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Intracranial Aneurysm in Pediatric Population: A Single-Center Experience 颅内动脉瘤在儿科人群:单中心经验
IF 0.7 4区 医学 Q4 CLINICAL NEUROLOGY Pub Date : 2022-04-08 DOI: 10.1159/000524523
G. D. de Aguiar, A. Ozanne, A. Elawady, T. Samoyeau, H. Niknejad, J. Caroff, Jeickson Vergara Martinez, C. Mihalea, S. Gallas, V. Chalumeau, L. Ikka, J. Moret, L. Spelle
Purpose: Pediatric intracranial aneurysms (PIA) are rarer and more complex when compared to adult aneurysms. In general, the clinical presentation of PIA is due to a mass effect, but the presenting symptoms can be also related to ischemia, subarachnoid hemorrhage (SAH), or in a combination of different symptoms. This paper aimed to report a single-center experience with clinical and angiographic aspects of brain aneurysm in children. Methods: We retrospectively reviewed our prospectively maintained database for patients with intracranial aneurysms in our institution from July 2015 to February 2021. Among these, all patients under 18 years of age submitted to a diagnostic or therapeutic procedure for an intracranial aneurysm were included. Results: Twelve patients were submitted to diagnostic or therapeutic procedures in our department. Three of them had multiple aneurysms, and in total, 17 intracranial aneurysms were assessed in this study. The most frequent location was in the middle cerebral artery (7 cases/41%). Five out of twelve children (42%) presented SAH due to ruptured aneurysm. Three patients (25%) had symptoms due to the mass effect from large aneurysms, with compression of cranial nerves or brainstem. Aneurysms diameters ranged from 1.5 mm to 34 mm (mean 14.2 mm), with six aneurysms being giant and eight being nonsaccular/fusiform. Twelve aneurysms were submitted to endovascular treatment, with one treatment-related clinical complication and later death. Conclusion: PIAs are rare diseases that can arise from a variety of different underlying pathological mechanisms. The management of these conditions requires a detailed understanding of the pathology and a multidisciplinary approach. Despite the availability of new technologies, parent vessel occlusion remains a valid option for aneurysms in the pediatric population.
目的:与成人动脉瘤相比,儿童颅内动脉瘤(PIA)更为罕见和复杂。一般来说,PIA的临床表现是由于肿块效应,但所表现的症状也可能与缺血、蛛网膜下腔出血(SAH)或不同症状的组合有关。本文旨在报告儿童脑动脉瘤的临床和血管造影方面的单中心经验。方法:回顾性分析2015年7月至2021年2月在我院前瞻性维护的颅内动脉瘤患者数据库。其中,所有年龄在18岁以下的因颅内动脉瘤而接受诊断或治疗的患者均包括在内。结果:12例患者在我科接受诊断或治疗。其中3例有多发动脉瘤,本研究共评估颅内动脉瘤17例。多发部位为大脑中动脉(7例/41%)。12名儿童中有5名(42%)因动脉瘤破裂而出现SAH。3例患者(25%)因大动脉瘤的肿块效应而出现症状,并压迫脑神经或脑干。动脉瘤直径从1.5 mm到34 mm不等(平均14.2 mm),其中6个为巨型动脉瘤,8个为非囊状/梭状动脉瘤。12个动脉瘤接受血管内治疗,一例治疗相关的临床并发症和后来的死亡。结论:pia是一种罕见的疾病,可能由多种不同的潜在病理机制引起。这些条件的管理需要详细了解病理和多学科的方法。尽管新技术的可用性,家长血管闭塞仍然是儿童动脉瘤的有效选择。
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引用次数: 4
The Eyebrow Approach for the Management of Pediatric Frontal Epidural Abscesses Secondary to Diffuse Sinusitis 眉入路治疗小儿额部弥漫性鼻窦炎继发硬膜外脓肿
IF 0.7 4区 医学 Q4 CLINICAL NEUROLOGY Pub Date : 2022-04-08 DOI: 10.1159/000524537
Hana Hallak, Hussam Abou-Al-Shaar, A. Mallela, M. Mcdowell, Noel Jabbour, R. Padia, S. Greene, I. Pollack
