Central Nervous System Cysts

中枢神经系统囊肿
  • 文章类型: Journal Article
    Rathke的left囊肿(RCC)是良性的,位于大脑鞍区和鞍上区域的非肿瘤性病变,源自Rathke袋的残留物,垂体前叶的胚胎前体。尽管RCC经常无症状,并且在影像学研究中偶然发现,他们会出现各种症状,包括头痛,视觉障碍,以及由于相邻神经结构受压而导致的内分泌功能障碍。RCC的管理尤其具有挑战性,由于采取保守监测或手术干预的决定在很大程度上取决于囊肿的大小,增长潜力,以及症状的严重程度。经蝶窦手术是有症状的RCC的主要治疗方法,通过囊肿减压有效缓解症状。然而,复发仍然是一个重大问题,报告的比率高达33%,引发关于手术期间囊肿壁去除程度的争论。微创内窥镜技术的最新进展改善了手术效果,然而,术后并发症如垂体功能减退症和脑脊液漏的风险仍然存在。此外,对于复发性RCC患者或不适合重复手术的患者,立体定向放射外科已成为一种潜在的替代治疗方法.尽管承诺,RCC治疗中放疗的长期安全性和有效性需要进一步研究.这篇叙述性综述旨在全面概述RCC,整合最新的研究和临床指南来讨论病理生理学,临床表现,和管理策略,强调需要一种个性化的方法来治疗这种复杂的疾病。
    Rathke\'s cleft cysts (RCCs) are benign, non-neoplastic lesions located in the sellar and suprasellar regions of the brain, originating from remnants of Rathke\'s pouch, an embryonic precursor to the anterior pituitary gland. Although RCCs are frequently asymptomatic and discovered incidentally during imaging studies, they can present with a variety of symptoms, including headaches, visual disturbances, and endocrine dysfunction due to the compression of adjacent neural structures. The management of RCCs is particularly challenging, as the decision to pursue conservative monitoring or surgical intervention depends heavily on the cyst\'s size, growth potential, and the severity of symptoms. Transsphenoidal surgery is the primary treatment for symptomatic RCCs, offering effective relief from symptoms through decompression of the cyst. However, recurrence remains a significant issue, with rates reported up to 33%, prompting debates about the extent of cyst wall removal during surgery. Recent advancements in minimally invasive endoscopic techniques have improved surgical outcomes, yet the risk of postoperative complications such as hypopituitarism and cerebrospinal fluid leaks persists. Additionally, stereotactic radiosurgery has emerged as a potential alternative for patients with recurrent RCCs or those who are not suitable candidates for repeat surgery. Despite its promise, the long-term safety and efficacy of radiotherapy in RCC management require further investigation. This narrative review aims to provide a comprehensive overview of RCCs, integrating the latest research and clinical guidelines to discuss pathophysiology, clinical presentation, and management strategies, emphasizing the need for a personalized approach to treating this complex condition.
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  • 文章类型: Letter
    暂无摘要。
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  • 文章类型: Case Reports
    我们介绍了一例白质脑病伴钙化和囊肿(LCC)的患者,尽管对大型对侧囊肿进行了多次神经外科手术,但仍经历了进行性严重偏瘫。根据先前的病例报告,贝伐单抗被建议作为最终的治疗选择。在等待贝伐单抗报销批准的同时,临床和放射学疾病表现均有重大改善.LCC的病程可变且不可预测;对于严重和进行性神经功能缺损,应保留神经外科治疗。贝伐单抗已被报道为有希望的替代治疗选择。重要的是,在我们的病例中,观察到的临床改善归因于贝伐单抗的作用,如果在请求时启动。我们的案例强调了对LCC进行自然史研究的必要性,以及通过适当的临床试验进行系统评估而不是依靠已发表的病例报告中的轶事证据来验证治疗疗效的必要性。
    We present the case of a patient with leukoencephalopathy with calcifications and cysts (LCC), who experienced progressive severe hemiparesis despite multiple neurosurgical interventions of a large contralateral cyst. Bevacizumab was proposed as an ultimate treatment option based on prior case reports. While awaiting reimbursement approval for bevacizumab, major improvement occurred in both clinical and radiological disease manifestations. The disease course of LCC is variable and unpredictable; neurosurgical treatment should be reserved for severe and progressive neurological deficits. Bevacizumab has been reported as a promising alternative treatment option. Importantly, in our case the observed clinical improvement would have been attributed to the effects of bevacizumab, if started when requested. Our case underscores the need for a natural history study for LCC and the necessity of validating treatment efficacy by systematic evaluation through appropriate clinical trials rather than relying on anecdotal evidence from published case reports.
