Child's Nervous System最新文献

{"title":"Molecular-targeted therapy for childhood low-grade glial and glioneuronal tumors.","authors":"Benjamin I Siegel, Elizabeth S Duke, Lindsay B Kilburn, Roger J Packer","doi":"10.1007/s00381-024-06486-6","DOIUrl":"10.1007/s00381-024-06486-6","url":null,"abstract":"<p><p>Since the discovery of the association between BRAF mutations and fusions in the development of childhood low-grade gliomas and the subsequent recognition that most childhood low-grade glial and glioneuronal tumors have aberrant signaling through the RAS/RAF/MAP kinase pathway, there has been a dramatic change in how these tumors are conceptualized. Many of the fusions and mutations present in these tumors are associated with molecular targets, which have agents in development or already in clinical use. Various agents, including MEK inhibitors, BRAF inhibitors, MTOR inhibitors and, in small subsets of patients NTRK inhibitors, have been used successfully to treat children with recurrent disease, after failure of conventional approaches such as surgery or chemotherapy. The relative benefits of chemotherapy as compared to molecular-targeted therapy for children with newly diagnosed gliomas and neuroglial tumors are under study. Already the combination of an MEK inhibitor and a BRAF inhibitor has been shown superior to conventional chemotherapy (carboplatin and vincristine) in newly diagnosed children with BRAF-V600E mutated low-grade gliomas and neuroglial tumors. However, the long-term effects of such molecular-targeted treatment are unknown. The potential use of molecular-targeted therapy in early treatment has made it mandatory that the molecular make-up of the majority of low-grade glial and glioneuronal tumors is known before initiation of therapy. The primary exception to this rule is in children with neurofibromatosis type 1 who, by definition, have NF1 loss; however, even in this population, gliomas arising in late childhood and adolescence or those not responding to conventional treatment may be candidates for biopsy, especially before entry on molecular-targeted therapy trials.</p>","PeriodicalId":9970,"journal":{"name":"Child's Nervous System","volume":null,"pages":null},"PeriodicalIF":1.3,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141320657","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Imaging of pediatric glioneuronal and neuronal tumors.","authors":"Vivek Pai, Suzanne Laughlin, Birgit Ertl-Wagner","doi":"10.1007/s00381-024-06502-9","DOIUrl":"10.1007/s00381-024-06502-9","url":null,"abstract":"<p><p>Glioneuronal tumors (GNTs) are an expanding group of primary CNS neoplasms, commonly affecting children, adolescents and young adults. Most GNTs are relatively indolent, low-grade, WHO grade I lesions. In the pediatric age group, GNTs have their epicenter in the cerebral cortex and present with seizures. Alterations in the mitogen-activated protein kinase (MAPK) pathway, which regulates cell growth, are implicated in tumorigenesis. Imaging not only plays a key role in the characterization and pre-surgical evaluation of GNTs but is also crucial role in follow-up, especially with the increasing use of targeted inhibitors and immunotherapies. In this chapter, we review the clinical and imaging perspectives of common pediatric GNTs.</p>","PeriodicalId":9970,"journal":{"name":"Child's Nervous System","volume":null,"pages":null},"PeriodicalIF":1.3,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141497264","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Stented endoscopic third ventriculostomy: technique, safety, and indications-a multicenter multinational study.","authors":"Lee Azolai, Valentina Pennacchietti, Matthias Schulz, Henry W S Schroeder, Petr Vacek, Shlomi Constantini, Lidor Bitan, Jonathan Roth, Ulrich-Wilhelm Thomale","doi":"10.1007/s00381-024-06566-7","DOIUrl":"10.1007/s00381-024-06566-7","url":null,"abstract":"<p><strong>Purpose: </strong>Endoscopic third ventriculostomy (ETV) is an effective treatment for obstructive hydrocephalus. Secondary stoma closure may be life threatening and is the most common reason for late ETV failure, mostly secondary to local scarring. Local stents intended to maintain patency are rarely used. In this study, we summarize our experience using stented ETV (sETV), efficacy, and safety.</p><p><strong>Material and methods: </strong>Data was retrospectively collected from all consecutive patients who underwent ETV with stenting at four centers. Collected data included indications for using sETV, hydrocephalic history, surgical technique, outcomes, and complications.