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The paraneurium and the tumefactive appearance of peripheral nerve neurolymphomatosis: illustrative case 周围神经神经淋巴瘤病的副神经元和肿瘤性外观:例证
Pub Date : 2024-04-22 DOI: 10.3171/CASE24125
D. Barone, Ryan W Kendziora, S. Broski, David J. Schembri Wismayer, Robert J Spinner
BACKGROUND Peripheral neurolymphomatosis (NL) is an often-misdiagnosed condition characterized by lymphomatous infiltration within the peripheral nerves. Its rarity and complexity frequently result in delayed diagnosis and suboptimal patient outcomes. This study aims to elucidate the role of the paraneurium (circumneurium) in NL, emphasizing its diagnostic and therapeutic significance. OBSERVATIONS A 72-year-old man presented with lesions on his right lower eyelid. Initial diagnostics were inconclusive until an excisional biopsy confirmed extranodal marginal zone lymphoma. Following a complete metabolic response to rituximab treatment, the patient relapsed 14 months later with progressive lymphoma and bilateral sciatic nerve involvement, as confirmed by positron emission tomography–computed tomography and magnetic resonance imaging. LESSONS This paper underscores the critical role of the paraneurium in NL, enhancing understanding of its pathophysiology. Integrating advanced imaging techniques have proved essential in accurately identifying neurolymphomatous involvement within the paraneurium. This study paves the way for more effective management strategies in NL and similar conditions, focusing on improving patient care and outcomes.
背景外周神经淋巴瘤病(NL)是一种经常被误诊的疾病,其特征是外周神经内的淋巴瘤浸润。其罕见性和复杂性常常导致诊断延迟和患者预后不佳。本研究旨在阐明周围神经旁(circumneurium)在 NL 中的作用,强调其诊断和治疗意义。观察 一名 72 岁的男子因右下眼睑出现病变而就诊。最初的诊断结果并不明确,直到切除活检证实了结节外边缘区淋巴瘤。经正电子发射计算机断层扫描和磁共振成像证实,患者在接受利妥昔单抗治疗后出现完全代谢反应,14 个月后复发,淋巴瘤进展并累及双侧坐骨神经。启示 本文强调了副神经在 NL 中的关键作用,加深了对其病理生理学的理解。事实证明,整合先进的成像技术对于准确识别神经淋巴管旁的神经淋巴瘤受累情况至关重要。这项研究为更有效地管理 NL 和类似病症的策略铺平了道路,重点在于改善患者护理和治疗效果。
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引用次数: 0
Incidental durotomy resulting in a postoperative lumbosacral nerve root with eventration into the adjacent facet joint: illustrative cases 腰骶神经根术后偶发性硬膜外切除术导致邻近面关节连通:病例说明
Pub Date : 2024-04-22 DOI: 10.3171/CASE2418
MD Michael J. Kelly, MD Franziska C. S. Altorfer, MD Marco D. Burkhard, MD Russel C. Huang, MD Frank P. Cammisa Jr., MD J. Levi Chazen
BACKGROUND Radicular pain after lumbar decompression surgery can result from epidural hematoma/seroma, recurrent disc herniation, incomplete decompression, or other rare complications. A less recognized complication is postoperative nerve root herniation, resulting from an initially unrecognized intraoperative or, more commonly, a spontaneous postoperative durotomy. Rarely, this nerve root herniation can become entrapped within local structures, including the facet joint. The aim of this study was to illustrate our experience with three cases of lumbosacral nerve root eventration into an adjacent facet joint and to describe our diagnostic and surgical approach to this rare complication. OBSERVATIONS Three patients who had undergone lumbar decompression surgery with or without fusion experienced postoperative radiculopathy. Exploratory revision surgery revealed all three had a durotomy with nerve root eventration into the facet joint. Significant symptom improvement was achieved in all patients following liberation of the neural elements from the facet joints. LESSONS Entrapment of herniated nerve roots into the facet joint may be a previously underappreciated complication and remains quite challenging to diagnose even with the highest-quality advanced imaging. Thus, clinicians must have a high index of suspicion to diagnose this issue and a low threshold for surgical exploration.