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  • 文章类型: Journal Article
    本文就Rathke裂隙囊肿(RCC)和鞍区脑膜瘤的外科治疗进展作一综述。基于三项关键研究的结果。RCC是良性的,来自垂体腺发育的充满液体的残留物,通常无症状且偶然发现。然而,当出现症状或增大时,需要进行手术干预.StefanLinsler等人的研究。和其他人检查各种手术方法,包括经颅锁孔和经蝶入路技术,内镜经鼻和眶上锁孔入路治疗鞍区脑膜瘤。结果表明,经颅锁孔和经蝶入路RCC手术的成功率都很高,超过5.7年没有复发。尽管锁孔入路的并发症较少。对于鞍区脑膜瘤,鼻内镜和眶上锁孔技术之间的选择应基于肿瘤的特点,强调外科医生熟练掌握两种方法的重要性。这些研究强调需要针对患者和肿瘤特征量身定制的个性化治疗策略,并强调了正在进行的手术技能开发和进一步研究以完善微创技术的重要性。这项研究强调了个性化手术方法在改善RCC和鞍区脑膜瘤患者预后方面的关键作用。
    This study reviews recent progress in the surgical treatment of Rathke\'s cleft cysts (RCCs) and Sellar region meningiomas, based on findings from three key studies. RCCs are benign, fluid-filled remnants from pituitary gland development that are usually asymptomatic and found by chance. However, surgical intervention is needed when they become symptomatic or increase in size. Research by Stefan Linsler et al. and others examines various surgical methods, including transcranial keyhole and transsphenoidal techniques for RCCs, and endoscopic endonasal and supraorbital keyhole approaches for Sellar meningiomas. The results show that both transcranial keyhole and transsphenoidal surgeries for RCCs have high success rates with no recurrences over 5.7 years, although the keyhole approach has fewer complications. For Sellar meningiomas, the choice between endoscopic endonasal and supraorbital keyhole techniques should be based on tumor characteristics, highlighting the importance of surgeon proficiency in both methods. These studies emphasize the need for personalized treatment strategies tailored to patient and tumor characteristics and highlight the importance of ongoing surgical skill development and further research to refine minimally invasive techniques. This study highlights the crucial role of personalized surgical approaches in improving outcomes for patients with RCCs and Sellar region meningiomas.
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  • 文章类型: Journal Article
    目的:有症状的Rathke裂囊肿(RCC)的切除主要通过经鼻蝶入路进行。然而,目前文献中缺乏关于经颅锁孔入路治疗RCC的等效数据.为了找到关于复发的RCC手术治疗的一般建议,本研究的目的是分析和比较这两种技术。
    方法:对2004年1月至2019年8月期间接受手术治疗的29例患者进行回顾性分析。选择经蝶入路16例,经颅锁孔入路13例。对两种手术技术进行术前症状和囊肿特征的分析和比较。并发症,外科激进性,内分泌和眼科的结果和复发患者的随访。
    结果:两种技术的术后结果相同,神经功能缺损的成功率达到了92%,内分泌功能障碍占82%,视力缺陷占86%。相比之下,经蝶窦手术后发生重大术后并发症的可能性显著增加。经过5.7年的平均随访时间,两组的复发率相同,均为0%.