Background: Minimally invasive approaches to the anterior cranial fossa have evolved over the past few decades. The management of frontal epidural abscesses (EDAs) secondary to diffuse sinusitis in the pediatric population using minimally invasive techniques is scarcely reported in the literature. Herein, we report the utilization of a minimally invasive eyebrow approach for multidisciplinary concurrent evacuation of frontal EDA secondary to diffuse sinusitis and trephination of the frontal sinus in three pediatric patients. Case Reports: Three pediatric patients presented to the emergency room with severe headaches, visual changes, somnolence, and significant facial and periorbital swelling. Imaging revealed diffuse sinusitis with focal frontal epidural extension. In all cases, progressive clinical deterioration along with the radiographic findings mandated urgent surgical intervention. The eyebrow approach allowed for concomitant evacuation of the frontal EDA and trephination of the frontal sinus followed by functional endoscopic sinus surgery in the same setting. All patients tolerated the procedure well with complete resolution of their symptoms at the completion of antibiotic therapy and complete resolution of the EDA. Conclusion: The eyebrow approach is a minimally invasive technique that should be considered as part of the armamentarium in the management of select EDA in the pediatric population. It allows for multidisciplinary collaboration between neurosurgeons and otolaryngologists for concomitant evacuation of the EDA and trephination of the frontal sinus. This approach is a feasible, safe, and effective minimally invasive technique that can be employed for the management of EDA secondary to diffuse sinusitis in the pediatric population.
背景:在过去的几十年里,颅前窝的微创入路不断发展。应用微创技术治疗小儿漫漫性鼻窦炎继发额部硬膜外脓肿(EDAs)的文献报道很少。在此,我们报告了3例小儿弥漫性鼻窦炎和额窦穿刺术的应用微创眉入路多学科并发额叶EDA清除术。病例报告:三名儿科患者因严重头痛、视力改变、嗜睡和明显的面部和眶周肿胀而被送往急诊室。影像学显示弥漫性鼻窦炎伴局灶额部硬膜外延伸。在所有病例中,进行性临床恶化和影像学表现要求紧急手术干预。眉毛入路允许同时清除额叶EDA和额窦穿刺,然后在相同的情况下进行功能性内窥镜鼻窦手术。所有患者都能很好地耐受该手术,在抗生素治疗结束时症状完全缓解,EDA完全缓解。结论:眉入路是一种微创技术,应作为治疗儿童选择性EDA的手段之一。它允许神经外科医生和耳鼻喉科医师之间的多学科合作,以同时清除EDA和额窦穿刺。该方法是一种可行、安全、有效的微创技术,可用于治疗儿童人群中继发于弥漫性鼻窦炎的EDA。
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引用次数: 1
Xanthogranulomatous Colloid Cyst in a 13-Year-Old Boy: A Case Report and Surgical Implications 13岁男童黄色肉芽肿性胶体囊肿1例及手术意义
IF 0.7 4区 医学 Q4 CLINICAL NEUROLOGY Pub Date : 2022-04-05 DOI: 10.1159/000524480
G. Tyagi, G. Singh, M. Beniwal, Dwarakanath Srinivas
Introduction: Colloid cysts are relatively uncommon lesions in the pediatric population. The xanthogranulomatous (XG) variant is very rare with less than 30 reported cases. Case Report: In this report, the patient was a 13-year-old boy who presented with transient episodes of headache with blurring of vision. His MRI brain showed a T2 hyperintense well-defined cystic lesion, with an eccentrically located T2 hypointense partially enhancing nodule, at the foramen of Monro. He underwent middle frontal gyrus transcortical, transchoroidal gross total excision of the cyst. The histopathology of the lesion revealed an XG colloid cyst. The patient recovered well from the procedure and was relieved of the symptoms. Conclusion: XG colloid cyst may present with altered radiological features compared to the normal variant. This can pose a diagnostic dilemma, and it is important to differentiate it from a craniopharyngioma or a parasitic cyst, as in our case. When considered preoperatively, surgeons should be conscious to review their surgical strategies. Stereotactic aspiration of the XG cyst should be avoided as contents are thicker and heterogeneous than the usual. The spillage of cyst contents should be prevented. Also, the XG cysts are likely to have a poor cyst-fornix or -choroid plexus interface due to inflammation limiting complete resection.
