儿童后窝血管母细胞瘤:文献回顾

Aline Rabelo Rodrigues, Pedro Lucas Bessa Dos Reis, Gabriel Bagarolo Petronilho, Caio Araujo De Souza, Pamela Lopes Carvalho, Ricardo Santos de Oliveira, Rodrigo Inácio Pongeluppi, Matheus Ballestero
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引用次数: 0

摘要

后颅窝血管母细胞瘤(HBs)是一种少见的良性肿瘤,占颅内肿瘤的1.5-2.5%,占后颅窝肿瘤的7-8%。它们主要影响中枢神经系统,通常局限于50-60岁个体的小脑半球,在成人中发病率较高,在儿科人群中发病率有限。方法:采用PRISMA指南,对SciELO、Lilacs和PubMed等数据库进行了细致的检索,并采用关键词“血管母细胞瘤”和“后窝”。纳入标准涵盖2003-2023年以英语或葡萄牙语发表的文章,重点关注儿科病例。结果:从PubMed检索到16篇相关文章,从Lilacs检索到72篇相关文章,SciELO没有检索到相关结果。根据预先确定的标准进行严格评估,从Lilacs中确定了10篇讨论儿童后窝HBs的相关文章,包括影像学、病理生理学、免疫组织化学和治疗方式等主题。探讨了不同的主题,包括妊娠相关表现,肿瘤生长模式,以及手术切除,放疗,神经导航和术前栓塞等治疗方法。先进的成像技术,如高分辨率三维多融合医学成像,也进行了探索。结论:早期诊断和综合多学科策略对治疗儿童后窝HBs至关重要。遗传洞察和先进的成像技术,加上精确的手术干预,在改善预后和提高患者的生活质量方面发挥着关键作用。VHL突变的遗传背景,MRI在早期检测中的中心地位,以及完全手术切除的重要性,强调了对有效治疗不可或缺的多方面理解。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Posterior fossa hemangioblastoma in children: literature review
Introduction: Posterior fossa hemangioblastomas (HBs) are uncommon benign neoplasms, comprising 1.5-2.5% of intracranial tumors and 7-8% of posterior cranial fossa tumors. They predominantly impact the central nervous system, often localizing within the cerebellar hemispheres of individuals aged 50-60 years, with higher prevalence among adults and limited occurrence in the pediatric population. This review sheds light on the distinctive clinical characteristics of pediatric HBs Methods: Employing the PRISMA guidelines, databases like SciELO, Lilacs, and PubMed were meticulously searched, deploying keywords "hemangioblastoma" and "posterior fossa." Inclusion criteria spanned articles published from 2003-2023 in English or Portuguese, focusing on pediatric cases. Results: The search yielded 16 relevant articles from PubMed and 72 from Lilacs, with SciELO yielding no relevant results. Rigorous assessment against the predetermined criteria identified 10 pertinent articles from Lilacs discussing pediatric posterior fossa HBs, encompassing topics like imaging, pathophysiology, immunohistochemistry, and therapeutic modalities. Diverse themes were explored, including pregnancy-related manifestations, tumor growth patterns, and treatment approaches such as surgical resection, radiotherapy, neuronavigation, and presurgical embolization. Advanced imaging techniques, like high-resolution 3D multifusion medical imaging, were also explored. Conclusion: Early diagnosis and a comprehensive multidisciplinary strategy are essential in managing pediatric posterior fossa HBs. Genetic insights and advanced imaging techniques, coupled with precise surgical interventions, play a pivotal role in improving prognosis and elevating the quality of life for patients. The genetic context of VHL mutations, the centrality of MRI in early detection, and the prominence of complete surgical resection underscore the multifaceted comprehension indispensable for effective management.
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