</p><p><strong>Results: </strong>Sixty-seven cases were included. Forty had a primary sETV, and 27 had a secondary sETV (following a prior shunt, ETV, or both). The average age during surgery was 22 years. Main indications for sETV included an adjacent tumor (n = 15), thick or redundant tuber cinereum (n = 24), and prior ETV failure (n = 16). Fifty-nine patients (88%) had a successful sETV. Eight patients failed 11 ± 8 months following surgery. Reasons for failure included obstruction of the stent, reabsorption insufficiency, and CSF leak (n = 2 each), and massive hygroma and tumor spread (n = 1 each). Complications included subdural hygroma (n = 4), CSF leak (n = 2), and stent malposition (n = 1). There were no complications associated with two stent removals.</p><p><strong>Conclusion: </strong>Stented ETV appears to be feasible and safe. It may be indicated in selected cases such as patients with prior ETV failure, or as a primary treatment in cases with anatomical alterations caused by tumors or thickened tuber cinereum. Future investigations are needed to further elucidate its role in non-communicating hydrocephalus.</p>","PeriodicalId":9970,"journal":{"name":"Child's Nervous System","volume":null,"pages":null},"PeriodicalIF":1.3,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11511752/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141888602","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Role of sodium fluorescein in pediatric low-grade glioma surgery: an update.","authors":"Camilla de Laurentis, Claudia Pasquali, Pierre-Aurélien Beuriat, Carmine Mottolese, Alexandru Szathmari, Matthieu Vinchon, Federico Di Rocco","doi":"10.1007/s00381-024-06402-y","DOIUrl":"10.1007/s00381-024-06402-y","url":null,"abstract":"<p><strong>Purpose: </strong>Complete surgical resection is still the mainstay in the treatment of central nervous system low-grade tumors, eventually resulting curative. The complete surgical removal of these lesions, however, may be difficult in some cases because of their infiltrative nature. Intraoperative adjuncts may be a game changer. Sodium fluorescein (SF) is among the ideal candidates as intraoperative tools to favor the actual recognition of the tumor extension, since it accumulates in areas of altered blood-brain barrier, a typical characteristic of pediatric gliomas, and has a low rate of adverse events. This work proposes an update of previous works about the evaluation of the feasibility and usefulness of a systematic use of SF in a low-grade lesion group of pediatric patients.</p><p><strong>Methods: </strong>Pediatric patients operated on for a resection or a biopsy of a low-grade glial or glioneuronal lesion (WHO grade I and II) at our Institution between September 2021 and December 2023, with the intraoperative use of sodium fluorescein (SF), were enrolled in the study. We collected pre-operative and postoperative clinical and radiological data, intraoperative findings, and post-operative pathological diagnoses.</p><p><strong>Results: </strong>No adverse events were registered related to the intraoperative use of SF. SF appeared useful for the localization of boundaries of tumors, especially when characterized by a high degree of infiltration or by a deep-seated location, and for the checking of possible tumor remnants at the end of surgery. A good tumor-to-healthy tissue contrast was registered when tumor visualization was in a range between 1 to 2 h and 30 min after SF injection. Possible \"false positives\" due to intraoperative vascular wall injury and clearance of SF from both tumor and healthy tissue were observed in some cases and still remain open issues.</p><p><strong>Conclusions: </strong>SF is a feasible and safe intraoperative adjunct tool in the surgical removal of pediatric low-grade tumors. SF may show its usefulness especially in selected cases, such as deep-seated lesions and infiltrating tumors. Its safety profile, user-friendly management, and potential utility in both tumor resections and neuronavigated biopsies favor its wider use in the surgical treatment of pediatric low-grade tumors.