    结论:关于其具有较低并发症发生率的同等结局,作者建议使用眶上锁孔入路对解剖结构允许两种技术的RCC进行治疗.然而,该决定应始终考虑外科医生的个人经验和其他个体患者特征。需要进行更多病例和更长随访时间的进一步研究,以分析所选方法对复发的影响。
    OBJECTIVE: Resections of symptomatic Rathke\'s cleft cysts (RCCs) are mainly performed via an endonasal transsphenoidal approach. However, there is a lack of equivalent data in current literature concerning transcranial keyhole approach in the treatment of RCCs. In order to find general recommendations for the surgical treatment of RCCs also with regard to recurrence, the object of this study is the analysis and comparison of both techniques.
    METHODS: Twenty-nine patients having been surgically treated between January 2004 and August 2019 were retrospectively analysed. The transsphenoidal approach was chosen in 16 cases and the transcranial keyhole approach in 13 cases. Both surgical techniques were analyzed and compared concerning preoperative symptoms and cyst characteristics, complications, surgical radicality, endocrinological and ophthalmological outcome and recurrences in patients´ follow up.
    RESULTS: The postoperative outcome of both techniques was identic and showed highly satisfying success rates with 92% for neurological deficits, 82% for endocrinological dysfunctions and 86% for visual deficits. In contrast, momentous postoperative complications were significantly more likely after transsphenoidal operations. After a mean follow-up time of 5.7 years, the recurrence rates of both cohorts were the same with 0% each.
    CONCLUSIONS: Regarding its equal outcome with its lower complication rate, the authors suggest using the supraorbital keyhole approach for RCCs whose anatomical configuration allow both techniques. Yet, the decision should always consider the surgeon\'s personal experience and other individual patient characteristics. Further studies with higher numbers of cases and longer follow-up periods are necessary to analyse the effect of the selected approach on recurrence.
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  • 文章类型: Journal Article
    背景:我们的目的是通过检测脑水肿来确定表观扩散系数是否能够预测有症状的松果体囊肿的存在。
    方法:我们回顾性分析45例松果体囊肿切除前后和51例无松果体囊肿患者的MRI,比较丘脑的ADC值,中央,脑室周围和皮质下白质。此外,我们评估了相应患者的囊肿大小和形态,并分析了其与ADC值的相关性。
    结果:有症状的松果体囊肿患者与对照组之间的差异不显著(p=0.200-0.968)。切除囊肿后,ADC比率没有显着变化(p=0.575-0.862)。囊肿大小与ADC比率没有显着相关性(p=0.071-0.918)。原始数据分析显示出更多的意义,尤其是脑室周围和中央白质,这导致两个亚组的ADC比率存在显著的半球间差异(p<0.001和p=0.031)。1.5T的MRI显示始终高于3T的值,但大多不明显。
    结论:我们的分析显示没有证据表明松果体囊肿导致静脉压迫引起的脑水肿。由于变异性高于所看到的差异,ADC序列似乎不是有症状的松果体囊肿的适当诊断工具。
    BACKGROUND: Our aim was to determine whether the Apparent Diffusion Coefficient is able to predict the presence of a symptomatic pineal cyst by detecting cerebral edema.
    METHODS: We retrospectively analyzed MRIs of 45 patients with pineal cysts before and after resection and 51 patients without pineal cysts, comparing ADC values of thalamus, central, periventricular and subcortical white matter. Furthermore we evaluated cyst size and morphology and analyzed its correlation to ADC values in corresponding patients.
    RESULTS: Differences between patients with symptomatic pineal cyst and control group were not significant (p = 0.200 - 0.968). ADC ratios did not change significantly after resection of the cyst (p = 0.575 - 0.862). Cyst size showed no significant correlation to ADC ratios (p = 0.071 - 0.918). Raw data analyses revealed more significance, especially periventricularly and in central white matter, which resulted in significant interhemispheric differences in ADC ratios in both subgroups (p < 0.001 and p = 0.031). MRI of 1.5T showed consistently higher values than 3T but mostly insignificant.