简介:胶体囊肿是儿科人群中相对罕见的病变。黄色肉芽肿(XG)变种是非常罕见的,少于30例报告。病例报告:在这个报告中,病人是一个13岁的男孩,他表现为短暂的头痛发作和视力模糊。他的MRI显示,在Monro孔处,T2高信号清晰的囊性病变,伴偏位T2低信号部分增强的结节。他接受了中额回经皮质、经脉络膜囊肿的大体全切除。病变组织病理显示为XG胶体囊肿。病人手术后恢复良好,症状减轻。结论:与正常样囊肿相比,XG胶体囊肿的影像学表现可能有所改变。这可能会造成诊断上的困境,将其与颅咽管瘤或寄生囊肿(如本病例)区分开来是很重要的。当术前考虑时,外科医生应该有意识地回顾他们的手术策略。由于XG囊肿的内容物比平常更厚且不均匀,应避免立体定向穿刺。应防止囊肿内容物外溢。此外,由于炎症限制了完全切除,XG囊肿可能有一个较差的囊肿穹窿或脉络丛界面。
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引用次数: 0
Cervicomedullary Gliomas in Pediatric Age: A Systematic Review of the Literature and Tertiary Care Center Experience 儿童年龄的颈髓核胶质瘤:文献和三级护理中心经验的系统回顾
IF 0.7 4区 医学 Q4 CLINICAL NEUROLOGY Pub Date : 2022-03-18 DOI: 10.1159/000524165
A. Trezza, Camilla de Laurentis, V. Biassoni, G. Carrabba, E. Schiavello, F. Canonico, P. Remida, Alessandra Moretto, M. Massimino, C. Giussani
Introduction: Cervicomedullary gliomas (CMGs) are usually low-grade tumors often found in the pediatric age. Histological findings, treatments, and classification have been much the same for 40 years, although histological and molecular classifications have largely been developed for other pediatric CNS tumors. The management and treatment of pediatric CMG are still conducted by many authors according to their anatomical location and characteristics, independently from histology. Methods: We conducted a literature review in PubMed (Medline) to identify relevant contributions about pediatric CMG published until December 31, 2021. We also analyzed a series of 10 patients with CMG treated from 2006 to 2021 at IRCCS Istituto Nazionale dei Tumori. The aim of the present review was to see whether and how the diagnosis, treatment, and classification of CMGs in children have developed over time, especially in the context of molecular advancements, and to analyze our single-center experience in the last 15 years. Results: Thirty articles have been included in the review. Articles have been divided into two historical periods (1981–2000 and 2001–2021) and data from different series were analyzed to see how much the management and treatment of pediatric CMG have changed during years. Analysis of our series of 10 patients affected by CMG was also performed to compare it with the literature. Discussion: Management and classification of CMG in children have not dramatically changed during years. However, new insight from molecular diagnostics and target therapies and the development of radiological, neurophysiological, and radiotherapy techniques have updated treatment modalities in the last 20 years. Treatment modalities and their innovations have been reviewed and discussed. Further studies are needed to standardize and customize treatment protocols for these tumors.