背景腰椎减压术后的放射性疼痛可能是由硬膜外血肿/血肿、复发性椎间盘突出症、减压不完全或其他罕见并发症引起的。术后神经根疝是一种较少见的并发症,是由最初未被发现的术中神经根疝或更常见的术后自发性神经根疝造成的。在极少数情况下,这种神经根疝会缠绕在包括面关节在内的局部结构中。本研究旨在说明我们对三例腰骶部神经根连入邻近面关节的经验,并描述我们对这种罕见并发症的诊断和手术方法。观察结果 三名接受过腰椎减压手术并行或不行椎间融合术的患者术后出现了根性病变。探查性翻修手术发现,这三位患者都有神经根连入面关节的穹隆切开术。在将神经元从面关节中解放出来后,所有患者的症状都得到了明显改善。启示 将疝出的神经根插入面关节可能是一种以前未被充分重视的并发症,即使使用最高质量的先进成像技术,诊断这种并发症仍具有相当大的挑战性。因此,临床医生在诊断时必须高度怀疑,并降低手术探查的门槛。
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引用次数: 0
Arachnoid bands in hemifacial spasm: an overlooked etiology? Illustrative case 半面痉挛中的蛛网膜带:被忽视的病因?示例病例
Pub Date : 2024-04-15 DOI: 10.3171/CASE24164
Mariam Al-Mutawa, H. Schroeder
BACKGROUND Primary hemifacial spasm (HFS) is usually caused by arterial compression of the facial nerve at the root exit zone at the brainstem. Rarely, a purely venous compression is seen. However, arachnoid bands strangulating the facial nerve have not been recognized as a cause of hemifacial spasm. OBSERVATIONS The authors present a case of a 24-year-old female who had experienced HFS for 9 years. Endoscopic inspection of the root exit zone revealed no vascular compression but an arachnoid band strangulating the facial nerve. After cutting the band, the lateral spread response disappeared, and the patient was immediately spasm free after the surgery. Two years later, she was still doing well without any spasms. LESSONS This is the third patient in the authors’ series of 535 patients who had no vascular conflict but rather a strangulation of the nerve by arachnoid bands. All patients have remained spasm free. To the authors’ knowledge, no other group has reported arachnoid bands as an etiological factor of HFS so far.
背景原发性半面痉挛(HFS)通常是由于脑干根部出口区域的面神经受到动脉压迫所致。纯静脉压迫的情况很少见。然而,蛛网膜带勒住面神经尚未被认为是半面痉挛的病因。观察 作者介绍了一例 24 岁女性的病例,她经历了 9 年的半面肌痉挛。内窥镜检查发现,面神经根出口区没有血管压迫,但有一条蛛网膜束带勒住了面神经。切断蛛网膜带后,侧向扩散反应消失,术后患者立即摆脱了痉挛。两年后,她的情况依然良好,没有出现任何痉挛。启示 这是作者 535 例系列手术中的第三例患者,他们都没有血管冲突,而是被蛛网膜束带勒住了神经。所有患者均未发生痉挛。据作者所知,迄今为止还没有其他研究小组报告过蛛网膜带是导致 HFS 的病因之一。
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引用次数: 0
Evaluation of the shrinkage process of a neck remnant after stent-coil treatment of a cerebral aneurysm using silent magnetic resonance angiography and computational fluid dynamics analysis: illustrative case 利用无声磁共振血管造影术和计算流体动力学分析评估支架盘旋治疗脑动脉瘤后颈部残余物的收缩过程:示例病例
Pub Date : 2024-04-15 DOI: 10.3171/CASE24141
T. Satoh, K. Sugiu, M. Hiramatsu, J. Haruma, I. Date
BACKGROUND Silent magnetic resonance angiography (MRA) mitigates metal artifacts, facilitating clear visualization of neck remnants after stent and coil embolization of cerebral aneurysms. This study aims to scrutinize hemodynamics at the neck remnant by employing silent MRA and computational fluid dynamics. OBSERVATIONS The authors longitudinally tracked images of a partially thrombosed anterior communicating artery aneurysm’s neck remnant, which had been treated with stent-assisted coil embolization, using silent MRA over a decade. Computational fluid dynamics delineated the neck remnant’s reduction process, evaluating hemodynamic parameters such as flow rate, wall shear stress magnitude and vector, and streamlines. The neck remnant exhibited diminishing surface area, volume, neck size, dome depth, and aspect ratio. Its reduction correlated with a decline in the flow rate ratio of the remnant dome to the inflow parent artery. Analysis delineated regions within the contracting neck remnant characterized by consistently low average wall shear stress magnitude and variation, accompanied by notable variations in wall shear stress vector directionality. LESSONS Evaluation of neck remnants after stent-coil embolization is possible through silent MRA and computational fluid dynamics. Predicting the neck remnant reduction may be achievable through hemodynamic parameter analysis.
