Infratentorial neoplasms

幕下肿瘤
  • 文章类型: Case Reports
    我们报告了一名63岁的男子,患有中线后颅窝肿瘤和特殊的影像学特征,我们不确定术前病变的性质。组织病理学显示它是颅咽管瘤。它似乎来自下延髓膜,以前文献中没有描述的网站。讨论了文献中提到的前四个案例以及对这个不常见地点起源的推测。
    We report the case of a 63-year-old man with a midline posterior fossa tumor and peculiar imaging features where we were unsure of the nature of the lesion preoperatively. Histopathology revealed it to be a craniopharyngioma. It appeared to arise from the inferior medullary velum, a site not described before in the literature. The previous four cases mentioned in literature and speculations on the origins in this uncommon site are discussed.
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  • 文章类型: Journal Article
    目的:吞咽困难常见于后颅窝肿瘤(PFT)切除的个体,对个体的生活质量产生负面影响,营养状况,和整体健康。我们旨在定量综合PFT切除术后吞咽困难患病率研究的数据。
    方法:PubMed,WebofScience,Cochrane图书馆,Embase,中国国家知识基础设施(CNKI),万方数据库,我们在VIP数据库中搜索了评估PFT术后吞咽困难患病率的病例对照和横断面研究.进行荟萃分析以确定吞咽困难的患病率。进行亚组和荟萃回归分析以确定研究中异质性的来源。
    结果:共纳入22项研究,涉及20921例。随机效应模型的荟萃分析显示,PFT切除术后吞咽困难的合并全球患病率为21.7%(95%置信区间:16.9-26.6)。亚组和荟萃回归分析表明,参与者年龄(P<0.001),评估方法(P=0.004),和研究参与者的地理区域(P=0.001)是研究中异质性的来源.
    结论:PFT切除后吞咽困难的患病率很高。应通过筛查及早发现吞咽困难高风险的PFTs个体。需要对吞咽困难进行多学科诊断和治疗,以改善PFT切除术后早期的预后。
    OBJECTIVE: Dysphagia is common in individuals who have undergone posterior fossa tumor (PFT) resection and negatively impacts on the individual\'s quality of life, nutritional status, and overall health. We aimed to quantitatively synthesize data from studies of the prevalence of dysphagia following PFT resection.
    METHODS: PubMed, Web of Science, the Cochrane Library, Embase, China National Knowledge Infrastructure (CNKI), Wanfang database, and VIP database were searched for case-control and cross-sectional studies that evaluated the prevalence of dysphagia after PFT surgery. Meta-analyses were performed to determine the prevalence of dysphagia. Subgroup and meta-regression analyses were performed to determine the sources of heterogeneity among the studies.
    RESULTS: A total of 22 studies were included, involving 20,921 cases. A meta-analysis of the random-effects model showed that the pooled global prevalence of dysphagia following PFT resection was 21.7% (95% confidence interval: 16.9-26.6). The subgroup and meta-regression analyses demonstrated that participant age (P < 0.001), assessment methods (P = 0.004), and geographical region of the study participants (P = 0.001) were sources of heterogeneity among the studies.
    CONCLUSIONS: Dysphagia has a high prevalence following PFT resection. Individuals with PFTs who are at a high risk for dysphagia should be identified early through screening. Multidisciplinary diagnosis and treatment of dysphagia are required to improve the outcomes in the early stages after PFT resection.