引言:颈髓核胶质瘤(CMGs)通常是儿童年龄段常见的低级别肿瘤。40年来,组织学发现、治疗和分类基本相同,尽管组织学和分子分类在很大程度上是针对其他儿童中枢神经系统肿瘤制定的。儿童CMG的管理和治疗仍然由许多作者根据其解剖位置和特征进行,独立于组织学。方法:我们在PubMed(Medline)上进行了一项文献综述,以确定截至2021年12月31日发表的关于儿科CMG的相关贡献。我们还分析了2006年至2021年在IRCCS国家肿瘤研究所接受治疗的10名CMG患者。本综述的目的是了解儿童CMG的诊断、治疗和分类是否以及如何随着时间的推移而发展,特别是在分子进步的背景下,并分析我们在过去15年中的单中心经验。结果:共有30篇文章被纳入综述。文章分为两个历史时期(1981-2000年和2001-2021年),分析了不同系列的数据,以了解多年来儿科CMG的管理和治疗发生了多大变化。我们还对10名CMG患者进行了分析,将其与文献进行了比较。讨论:多年来,儿童CMG的管理和分类没有显著变化。然而,在过去20年中,分子诊断和靶向治疗的新见解以及放射学、神经生理学和放射治疗技术的发展更新了治疗模式。对治疗方式及其创新进行了审查和讨论。需要进一步的研究来标准化和定制这些肿瘤的治疗方案。
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引用次数: 1
Crouzon’s Syndrome with a Dominant Sinus Pericranii Draining Transverse Sinus: Report of a Rare Association and Review of Literature 克鲁松综合征伴优势鼻窦围壁引流横鼻窦:罕见关联报告及文献回顾
IF 0.7 4区 医学 Q4 CLINICAL NEUROLOGY Pub Date : 2022-03-18 DOI: 10.1159/000524134
S. Kumari, S. Verma, P. Singh
Introduction: Crouzon’s syndrome and sinus pericranii (SP) are rare entities. Only few cases having both the features are reported. SP most commonly drains in relation to superior sagittal sinus and their communication to major posterior dural sinuses is rare. Case Report: We report a rare case of Crouzon’s syndrome with SP at a suboccipital location with termination of left transverse sinus into the SP draining further through the extracranial suboccipital and extravertebral cervical venous plexi into external jugular veins. Distal transverse sinus and sigmoid sinus on the left side were absent. Conclusion: Crouzon’s syndrome with SP is an extremely rare entity. SP with communication to major posterior dural venous sinuses is also rare and mostly associated with multi-suture craniosynostosis. Management depends on the volume of venous blood they are draining. Most of them are dominant type and their occlusion is not feasible. Preoperative diagnosis of a dominant SP is essential for proper surgical planning as it needs to be preserved mandatorily to prevent cerebral venous infarction.
简介:Crouzon综合征和颅周窦(SP)是罕见的实体。只有少数病例同时具有这两种特征。SP最常与上矢状窦相连,与硬脑膜后窦相通的情况很少。病例报告:我们报告一例罕见的Crouzon综合征伴枕下位置的SP,左侧横窦终止进入SP,通过颅外枕下和椎外颈静脉丛进入颈外静脉。左侧远端横窦和乙状窦未见。结论:伴有SP的Crouzon综合征是一种极为罕见的疾病。与硬脊膜后静脉窦相通的SP也很少见,且多与多缝合线颅缝闭塞有关。治疗取决于他们抽走的静脉血量。多数为优势型,闭塞不可行。术前诊断优势性脑脊液对于正确的手术计划至关重要,因为它需要被强制保留以防止脑静脉梗死。
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引用次数: 0
The Challenges of Making Comparisons between Research and Clinical Cohorts 在研究和临床队列之间进行比较的挑战
IF 0.7 4区 医学 Q4 CLINICAL NEUROLOGY Pub Date : 2022-03-17 DOI: 10.1159/000524141
A. Houtrow
Letters to the editor do not have abstracts, I believe.