    CONCLUSIONS: Our analysis revealed no evidence that pineal cysts lead to intracerebral edema caused by venous compression. Since variability was higher than the differences seen, ADC sequences do not appear to be an appropriate diagnostic tool for symptomatic pineal cysts.
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  • 文章类型: Journal Article
    Rathke裂隙囊肿(RCC)是在鞍区或鞍上区发现的一种常见类型的病变。它们通常是临床监测的,但在某些情况下,可能需要手术。然而,他们的自然进程还没有得到很好的理解,和手术的结果是不确定的。这项研究的目的是评估Rathke’s裂隙囊肿的自然病史,在没有治疗的情况下进行临床监测的患者,并确定手术结果和随时间复发的发生率。
    国家多中心研究从2000年开始诊断为Rathke裂隙囊肿(RCC-Spain)的患者,随后在西班牙的15个三级中心进行。共有177例诊断为RCC的患者随访67.3个月(6-215),88例患者接受了手术,(81名患者在诊断后立即接受手术,7名患者随后生长)随访68.8个月(3-235)。
    在73.5%(133)的患者中,囊肿大小保持稳定或减小。只有44例患者(24.3%)经历了囊肿增加,其中9例(5.1%)经历了大于3毫米的增加。在大多数接受手术的患者中,头痛和视力改变得到了改善,中位时间为96个月后8例(9.1%)出现复发,没有发现复发的预测因子。
    没有初始压迫症状的Rathke\的left囊肿生长概率低,所以建议保守管理。接受经蝶入路手术的患者经历了快速的临床改善,和复发是罕见的。然而,它们可以在很长一段时间后发生,尽管尚未发现复发的预测因子。
    Rathke\'s cleft cysts (RCC) are a common type of lesion found in the sellar or suprasellar area. They are usually monitored clinically, but in some cases, surgery may be required. However, their natural progression is not yet well understood, and the outcomes of surgery are uncertain. The objective of this study is to evaluate the natural history of Rathke\'s cleft cysts in patients who are clinically monitored without treatment, and to determine the outcomes of surgery and the incidence of recurrences over time.
    UNASSIGNED: National multicentric study of patients diagnosed of Rathke\'s cleft cyst (RCC- Spain) from 2000 onwards and followed in 15 tertiary centers of Spain. A total of 177 patients diagnosed of RCC followed for 67.3 months (6-215) and 88 patients who underwent surgery, (81 patients underwent immediate surgery after diagnosis and 7 later for subsequent growth) followed for 68.8 months (3-235).
    UNASSIGNED: The cyst size remained stable or decreased in 73.5% (133) of the patients. Only 44 patients (24.3%) experienced a cyst increase and 9 of them (5.1%) experienced an increase greater than 3 mm. In most of the patients who underwent surgery headaches and visual alterations improved, recurrence was observed in 8 (9.1%) after a median time of 96 months, and no predictors of recurrence were discovered.
    UNASSIGNED: Rathke\'s cleft cysts without initial compressive symptoms have a low probability of growth, so conservative management is recommended. Patients who undergo transsphenoidal surgery experience rapid clinical improvement, and recurrences are infrequent. However, they can occur after a long period of time, although no predictors of recurrence have been identified.