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  • 文章类型: Journal Article
    目的:儿童后颅窝肿瘤(PFT)幸存者经历长期认知后遗症,包括记忆障碍,其中辐照是主要的危险因素之一。本研究的目的是(1)探索情节损害的概况,语义,辐照和未辐照PFT幸存者的工作和程序记忆系统,(2)测试自传问卷和评估情景记忆的两阶段生态测试(Epireal)是否比常用测试对辐射引起的海马损伤更敏感。
    方法:共有60名参与者(22名接受照射的PFT幸存者,17名未经辐照的PFT幸存者,和21个对照)被纳入前瞻性IMPALA研究。他们都接受了一系列广泛的测试,在相隔3周的两个为期2天的会议中评估不同的记忆系统。我们进行了组间比较,并分析了损伤情况,使用-1.65SDs作为截止值。对于受照射的患者,计算了与记忆相关的关键大脑结构的平均辐射剂量之间的相关性(海马,小脑,和纹状体)和相应的记忆得分。
    结果:PBT幸存者在常规发作性测试中的表现明显差于对照组(p<0.001),语义记忆和工作记忆:64%的受照射患者和35%的未受照射患者在至少两个记忆系统中存在缺陷,情景记忆障碍对接受照射的人群更有特异性。Epireal的效果比其他情景记忆测试更大,使我们能够在另外18%的受照射患者中检测到缺陷。这些缺陷与左侧海马的平均辐射剂量相关。
    结论:记忆障碍是PFT幸存者常见的长期认知后遗症,尤其是放疗后.新的情景记忆生态测试比常规测试对辐射引起的缺陷更敏感,可以产生颞叶内侧辐射毒性的特定标记。
    OBJECTIVE: Pediatric posterior fossa tumor (PFT) survivors experience long-term cognitive sequelae, including memory disorders, for which irradiation is one of the main risk factors. The aims of the present study were to (1) explore the profile of impairment in episodic, semantic, working and procedural memory systems in irradiated versus nonirradiated PFT survivors, and (2) test whether an autobiographical questionnaire and a two-phase ecological test (Epireal) assessing episodic memory are more sensitive to radiation-induced hippocampal damage than commonly used tests.
    METHODS: A total of 60 participants (22 irradiated PFT survivors, 17 nonirradiated PFT survivors, and 21 controls) were included in the prospective IMPALA study. They all underwent a broad battery of tests assessing the different memory systems in two 2-day sessions 3 weeks apart. We performed between-groups comparisons and analyzed impairment profiles, using -1.65 SDs as a cut-off. For irradiated patients, correlations were calculated between mean radiation doses to key brain structures involved in memory (hippocampus, cerebellum, and striatum) and corresponding memory scores.
    RESULTS: PBT survivors performed significantly more poorly than controls (p < 0.001) on conventional tests of episodic, semantic and working memory: 64% of irradiated patients and 35% of nonirradiated patients had a deficit in at least two memory systems, with episodic memory impairment being more specific to the irradiated group. Epireal had a larger effect size than the other episodic memory tests, allowing us to detect deficits in a further 18% of irradiated patients. These deficits were correlated with the mean radiation dose to the left hippocampus.
    CONCLUSIONS: Memory impairment is a frequent long-term cognitive sequela in PFT survivors, especially after radiation therapy. New ecological tests of episodic memory that are more sensitive to radiation-induced deficits than conventional tests could yield specific markers of the toxicity of medial temporal lobe irradiation.
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  • 文章类型: Journal Article
    目的:我们的目的是报告流行病学,手术结果,在大型单中心病例系列中,后颅窝肿瘤患儿的生存率。
    方法:对2011年1月至2019年1月接受后颅窝肿瘤手术治疗的儿童患者进行回顾性分析。
    结果:共有135名儿科患者,诊断时平均年龄为7.5岁,平均随访时间为35.7个月,包括在研究中。大多数肿瘤位于中线内,在71.4%的患者中观察到脑室增宽。毛细胞星形细胞瘤涵盖了大多数肿瘤(34.1%),其次是髓母细胞瘤(27.4%)和室管膜瘤(11.8%)。71.8%的患者实现了总切除(GTR),复发率为20%。25.9%的患者出现手术并发症。GTR显著影响后颅窝肿瘤患者的5年总生存期(OS)和4年无进展生存期(PFS)。接受GTR的患者5年OS为89.7%,与接近全切除的72.7%和次全切除的70.8%相比。接受GTR的患者的4年PFS为82.5%,而接受近全切除术的患者为63.6%,接受次全切除术的患者为54.2%.