我相信,给编辑的信是没有摘要的。
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引用次数: 1
Sella Bugged! Abscess Inside a Craniopharyngioma: Case Report with Literature Review Sella Bugged!颅咽管瘤内脓肿1例报告并文献复习
IF 0.7 4区 医学 Q4 CLINICAL NEUROLOGY Pub Date : 2022-03-09 DOI: 10.1159/000523977
G. Tyagi, Santhosh Kumar S A, Sandeep Kandregula, Radhika Mhatre, A. Uppar
Introduction: Abscess within a craniopharyngioma (CPG) is extremely rare and only 8 such cases have been reported in literature. Most patients present with hypopituitarism and visual disturbances. We report the first ever case of a CPG with abscess in a pediatric patient. Case Report: A 10-year-old girl presented with visual deterioration and bitemporal hemianopia. Her CT and MRI brain suggested of a sellar-suprasellar CPG. Due to ill-developed sino-nasal anatomy, a transcranial approach was made for the lesion. The lesion was well capsulated, thick walled, and appeared inflamed. Upon incising the wall, thick yellowish pus was drained out in a controlled manner. This was followed by a partial resection of the CPG wall and eccentric, adhered, calcified residue was left behind with an Ommaya drain. The abscess culture grew Enterococcus species and histopathology revealed adamantinomatous CPG. Patient underwent culture sensitive antibiotics course followed by radiation for the residue. She was doing well at 1-year follow-up with clinical and radiological improvement. Conclusion: This is the first report of a pediatric case with secondary abscess in CPG. Operative management of such a case includes controlled drainage of pus without dissemination into the surrounding arachnoid space. The tumor and abscess have to be addressed as separate surgical entities; infection control and wherever complete resection is not feasible, partial safe resection followed by radiotherapy is a viable option.
引言:颅咽管瘤内脓肿极为罕见,文献中仅报道了8例此类病例。大多数患者表现为垂体功能减退和视觉障碍。我们报告了有史以来第一例CPG并发脓肿的儿科患者。病例报告:一名10岁女孩出现视力下降和双颞侧偏盲。她的CT和MRI大脑显示为鞍上CPG。由于鼻窦解剖结构发育不良,对病变进行了经颅入路治疗。病变包膜良好,壁厚,出现炎症。切开壁后,粘稠的黄色脓液以可控的方式排出。随后对CPG壁进行了部分切除,并用Ommaya引流管留下了偏心、粘附、钙化的残留物。脓肿培养生长出肠球菌种,组织病理学显示为造釉细胞性CPG。患者接受了对培养敏感的抗生素疗程,随后对残留物进行了放射治疗。她在一年的随访中表现良好,临床和放射学有所改善。结论:这是第一例CPG继发脓肿的儿科病例报告。这种病例的手术治疗包括控制脓液的排出,而不扩散到周围的蛛网膜间隙。肿瘤和脓肿必须作为单独的外科实体来处理;感染控制,在完全切除不可行的地方,部分安全切除后放疗是可行的选择。
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引用次数: 0
Retained Medullary Cord Associated with Terminal Myelocystocele and Intramedullary Arachnoid Cyst 终末髓囊性囊肿和髓内蛛网膜囊肿相关的保留髓索
IF 0.7 4区 医学 Q4 CLINICAL NEUROLOGY Pub Date : 2022-03-08 DOI: 10.1159/000523976
Ai Kurogi, N. Murakami, N. Mukae, T. Shimogawa, T. Shono, Satoshi O. Suzuki, T. Morioka
Introduction: The retained medullary cord (RMC) is a newly defined entity of closed spinal dysraphism that is thought to originate from regression failure of the medullary cord during the last phase of secondary neurulation. The terminal myelocystocele (TMC) is an unusual type of closed spinal dysraphism, characterized by localized cystic dilatation of the terminal part of the central canal that then herniates through a posterior spinal bifida. The co-occurrence of RMC and TMC is extremely rare. Case Presentation: We treated a baby girl with a huge sacrococcygeal meningocele-like sac with two components. Untethering surgery and repair surgery for the sac revealed that RMC, associated with intramedullary arachnoid cyst (IMAC), was terminated at the bottom of the rostral cyst, forming the septum of the two cystic components, and the caudal cyst was TMC derived from the central canal-like ependymal lining lumen (CC-LELL) of the RMC at the septum. IMAC within the RMC communicated with TMC, and both contained xanthochromic fluid with the same properties. Conclusion: We speculated that the mass effect of the coexistent IMAC impeded the flow of cerebrospinal fluid in the CC-LELL within the RMC and eventually formed a huge TMC. In surgical strategies for such complex pathologies, it is important to identify the electrophysiological border between the functional cord and nonfunctional RMC and the severe RMC to untether the cord, as with a typical or simple RMC.