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  • 文章类型: Journal Article
    松果体囊肿经常作为磁共振成像中的偶然发现,通常没有症状,然而,一些患者表现出可能与囊肿相关的症状表现,即使没有脑积水.这些症状的病因仍然存在争议。这项研究旨在调查淋巴内皮细胞(LEC)标志物的存在以及有症状的非脑积水患者的松果体囊肿中炎症或免疫反应的指征。该研究包括八名接受囊肿手术切除的患者。免疫组织化学用于评估LYVE-1,PDPN,和VEGFR3作为LEC标记,与IL-6和CD3一起用于炎症或免疫活性的适应症。我们的分析显示缺乏炎症标志物或免疫反应。然而,观察到VEGFR3的不同表达,可能位于松果体囊肿组织内的神经元。我们建议松果体囊肿内的这些VEGFR3神经元可能导致这些患者报告的头痛症状。需要进一步的调查来证实这一假设。
    Pineal cysts are frequently encountered as incidental findings in magnetic resonance imaging, usually devoid of symptoms, yet some patients exhibit symptomatic manifestations possibly associated with the cyst, even in the absence of hydrocephalus. The etiology of these symptoms remains contentious. This study aims to investigate the presence of lymphatic endothelial cell (LEC) markers and indications of inflammation or immune response within the pineal cysts of patients experiencing symptomatic non-hydrocephalic presentations. Eight patients who underwent surgical excision of their cysts were included in the study. Immunohistochemistry was utilized to assess the expression of LYVE-1, PDPN, and VEGFR3 as LEC markers, alongside IL-6 and CD3 for indications of inflammation or immune activity. Our analysis revealed an absence of inflammatory markers or immune response. However, a distinct expression of VEGFR3 was observed, likely localized to neurons within the pineal cyst tissue. We propose that these VEGFR3+ neurons within the pineal cyst may contribute to the headache symptoms reported by these patients. Further investigations are warranted to substantiate this hypothesis.
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  • 文章类型: Journal Article
    目的:本研究旨在阐明有症状的Rathke’sleft囊肿(RCC)的最佳鼻内镜手术策略。
    方法:我们回顾性分析了接受EEA手术的RCC患者。提出了手术和重建方法选择的策略。患者分为开窗或闭窗组。术前、术后症状,成像,眼科,和内分泌检查进行了审查。确定并发症的发生率和复发率。
    结果:75人都接受了初级手术。开窗封闭组32例,开窗组43例。中位随访期为39个月。三个主要投诉是头痛(n=51,68.00%),视力障碍(n=45,60.00%),和垂体功能障碍(n=16,21.33%)。在术前头痛的51名患者中,48例(94.12%)报告术后症状改善。45例患者中有23例(51.11%)视力障碍得到改善。16人中有14人(87.50%)垂体功能障碍得到改善。两组之间的症状缓解率没有明显差异。有3例患者(3/75,4.00%)出现囊肿再积聚。其中之一(1/75,1.33%),需要再次手术,使用翼状方法治愈。在并发症方面,2例(2/75,2.67%)发生脑部感染。他们都在抗生素治疗后恢复。术后无脑脊液鼻漏发生。开放组1例(1/75,1.33%)出现鼻出血。没有持续性垂体功能减退或尿崩症(DI)。头痛相关因素分析显示蜡样结节的存在与其相关。
    结论:在开窗尽可能开放的情况下,经鼻内镜手术成功治疗RCC几乎没有问题。术前识别T2WI低信号结节可能是手术指征的潜在参考因素。
    OBJECTIVE: The study intends to clarify the optimal endoscopic endonasal surgical strategy for symptomatic Rathke\'s cleft cysts (RCCs).
    METHODS: We retrospectively analyzed patients with RCCs that underwent EEA surgery. The strategy for surgical and reconstruction method selection was presented. Patients were split into groups of fenestration open or closed. Pre- and postoperative symptoms, imaging, ophthalmologic, and endocrinologic exams were reviewed. The incidence of complications and the recurrence rates were determined.
    RESULTS: The 75 individuals were all received primary operations. The fenestration closed group contained 32 cases, while the fenestration open group contained 43 cases. The median follow-up period was 39 months. The three primary complaints were headache (n = 51, 68.00%), vision impairment (n = 45, 60.00%), and pituitary dysfunction (n = 16, 21.33%). Of the 51 patients with preoperative headaches, 48 (94.12%) reported improvement in their symptoms following surgery. Twenty-three out of 45 patients (51.11%) experienced an improvement in visual impairment. Pituitary dysfunction was found improved in 14 out of 16 individuals (87.50%). There was no discernible difference in the rate of symptom alleviation between both groups. There were three patients (3/75, 4.00%) had cyst reaccumulation. One of them (1/75, 1.33%), which needed reoperation, was healed using pterional approach. In term of complications, cerebral infections occurred in two patients (2/75, 2.67%). Both of them recovered after antibiotic treatment. No postoperative cerebrospinal fluid rhinorrhea occurred. One patient (1/75, 1.33%) in the open group experienced epistaxis. There was no persistent hypopituitarism or diabetes insipidus (DI). Analysis of headache related factors showed that the presence of wax like nodules was related to it.
