on-cology. 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, treat-ment, 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 me-dian 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.

摘要

多形性胶质母细胞瘤(GBM)是一种侵袭性脑肿瘤,在诊断和治疗方面带来了重大挑战,尤其是在儿童患者中。了解GBM的发病机制、分子生物学、症状表现和影像学特征对于有效治疗至关重要。本综述总结了目前关于儿童GBM的知识,重点关注诊断和治疗。GBM通常起源于大脑半球,大体特征表现为形态异质性和侵袭性细胞群。基因组研究的最新进展揭示了与原发性和继发性GBM相关的不同分子途径。临床症状差异很大,但通常包括神经功能缺损和颅内压升高。磁共振成像(MRI)因其出色的软组织对比度,对于GBM的诊断和监测至关重要。新兴技术,如扩散加权成像(DWI)和灌注加权成像(PWI),有助于了解肿瘤的微观结构和血管情况,辅助制定治疗策略和评估治疗反应。尽管影像学取得了进展,但由于其分子异质性和独特的生物学行为,在准确诊断和管理儿童GBM方面仍存在挑战。新的治疗方法,包括靶向治疗和免疫治疗,为改善GBM患儿的治疗效果带来了希望。正在进行临床试验,以评估这些治疗方法与手术、放疗和化疗等标准治疗方案相比的效果,以满足儿科肿瘤学未满足的需求。针对患者和肿瘤的个体特征量身定制的多学科方法,对于优化儿童GBM患者的治疗和预后至关重要。本综述强调了先进MRI技术在诊断、治疗和监测中的作用,同时强调需要进一步研究和开展临床试验,以开发针对这种毁灭性疾病的更有效治疗方法。多形性胶质母细胞瘤(GBM)是一种复杂的侵袭性脑肿瘤,在成人和儿童中都带来了重大的诊断和治疗挑战。了解GBM的发病机制、分子生物学、症状表现和影像学特征对于有效治疗至关重要。本综述总结了目前关于儿童GBM,特别是儿童弥漫性高级别胶质瘤(pHGG)的知识,重点关注诊断和治疗。GBM通常起源于大脑半球,大体特征表现为形态异质性和侵袭性细胞群。基因组研究的最新进展揭示了与原发性和继发性GBM相关的不同分子途径。临床症状差异很大,但通常包括神经功能缺损和颅内压升高。磁共振成像(MRI)因其出色的软组织对比度,对于GBM的诊断和监测至关重要。新兴技术,如扩散加权成像(DWI)和灌注加权成像(PWI),有助于了解肿瘤的微观结构和血管情况,辅助制定治疗策略和评估治疗反应。尽管影像学取得了进展,但由于其分子异质性和独特的生物学行为,在准确诊断和管理儿童GBM方面仍存在挑战。新的治疗方法,包括靶向治疗和免疫治疗,为改善GBM患儿的治疗效果带来了希望。正在进行临床试验,以评估这些治疗方法与手术、放疗和化疗等标准治疗方案相比的效果,以满足儿科肿瘤学未满足的需求。针对患者和肿瘤的个体特征量身定制的多学科方法,对于优化儿童GBM患者的治疗和预后至关重要。本综述强调了先进MRI技术在诊断、治疗和监测中的作用,同时强调需要进一步研究和开展临床试验,以开发针对这种毁灭性疾病的更有效治疗方法。最近的研究表明,儿童GBM的中位生存期为12 - 18个月,治疗反应因分子亚型而异。临床试验表明,异柠檬酸脱氢酶(IDH)野生型肿瘤预后较差,而靶向治疗正在改善特定患者群体的治疗效果。

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