髓系保留(RMC)是一种新定义的闭合性脊柱发育异常,被认为是源于继发神经末期髓系退行性失败。终末髓囊性膨出(TMC)是一种不寻常的闭合性脊柱发育异常,其特征是中央椎管末端局部囊性扩张,然后通过后脊柱裂突出。RMC和TMC同时发生是极为罕见的。病例介绍:我们治疗了一个女婴的巨大骶尾骨脑膜样囊,有两个组成部分。结扎术和囊腔修复术显示,RMC伴髓内蛛网膜囊肿(IMAC)终止于鼻侧囊肿底部,形成两个囊腔成分的隔膜,尾端囊肿为RMC中隔的中央管样室管膜内衬管腔(CC-LELL)衍生的TMC。RMC内的IMAC与TMC连通,两者都含有具有相同性质的色原液。结论:我们推测共存的IMAC的质量效应阻碍了RMC内CC-LELL内脑脊液的流动,最终形成巨大的TMC。在治疗此类复杂病理的手术策略中,重要的是要确定功能性脊髓和非功能性RMC之间的电生理边界,以及严重的RMC以解开脊髓,就像典型或简单的RMC一样。
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引用次数: 1
MicroRNA-138 Regulates Spinal Cord Development by Activating the Shh in Fetal Rats. MicroRNA-138通过激活胎鼠Shh调控脊髓发育。
IF 0.7 4区 医学 Q4 CLINICAL NEUROLOGY Pub Date : 2022-01-01 DOI: 10.1159/000527587
Zheng Ma, Cui-Yun Li, Li-Juan Wang, Yan Xia, Cheng-An Feng, Yu-Fang Peng, Yan-Bing Han, Yan Fan, Ying-Chun Ba

Introduction: Dysregulation of spinal cord development can lead to serious neuronal damage and dysfunction, causing significant health problems in newborns. MiRNA-138 appears to be crucial for proliferation, differentiation, and apoptosis of cells. However, the regulation of miRNA-138 and downstream molecules in embryonic spinal cord development remain elusive. The aim of this experiment is to determine whether overexpression of miRNA-138 or RNA interference (RNAi) can regulate the development of spinal cord in fetal rats.

Methods: Two plasmid vectors including pLenti-III-mico-GFP (miRNA-138 open reading frame [ORF]) and pLenti-III-miR-Off (miRNA-138 short hairpin) were constructed and injected into the tail vein of rats on the 14th day of pregnancy. Hematoxylin-eosin (HE) staining was used to observe the cell morphology. QRT-PCR, Western blot, and immunostaining confirmed the regulatory relationship between miRNA-138 and downstream molecules sonic hedgehog (Shh).

Results: Overexpression of miRNA-138 increased neuron regeneration significantly and decreased neuronal apoptosis when compared with the control. Silencing of miRNA-138 increased neuronal apoptosis and spinal cord atrophy significantly. Furthermore, miRNA-138 ORF treatment effectively increased the expression level of miRNA-138 and also upregulated the level of Shh. Comparatively, knockdown of miRNA-138 downregulated Shh levels in myelodysplastic regions.

Conclusion: These findings indicated that miRNA-138 overexpression could protect the spinal cord development of fetal rats, and the underlying mechanisms were associated with Shh expression. The present study provides a novel strategy to promote the molecular mechanism of embryonic spinal cord development.