    CONCLUSIONS: RCC was successfully treated with endoscopic endonasal surgery with few problems when the fenestration was kept as open as feasible. Preoperative identification of T2WI hypointense nodules may be a potential reference factor for surgical indication.
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  • 文章类型: Journal Article
    背景:中窝蛛网膜囊肿(MFACs)很少见,先天性病变可能破裂并引起颅内压升高的症状。我们试图描述在MCAC中视神经水肿的存在和相关因素,重点关注眼科评估对指导囊肿治疗的实用性。
    方法:我们回顾了在我们机构进行眼科评估的所有MCF患者的临床和影像学信息。头痛,颅神经麻痹,呕吐,精神状态改变,疲劳,癫痫发作被认为是MFAC相关症状。单变量和多变量分析评估了与视神经水肿相关的因素。
    结果:纳入了2003年至2022年的51例患者。囊肿的中位体积为169.9cm3(四分位间距:70.5,647.7)。19例(37.3%)患者发生硬膜下血肿/水瘤破裂的证据。18例(35.3%)患者因囊肿和/或破裂相关的颅内出血而接受了手术。11例(21.6%)患者出现视神经水肿;所有患者均有症状并经历囊肿破裂。这些患者中有10人接受了手术。术后,80%的病例解决了视神经水肿。囊肿体积和症状与视神经水肿无关;然而,囊肿破裂患者,尤其是那些有创伤性破裂的人,更有可能发生视神经水肿和接受手术(P<0.001)。
    结论:我们发现21.6%的评估的MCO出现视神经水肿,占破裂病例的57.9%。在未破裂的囊肿中未发现视水肿。囊肿开窗术可改善视神经水肿和患者症状。结合临床病史和神经影像学,视神经水肿可能有助于指导MFAC治疗,尤其是囊肿破裂患者。
    BACKGROUND: Middle fossa arachnoid cysts (MFACs) are rare, congenital lesions that may rupture and cause symptoms of elevated intracranial pressure. We sought to describe the presence of and factors associated with optic nerve edema in MFACs, focusing on the utility of ophthalmologic evaluations for guiding cyst management.
    METHODS: We reviewed clinical and radiographic information for all patients with MFACs with ophthalmologic evaluations at our institution. Headache, cranial nerve palsy, emesis, altered mental status, fatigue, and seizures were considered MFAC-related symptoms. Univariate and multivariable analyses evaluated factors associated with optic edema.
    RESULTS: Fifty-one patients between 2003 and 2022 were included. Cysts were a median volume of 169.9 cm3 (interquartile range: 70.5, 647.7). Evidence of rupture with subdural hematoma/hygroma occurred in 19 (37.3%) patients. Eighteen (35.3%) patients underwent surgery for their cyst and/or rupture-associated intracranial bleed. Eleven (21.6%) patients had optic edema; all were symptomatic and experienced cyst rupture. Ten of these patients received surgery. Postoperatively, optic edema resolved in 80% of cases. Cyst volume and symptoms were not associated with optic edema; however, patients with ruptured cysts, particularly those with traumatic rupture, were more likely to have optic edema and receive surgery (P < 0.001).
    CONCLUSIONS: We found optic edema in 21.6% of evaluated MFACs, and this comprised of 57.9% of ruptured cases. Optic edema was not found in unruptured cysts. Cyst fenestration improved optic edema and patient symptoms. In conjunction with clinical history and neuroimaging, optic edema may help guide MFAC management, particularly in patients with cyst rupture.
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