    结论:手术切除仍是小儿后颅窝肿瘤的主要治疗方法,更高的切除率与更好的生存结果相关。尽管分子诊断资源有限,我们的机构已经证明,对于这些患者,具有高手术量的专门神经肿瘤中心仍然可以取得良好的生存结局.
    OBJECTIVE: We aim to report the epidemiology, surgical outcomes, and survival rates of pediatric patients with posterior fossa tumors in a large single-center case series.
    METHODS: A retrospective analysis was conducted on pediatric patients who underwent surgical treatment for posterior fossa tumors between January 2011 and January 2019.
    RESULTS: A total of 135 pediatric patients, with an average age of 7.5 years at diagnosis and a mean follow-up of 35.7 months, were included in the study. Most tumors were located within the midline, with ventriculomegaly observed in 71.4% of the patients. Pilocytic astrocytomas encompassed the majority of tumors (34.1%), followed by medulloblastomas (27.4%) and ependymomas (11.8%). Gross total resection (GTR) was achieved in 71.8% of the patients, with a recurrence rate of 20%. Surgical complications were observed in 25.9% of the patients. GTR significantly impacted 5-year overall survival (OS) and 4-year progression-free survival (PFS) in patients with posterior fossa tumors. Patients who underwent GTR had a 5-year OS of 89.7%, compared to 72.7% for near-total resection and 70.8% for subtotal resection. The 4-year PFS for patients who underwent GTR was 82.5%, whereas it was 63.6% for patients who underwent near-total resection and 54.2% for patients who underwent subtotal resection.
    CONCLUSIONS: Surgical resection remains the main treatment for pediatric posterior fossa tumors, and higher resection rates are linked to better survival outcomes. Despite limited resources for molecular diagnosis, our institution has demonstrated that a specialized neurooncological center with a high surgical volume can still achieve favorable survival outcomes for these patients.
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  • 文章类型: Journal Article
    后颅窝A组(PFA)室管膜瘤是在婴儿和幼儿中诊断出的致命脑癌。PFA线性基因组中缺乏驱动事件,导致我们在其3D基因组中搜索特征。这里,我们从不同的儿童肿瘤类型中重建了3D基因组,并发现了PFA中的全局拓扑结构,这让人联想到各种人体组织中的干细胞和祖细胞.PFA中唯一存在的一个显着特征是PFA中的B型超远距离相互作用(TULIPs),沿着线性基因组间隔很远的区域,这些区域在3D核空间中以惊人的强度相互作用。TULIP发生在所有PFA样品中,并在可预测的基因组坐标处复发,它们的形成是由EZHIP的表达诱导的。TULIP在PFA样品中的普遍性表明可以在治疗上利用的分子原理的保守性。
    Posterior fossa group A (PFA) ependymoma is a lethal brain cancer diagnosed in infants and young children. The lack of driver events in the PFA linear genome led us to search its 3D genome for characteristic features. Here, we reconstructed 3D genomes from diverse childhood tumor types and uncovered a global topology in PFA that is highly reminiscent of stem and progenitor cells in a variety of human tissues. A remarkable feature exclusively present in PFA are type B ultra long-range interactions in PFAs (TULIPs), regions separated by great distances along the linear genome that interact with each other in the 3D nuclear space with surprising strength. TULIPs occur in all PFA samples and recur at predictable genomic coordinates, and their formation is induced by expression of EZHIP. The universality of TULIPs across PFA samples suggests a conservation of molecular principles that could be exploited therapeutically.
