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小儿后颅窝非典型畸胎样/横纹肌样瘤:一例报告、新治疗策略及展望

Pediatric Posterior Fossa ATRT: A Case Report, New Treatment Strategies and Perspectives.

作者信息

Paun Luca, Lavé Alexandre, Jannelli Gianpaolo, Egervari Kristof, Janssen Insa, Schaller Karl, von Bueren André O, Bartoli Andrea

机构信息

Division of Neurosurgery, Department of Clinical Neurosciences, Geneva University Hospitals and University of Geneva Faculty of Medicine, 1205 Geneva, Switzerland.

Department of Neurosurgery, Site Sainte-Anne, Groupe Hospitalier Universitaire Paris Psychiatrie et Neurosciences, Université Paris Cité, 75014 Paris, France.

出版信息

Brain Sci. 2023 Apr 24;13(5):712. doi: 10.3390/brainsci13050712.

Abstract

Posterior fossa atypical teratoid rhabdoid tumor (ATRT) is a rare childhood tumor usually associated with a dismal prognosis. Although upfront surgical gross total resection (GTR) has classically been the first line of treatment, new multimodal treatments, including two-stage surgery, are showing promising results in terms of overall survival (OS) and complication rate. We present a case of a 9-month-old child treated with two-staged surgery and chemotherapy. When deemed risky, multimodal treatments, including staged surgeries, can be a safe alternative to reduce surgical mortality and morbidity. At 23 months old, the patient had normal global development and no major impact on quality of life. We, therefore, discuss the most recent advancements from a treatment perspective, including molecular targeting.

摘要

后颅窝非典型畸胎样横纹肌样瘤(ATRT)是一种罕见的儿童肿瘤,通常预后较差。尽管传统上首次治疗方案是 upfront 手术全切(GTR),但包括两阶段手术在内的新多模式治疗在总生存期(OS)和并发症发生率方面显示出了有前景的结果。我们报告一例接受两阶段手术和化疗的 9 个月大儿童病例。当被认为有风险时,包括分期手术在内的多模式治疗可以作为一种安全的替代方案,以降低手术死亡率和发病率。该患者在 23 个月大时整体发育正常,对生活质量没有重大影响。因此,我们从治疗角度,包括分子靶向治疗方面,讨论了最新进展。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c0b5/10216074/7c72b0916fea/brainsci-13-00712-g001.jpg

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