</p>","PeriodicalId":9970,"journal":{"name":"Child's Nervous System","volume":null,"pages":null},"PeriodicalIF":1.3,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140862280","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Radiotherapy for pediatric low-grade glioma.","authors":"Indu Bansal, Thomas E Merchant","doi":"10.1007/s00381-024-06460-2","DOIUrl":"10.1007/s00381-024-06460-2","url":null,"abstract":"<p><strong>Introduction: </strong>Radiotherapy is a highly effective treatment for pediatric low-grade glioma, serving as the standard for evaluating progression-free and overall survival, as well as vision preservation. Despite its proven efficacy, concerns about treatment complications have led to increased use of chemotherapy and targeted therapy, which are associated with poorer progression-free survival outcomes.</p><p><strong>Methods: </strong>This review by Indu Bansal and Thomas E. Merchant examines the indications, timing, and results of radiotherapy for pediatric low-grade glioma. The authors provide a comprehensive analysis of clinical management strategies, addressing the controversies surrounding the use and timing of radiotherapy compared to other therapies.</p><p><strong>Results: </strong>The review highlights that while radiotherapy is essential for certain patients, particularly those who are not candidates for complete resection due to the tumor's infiltrative nature or location, it is often deferred in favor of systemic therapies. This deferral can lead to significant morbidity, including poor visual outcomes. Reports indicate that systemic therapy negatively impacts progression-free survival in patients who eventually undergo radiotherapy. Newer radiotherapy techniques have been developed to minimize complications, offering potential benefits over traditional methods.</p><p><strong>Discussion: </strong>The evolving clinical management of pediatric low-grade glioma involves balancing the benefits of radiotherapy with concerns about its side effects. Although systemic therapies are increasingly favored, their associated inferior progression-free survival and potential for significant morbidity underscore the need for careful consideration of radiotherapy, particularly in older children, adolescents, or those with progressive disease post-systemic therapy. The emerging role of targeted therapy presents additional challenges, including uncertainties about long-term side effects and its interaction with radiotherapy. Further research is needed to optimize treatment strategies and improve outcomes for pediatric patients with low-grade glioma.</p>","PeriodicalId":9970,"journal":{"name":"Child's Nervous System","volume":null,"pages":null},"PeriodicalIF":1.3,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141075453","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"ISPN and pediatric neurosurgery: past, present, and future-overview for our 50th anniversary.","authors":"Shlomi Constantini","doi":"10.1007/s00381-024-06589-0","DOIUrl":"10.1007/s00381-024-06589-0","url":null,"abstract":"","PeriodicalId":9970,"journal":{"name":"Child's Nervous System","volume":null,"pages":null},"PeriodicalIF":1.3,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142139402","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Upper brainstem pediatric low-grade gliomas: review and neuroendoscopic approach.","authors":"Roberto Alexandre Dezena, Murillo Martins Correia, Lívia Grimaldi Abud Fujita, Daniel Gonçalves de Souza, Luiz Fernando Alves Pereira, Gustavo Branquinho Alberto, Luíza Carolina Moreira Marcolino, Larissa Batista Xavier, Samuel Pedro Pereira Silveira","doi":"10.1007/s00381-024-06527-0","DOIUrl":"10.1007/s00381-024-06527-0","url":null,"abstract":"<p><p>Pediatric brain tumors, particularly those affecting the brainstem, present a significant challenge due to their intricate anatomical location and diverse classification. This review explores the classification, anatomical considerations, and surgical approaches for pediatric brainstem tumors, focusing on recent updates from the World Health Organization (WHO) classification. Brainstem tumors encompass a spectrum from diffuse gliomas to focal intrinsic and exophytic types, each presenting unique clinical and surgical challenges. Surgical strategies have evolved with advancements in neuroimaging and surgical techniques, emphasizing approaches such as neuroendoscopy and tailored incisions to minimize damage to critical structures. Despite the complexities involved, recent developments offer promising outcomes in tumor resection and patient management, highlighting ongoing advancements in neurosurgical care for pediatric brain tumors.