脊髓发育失调可导致严重的神经元损伤和功能障碍,对新生儿造成严重的健康问题。MiRNA-138似乎对细胞的增殖、分化和凋亡至关重要。然而,miRNA-138及其下游分子在胚胎脊髓发育中的调控作用尚不明确。本实验旨在确定miRNA-138过表达或RNA干扰(RNAi)是否可以调节胎鼠脊髓的发育。方法:构建mgm - iii - micro - gfp (miRNA-138开放阅读框[ORF])和mgm - iii - mir - off (miRNA-138短发夹)两个质粒载体,于妊娠第14天注入大鼠尾静脉。苏木精-伊红(HE)染色观察细胞形态。QRT-PCR、Western blot和免疫染色证实了miRNA-138与下游分子sonic hedgehog (Shh)之间的调控关系。结果:与对照组相比,过表达miRNA-138可显著促进神经元再生,减少神经元凋亡。miRNA-138的沉默显著增加了神经元凋亡和脊髓萎缩。此外,miRNA-138 ORF处理有效地增加了miRNA-138的表达水平,也上调了Shh的水平。相比之下,miRNA-138的敲低下调了骨髓增生异常区域的Shh水平。结论:miRNA-138过表达对胎鼠脊髓发育具有保护作用,其机制可能与Shh表达有关。本研究为促进胚胎脊髓发育的分子机制提供了一种新的策略。
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引用次数: 0
Pediatric Cerebral Meningioma: A Single-Center Study with 10 Children Not Associated with Neurofibromatosis Type 2 and Literature Review. 儿童脑膜瘤:10例与2型神经纤维瘤病无关的儿童的单中心研究和文献综述
IF 0.7 4区 医学 Q4 CLINICAL NEUROLOGY Pub Date : 2022-01-01 DOI: 10.1159/000526541
Isabella Opoku, Liu Yang, Peng Sun, Mading Zhou, Yutong Liu, Jian Ren, Jianxin Du, Ling Feng, Gao Zeng

Introduction: Pediatric meningiomas (PMs) are rare tumors; they differ from their adult counterparts by their atypicality of location, higher rates of malignant change, male preponderance, recurrence, and sometimes, their association with neurofibromatosis. This case series analyzes the clinical behavior, pathological presentation, location, and its association with neurofibromatosis type 2 (NF2).

Methods: This case series consists of pediatric patients between the ages of 4 and 16 years who were hospitalized in the neurosurgical department of our hospital from 2012 to 2021 with different neurological symptoms and a literature review using the PubMed/MEDLINE database.

Results: Sixty percent of the patients were males, while 40% were females. The most common neurological manifestations were signs of increased intracranial pressure. NF2 was absent in all patients. The predominant histopathology subtypes are atypical and WHO grade II, representing 30% and 40%, respectively.

Conclusion: This study supports the relationship between NF2 and pediatric cerebral meningioma but at a lower concomitant rate from 0 to 13%, taking into consideration our original data and the literature review, contrasting some reported cases, which suggest rates as high as 33%, 50%, and 100% in a very small number of patients. Gross total resection without postoperative radiation therapy for nonmalignant and non-NF2-associated PM proved to be a sufficient and a good treatment option.

儿童脑膜瘤是一种罕见的肿瘤;他们不同于他们的成人同行的非典型性的位置,更高的恶性变化率,男性优势,复发,有时,他们与神经纤维瘤病的关联。本病例系列分析临床行为、病理表现、部位及其与2型神经纤维瘤病(NF2)的关系。方法:本病例系列包括2012年至2021年在我院神经外科住院的4至16岁的不同神经症状的儿童患者,并使用PubMed/MEDLINE数据库进行文献综述。结果:男性占60%,女性占40%。最常见的神经学表现是颅内压升高。所有患者均无NF2。主要的组织病理学亚型是非典型和WHO II级,分别占30%和40%。结论:考虑到我们的原始数据和文献综述,本研究支持NF2与儿童脑膜瘤之间的关系,但伴随率从0到13%较低,与一些报道的病例相比,在极少数患者中,NF2的发生率高达33%,50%和100%。非恶性和非nf2相关的PM经证实是一种充分和良好的治疗选择。
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引用次数: 1
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Pediatric Neurosurgery
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