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  • 文章类型: Journal Article
    目的:由于目前尚无小儿后颅窝(PF)肿瘤的笔形束扫描(PBS)质子治疗(PT)的指南,这项研究调查了欧洲PT中心的规划技术,特别考虑脑干和脊髓的保留。
    方法:在19个治疗儿科患者的欧洲PBS-PT中心进行了一项调查和治疗计划比较。调查评估了治疗链的所有方面,包括但不限于划界,剂量限制和治疗计划。每个中心都计划了两个PF肿瘤病例进行局灶性照射,根据他们自己的临床实践,但基于共同的描述。病例1的处方剂量为54Gy(RBE),病例2的处方剂量为59.4Gy(RBE)。对于这两种情况,比较了计划策略和相关剂量指标。
    结果:17个(89%)中心回答了调查,16人(80%)参与治疗计划比较.在调查中,13个(68%)中心报告使用欧洲粒子治疗网络定义进行脑干勾画.在治疗计划研究中,虽然大多数中心使用三个光束方向,它们的配置在各个中心之间差异很大。脑干剂量也有很大的变化,脑干接近最大剂量(D2%),范围为52.7Gy(RBE)至55.7Gy(RBE)(病例1),从56.8Gy(RBE)到60.9Gy(RBE)(案例2)。
    结论:本研究评估了儿科PF肿瘤的欧洲PBS-PT计划。在例如划界实践中达成了协议,而在计划方法和因此对有风险器官的剂量方面观察到更广泛的差异。各中心之间的合作仍在进行中,争取共同的指导方针。
    As no guidelines for pencil beam scanning (PBS) proton therapy (PT) of paediatric posterior fossa (PF) tumours exist to date, this study investigated planning techniques across European PT centres, with special considerations for brainstem and spinal cord sparing.
    A survey and a treatment planning comparison were initiated across nineteen European PBS-PT centres treating paediatric patients. The survey assessed all aspects of the treatment chain, including but not limited to delineations, dose constraints and treatment planning. Each centre planned two PF tumour cases for focal irradiation, according to their own clinical practice but based on common delineations. The prescription dose was 54 Gy(RBE) for Case 1 and 59.4 Gy(RBE) for Case 2. For both cases, planning strategies and relevant dose metrics were compared.
    Seventeen (89 %) centres answered the survey, and sixteen (80 %) participated in the treatment planning comparison. In the survey, thirteen (68 %) centres reported using the European Particle Therapy Network definition for brainstem delineation. In the treatment planning study, while most centres used three beam directions, their configurations varied widely across centres. Large variations were also seen in brainstem doses, with a brainstem near maximum dose (D2%) ranging from 52.7 Gy(RBE) to 55.7 Gy(RBE) (Case 1), and from 56.8 Gy(RBE) to 60.9 Gy(RBE) (Case 2).
    This study assessed the European PBS-PT planning of paediatric PF tumours. Agreement was achieved in e.g. delineation-practice, while wider variations were observed in planning approach and consequently dose to organs at risk. Collaboration between centres is still ongoing, striving towards common guidelines.
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  • 文章类型: Case Reports
    中枢神经源性过度通气(CNH)是一种罕见的疾病,由呼吸中枢的化学或机械紊乱引起的。其特征在于没有脑外呼吸刺激。尽管缺乏心肺或代谢改变,但一名妇女在切除后颅窝脑膜瘤后出现了严重的呼吸性碱中毒和乳酸血症。脑计算机断层扫描(cCT)显示髓质区水肿。甘露醇和地塞米松治疗重建了正常的呼吸模式。乳酸血症可能是由于内脏乳酸利用率降低。在没有明显原因的情况下,应怀疑颅内病变。建议cCT确认水肿或缺血并及时治疗。
    Central neurogenic hyperventilation (CNH) is a rare disease, caused by chemical or mechanical disturbance of respiratory centers. It is characterized by the absence of extracerebral respiratory stimuli. A woman developed severe respiratory alkalosis and lactatemia after resection of a posterior fossa meningioma despite lack of cardio-respiratory or metabolic alterations. Cerebral computed tomography (cCT) revealed edema of the pontomedullary area. Treatment with mannitol and dexamethasone reestablished normal breathing patterns. Lactatemia was likely due to reduced splanchnic lactate utilization. Intracranial pathologies should be suspected in case of hyperventilation without overt reasons. cCT to confirm edema or ischemia and prompt treatment is suggested.