背景无声磁共振血管造影(MRA)可减轻金属伪影,有助于清晰观察脑动脉瘤支架和线圈栓塞术后的残余颈部。本研究旨在通过无声 MRA 和计算流体动力学仔细观察残余颈部的血流动力学。观察 作者使用无声 MRA 纵向追踪了一个部分血栓形成的前交通动脉动脉瘤颈部残余的图像,该动脉瘤曾在十年内接受过支架辅助线圈栓塞治疗。计算流体动力学描述了残余动脉瘤颈的缩小过程,评估了血流动力学参数,如流速、壁剪应力大小和矢量以及流线。残颈的表面积、体积、颈部大小、穹顶深度和长宽比都在不断缩小。其减少与残余穹隆与流入母动脉的流速比下降有关。分析划定了收缩残余颈部的区域,其特点是平均壁面剪应力大小和变化持续较低,同时壁面剪应力矢量方向性也有明显变化。启示 通过无声 MRA 和计算流体动力学,可以对支架盘栓塞后的残余颈部进行评估。通过血液动力学参数分析可以预测残余颈部的减少。
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引用次数: 0
Novel use of a closed-tip stent retriever to prevent distal embolism in the posterior circulation: illustrative case 使用闭合尖端支架回取器预防后循环远端栓塞的新方法:示例病例
Pub Date : 2024-04-15 DOI: 10.3171/CASE24137
Rikuo Nishii, Masanori Goto, Yuki Takano, Kota Nakajima, Takateru Takamatsu, Masanori Tokuda, Hikari Tomita, Mai Yoshimoto, Satohiro Kawade, Yasuhiro Yamamoto, Yuji Naramoto, Kunimasa Teranishi, Nobuyuki Fukui, T. Sunohara, R. Fukumitsu, Junichi Takeda, Masaomi Koyanagi, C. Sakai, Nobuyuki Sakai, T. Ohta
BACKGROUND In mechanical thrombectomy for tandem occlusions in vertebrobasilar stroke, distal emboli from the vertebral artery lesion should be prevented. However, no suitable embolic protection devices are currently available in the posterior circulation. Here, the authors describe the case of a vertebral artery lesion effectively treated with a closed-tip stent retriever as an embolic protection device in the posterior circulation. OBSERVATIONS A 65-year-old male underwent mechanical thrombectomy for basilar artery occlusion, with tandem occlusion of the proximal vertebral artery. After basilar artery recanalization via the nonoccluded vertebral artery, a subsequent mechanical thrombectomy was performed for the occluded proximal vertebral artery. To prevent distal embolization of the basilar artery, an EmboTrap III stent retriever was deployed as an embolic protection device within the basilar artery to successfully capture the thrombus. LESSONS A stent retriever with a closed-tip structure can effectively capture thrombi, making it a suitable distal embolic protection device in the posterior circulation.
背景 在对椎基底动脉卒中的串联闭塞进行机械取栓术时,应防止椎动脉病变造成远端栓塞。然而,目前还没有合适的后循环栓塞保护装置。在此,作者描述了一例在后循环中使用闭合顶端支架回取器作为栓塞保护装置有效治疗椎动脉病变的病例。观察 一名 65 岁的男性因基底动脉闭塞接受了机械性血栓切除术,同时伴有椎动脉近端串联闭塞。在通过未闭塞的椎动脉对基底动脉进行再通后,又对闭塞的近端椎动脉进行了机械取栓术。为防止基底动脉远端栓塞,在基底动脉内部署了 EmboTrap III 支架回取器作为栓塞保护装置,以成功捕获血栓。启示 具有闭合顶端结构的支架回取器可以有效捕获血栓,因此适合作为后循环的远端栓塞保护装置。
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引用次数: 0
A rare case of atypical intradural extramedullary glioblastoma diagnosed utilizing next-generation sequencing and methylation profiling: illustrative case 利用新一代测序和甲基化分析诊断出的一例罕见非典型硬膜外胶质母细胞瘤:说明性病例
Pub Date : 2024-04-15 DOI: 10.3171/CASE24103
MD William J. Shelton, BS Andrew P. Mathews, MD Karrar Aljiboori, MD J. Stephen Nix, MD Murat Gokden, MD Analiz Rodriguez
BACKGROUND Primary spinal cord tumors, especially primary spinal cord glioblastoma multiforme (PSC-GBM), are exceptionally rare, accounting for less than 1.5% of all spinal tumors. Their infrequency and aggressive yet atypical presentation make diagnosis challenging. In uncertain cases, a surgical approach for tissue diagnosis is often optimal. OBSERVATIONS A 76-year-old male presented with a rapidly progressing clinical history marked by worsening extremity weakness, urinary retention, and periodic fecal incontinence alongside diffuse changes on neuraxis imaging. The patient, in whom subacute polyneuropathy was initially diagnosed, received multiple rounds of steroids and intravenous immunoglobulin without clinical improvement. Histopathological review of the biopsy tissue yielded an initial diagnosis of spindle cell neoplasm. Next-generation sequencing (NGS) is done routinely on all neuropathology specimens at the authors’ institution, and methylation profiling is pursued in difficult cases. Ultimately, NGS and methylation profiling results were essential to an integrated final diagnosis of GBM. LESSONS PSC-GBM is a rare but highly aggressive occurrence of this tumor. Prolonged back pain, rapid neurological decline, and imaging changes warrant the consideration of lesional biopsy for precise disease characterization. In inconclusive cases, NGS has proved invaluable for clinical clarification and diagnosis, underscoring its importance for integrated diagnoses in guiding appropriate treatment strategies.