</p>","PeriodicalId":9970,"journal":{"name":"Child's Nervous System","volume":null,"pages":null},"PeriodicalIF":1.3,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142104811","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Molecular markers for pediatric low-grade glioma.","authors":"Adrian B Levine, Cynthia E Hawkins","doi":"10.1007/s00381-024-06639-7","DOIUrl":"10.1007/s00381-024-06639-7","url":null,"abstract":"<p><p>Over the past decade, our understanding of the molecular drivers of pediatric low-grade glioma (PLGG) has expanded dramatically. These tumors are predominantly driven by RAS/MAPK pathway activating alterations (fusions and point mutations), most frequently in BRAF, FGFR1, and NF1. Furthermore, additional second hits in tumor suppressor genes (TP53, ATRX, CDKN2A) can portend more aggressive behaviour. Accordingly, comprehensive molecular profiling-specifically genetic sequencing, often plus copy number profiling-has become critical for guiding the diagnosis and management of PLGG. In this review, we discuss the most important genetic alterations that inform on classification and prognosis of PLGG, highlighting their diagnostic and therapeutic relevance.</p>","PeriodicalId":9970,"journal":{"name":"Child's Nervous System","volume":null,"pages":null},"PeriodicalIF":1.3,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142388453","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Pediatric-type diffuse low-grade gliomas.","authors":"Asthik Biswas, Harini R Rao, Matthias W Wagner","doi":"10.1007/s00381-024-06500-x","DOIUrl":"10.1007/s00381-024-06500-x","url":null,"abstract":"<p><p>The World Health Organization's 5th edition classification of Central Nervous System (CNS) tumors differentiates diffuse gliomas into adult and pediatric variants. Pediatric-type diffuse low-grade gliomas (pDLGGs) are distinct from adult gliomas in their molecular characteristics, biological behavior, clinical progression, and prognosis. Various molecular alterations identified in pDLGGs are crucial for treatment. There are four distinct entities of pDLGGs. All four of these tumor subtypes exhibit diffuse growth and share overlapping histopathological and imaging characteristics. Molecular analysis is essential for differentiating these lesions.</p>","PeriodicalId":9970,"journal":{"name":"Child's Nervous System","volume":null,"pages":null},"PeriodicalIF":1.3,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141455664","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Cylinder tumor surgery in pediatric low-grade gliomas.","authors":"Sebastian Gaston Jaimovich, Kazuhito Takeuchi, Victoria Tcherbbis Testa, Eriko Okumura, Roberto Jaimovich, Giuseppe Cinalli","doi":"10.1007/s00381-024-06417-5","DOIUrl":"10.1007/s00381-024-06417-5","url":null,"abstract":"<p><strong>Background: </strong>Periventricular pediatric low-grade gliomas (pLGG) present a surgical challenge due to their deep-seated location, accessibility, and relationship with the subcortical network connections. Minimally invasive parafascicular approaches with tubular brain retractors (port brain surgery) have emerged, in recent years, as an alternative to conventional microsurgical and endoscopic approaches for removal of periventricular tumors.</p><p><strong>Objectives: </strong>To describe the minimally invasive approach with tubular brain retractors for periventricular pLGG, its technique, applications, safety, and efficacy.</p><p><strong>Methods: </strong>In this article, we describe the port brain surgery techniques for periventricular pLGG as performed in different centers, with different commercialized tubular retractor systems. Illustrative cases followed by a literature review are analyzed, with a detailed description of different approaches or techniques, comparing their advantages and disadvantages with contemporary microsurgical and endoscopic approaches.</p><p><strong>Conclusions: </strong>The port brain surgery with micro-exoscopic vision and endoscopic assistance, for the treatment of deep-seated lesions such as periventricular pLGG, is an alternative for achieving a functionally safe-gross total or subtotal-tumor resection, obtaining adequate tissue for pathological examination. This technique could offer a new dimension for a less-invasive, safe, and effective access to deep-seated tumors, offering the possibility to lower morbidity in experienced hands.</p>","PeriodicalId":9970,"journal":{"name":"Child's Nervous System","volume":null,"pages":null},"PeriodicalIF":1.3,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140850674","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}