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  • 文章类型: Journal Article
    目的:本研究旨在检测小儿幕下肿瘤患者的幕上皮质和皮质下形态学变化。
    方法:本研究纳入了24名年龄在4-18岁之间、被诊断为原发性幕下肿瘤的患者和41名年龄和性别相匹配的健康对照者。使用应用于T2轴图像的深度学习算法生成脑磁共振成像的合成磁化准备的快速梯度回波图像。皮质厚度,表面积,volume,和局部回旋指数(LGI),以及皮质下灰质体积,自动计算。使用一般线性模型比较患者和对照组的基于表面的形态测量参数,使用t检验和Mann-WhitneyU检验比较皮质下结构之间的体积。
    结果:在患者组中,皮质变薄是在左半脑膜上观察到的,在左尾中额叶(CMF)观察到皮质增厚,左梭形,左外侧眶额,左舌回,右CMF,右后扣带回,右上额叶(P<0.050)。患者组显示三角部体积减少,paracentral,前中心,和左半球的翼上回(P<0.05)。双侧上额叶和扣带回的表面积减少(P<0.05)。患者组在右侧中央前回和颞上回表现为LGI降低,左半侧,和后扣带回回,显示双侧尾状核和海马体积增加,虽然在双侧壳核观察到体积减少,苍白球,杏仁核(P<0.05)。在双侧CMF中,心室体积和肿瘤体积与皮质厚度呈正相关,而与LGI降低的区域呈负相关(P<0.05)。
    结论:后颅窝肿瘤导致皮质结构广泛的形态学改变,最突出的模式是伪善。
    结论:本研究阐明了儿童幕下肿瘤对神经系统的影响。为未来旨在减轻这些不利的皮质和皮质下变化并改善患者预后的治疗策略奠定基础。
    This study aimed to detect supratentorial cortical and subcortical morphological changes in pediatric patients with infratentorial tumors.
    The study included 24 patients aged 4-18 years who were diagnosed with primary infratentorial tumors and 41 age- and gender-matched healthy controls. Synthetic magnetization-prepared rapid gradient echo images of brain magnetic resonance imaging were generated using deep learning algorithms applied to T2-axial images. The cortical thickness, surface area, volume, and local gyrification index (LGI), as well as subcortical gray matter volumes, were automatically calculated. Surface-based morphometry parameters for the patient and control groups were compared using the general linear model, and volumes between subcortical structures were compared using the t-test and Mann-Whitney U test.
    In the patient group, cortical thinning was observed in the left supramarginal, and cortical thickening was observed in the left caudal middle frontal (CMF), left fusiform, left lateral orbitofrontal, left lingual gyrus, right CMF, right posterior cingulate, and right superior frontal (P < 0.050). The patient group showed a volume reduction in the pars triangularis, paracentral, precentral, and supramarginal gyri of the left hemisphere (P < 0.05). A decreased surface area was observed in the bilateral superior frontal and cingulate gyri (P < 0.05). The patient group exhibited a decreased LGI in the right precentral and superior temporal gyri, left supramarginal, and posterior cingulate gyri and showed an increased volume in the bilateral caudate nucleus and hippocampus, while a volume reduction was observed in the bilateral putamen, pallidum, and amygdala (P < 0.05). The ventricular volume and tumor volume showed a positive correlation with the cortical thickness in the bilateral CMF while demonstrating a negative correlation with areas exhibiting a decreased LGI (P < 0.05).
    Posterior fossa tumors lead to widespread morphological changes in cortical structures, with the most prominent pattern being hypogyria.
    This study illuminates the neurological impacts of infratentorial tumors in children, providing a foundation for future therapeutic strategies aimed at mitigating these adverse cortical and subcortical changes and improving patient outcomes.
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  • 文章类型: Case Reports
    儿童后颅窝肿瘤切除后,持续性脑积水很常见。然而,硬膜下水瘤的发生非常罕见。我们报告了一个14个月大的孩子在马斯喀特的儿科神经科诊所就诊的病例,阿曼在2021年发展了紧张的硬膜下水瘤,并伴有稳定的脑积水,在术后早期,后颅窝肿瘤切除术后。我们描述了独特的临床,与紧张的硬膜下水瘤发展相关的放射学和病理学特征。我们还讨论了脑脊液分流的管理,其中包括脑室-腹膜或腹膜下分流术。这种独特的状况与外部脑积水的区别在于对管理策略至关重要的特征。
    Persistent hydrocephalus is common in children after resection of posterior fossa tumours. However, occurrence of subdural hygroma is very rare. We report the case of a 14-month-old child who presented at a paediatric neurology clinic in Muscat, Oman in 2021 who developed a tense subdural hygroma with stable hydrocephalus, in the early postoperative period, following posterior fossa tumour resection. We describe the distinctive clinical, radiological and pathological features associated with the development of a tense subdural hygroma. We also discuss the management by cerebrospinal fluid diversion, which includes either a ventriculoperitoneal or subduroperitoneal shunt. This unique condition is distinguished from external hydrocephalus by features that are critical to the management strategy.
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  • 文章类型: Case Reports
    血管母细胞瘤是良性血管肿瘤,世界卫生组织一级,最常见的位置在小脑。由于出血过多,完整的显微外科手术切除可能是一个挑战,这就是术前栓塞的重要性。
    介绍了两个临床病例,一个25岁的女人和一个75岁的男人,由于梗阻性脑积水而出现颅内高压症状的患者;两种情况下都进行了脑室-腹腔分流术;此外,他们出现了小脑症状。都用乙烯-乙烯醇共聚物栓塞,血流量减少。之后,他们在栓塞后的第一周内接受了显微外科手术切除,获取,在这两种情况下,全切无血流动力学并发症,具有临床改善和良好的手术效果。值得一提的是,手术管理是允许合适手术方法的黄金标准,就像我们的病人一样,进行了枕下侧颅切开术。
    实性血管母细胞瘤的发生率低于囊性血管母细胞瘤。治疗是手术切除,这是一个挑战,在手术计划中总是被认为是动静脉畸形,包括术前栓塞,以降低围手术期的发病率和死亡率,并获得良好的疗效。
    UNASSIGNED: Hemangioblastomas are benign vascular neoplasms, World Health Organization grade I, with the most frequent location in the cerebellum. Complete microsurgical resection can be a challenge due to excessive bleeding, which is why preoperative embolization takes importance.
    UNASSIGNED: Two clinical cases are presented, a 25-year-old woman and a 75-year-old man, who presented with intracranial hypertension symptoms due to obstructive hydrocephalus; a ventriculoperitoneal shunt was placed in both cases; in addition, they presented with cerebellar signs. Both underwent embolization with ethylene vinyl alcohol copolymer, with blood flow reduction. After that, they underwent microsurgical resection within the 1st-week post embolization, obtaining, in both cases, gross total resection without hemodynamic complications, with clinical improvement and good surgical outcome. It is worth mentioning that surgical management is the gold standard that allows a suitable surgical approach, like in our patients, for which a lateral suboccipital craniotomy was performed.
    UNASSIGNED: Solid hemangioblastomas are less frequent than their cystic counterparts. The treatment is the surgical resection, which is a challenge and always has to be considered as an arteriovenous malformation in the surgical planning, including preoperative embolization to reduce perioperative morbidity and mortality and get good outcomes.
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