背景原发性脊髓肿瘤,尤其是原发性脊髓多形性胶质母细胞瘤(PSC-GBM)异常罕见,占所有脊髓肿瘤的比例不到 1.5%。脊髓胶质母细胞瘤的罕见性和侵袭性以及不典型的表现使诊断具有挑战性。在不确定的病例中,采用手术方法进行组织诊断通常是最佳选择。观察 一名 76 岁的男性患者,临床病史进展迅速,四肢无力、尿潴留和周期性大便失禁等症状不断加重,神经轴影像学表现为弥漫性改变。患者最初被诊断为亚急性多发性神经病,接受了多轮类固醇和静脉注射免疫球蛋白治疗,但临床症状未见好转。对活检组织进行组织病理学检查后,初步诊断为纺锤形细胞肿瘤。作者所在机构对所有神经病理标本进行了常规的下一代测序(NGS),并对疑难病例进行了甲基化分析。最终,NGS 和甲基化分析结果对 GBM 的综合最终诊断至关重要。教训 PSC-GBM 是一种罕见但侵袭性极强的肿瘤。长期背痛、神经功能迅速衰退以及影像学改变都需要考虑进行病灶活检,以准确确定疾病特征。在尚无定论的病例中,NGS 已被证明对临床澄清和诊断非常有价值,凸显了其在指导适当治疗策略的综合诊断中的重要性。
{"title":"A rare case of atypical intradural extramedullary glioblastoma diagnosed utilizing next-generation sequencing and methylation profiling: illustrative case","authors":"MD William J. Shelton, BS Andrew P. Mathews, MD Karrar Aljiboori, MD J. Stephen Nix, MD Murat Gokden, MD Analiz Rodriguez","doi":"10.3171/CASE24103","DOIUrl":"https://doi.org/10.3171/CASE24103","url":null,"abstract":"BACKGROUND Primary spinal cord tumors, especially primary spinal cord glioblastoma multiforme (PSC-GBM), are exceptionally rare, accounting for less than 1.5% of all spinal tumors. Their infrequency and aggressive yet atypical presentation make diagnosis challenging. In uncertain cases, a surgical approach for tissue diagnosis is often optimal. OBSERVATIONS A 76-year-old male presented with a rapidly progressing clinical history marked by worsening extremity weakness, urinary retention, and periodic fecal incontinence alongside diffuse changes on neuraxis imaging. The patient, in whom subacute polyneuropathy was initially diagnosed, received multiple rounds of steroids and intravenous immunoglobulin without clinical improvement. Histopathological review of the biopsy tissue yielded an initial diagnosis of spindle cell neoplasm. Next-generation sequencing (NGS) is done routinely on all neuropathology specimens at the authors’ institution, and methylation profiling is pursued in difficult cases. Ultimately, NGS and methylation profiling results were essential to an integrated final diagnosis of GBM. LESSONS PSC-GBM is a rare but highly aggressive occurrence of this tumor. Prolonged back pain, rapid neurological decline, and imaging changes warrant the consideration of lesional biopsy for precise disease characterization. In inconclusive cases, NGS has proved invaluable for clinical clarification and diagnosis, underscoring its importance for integrated diagnoses in guiding appropriate treatment strategies.","PeriodicalId":16554,"journal":{"name":"Journal of Neurosurgery: Case Lessons","volume":"34 12","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-04-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140702257","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Thoracic spinal cord injury after surgical removal of a ruptured cerebellar arteriovenous malformation in a patient in the Concorde position: illustrative case 协和体位患者小脑动静脉畸形破裂手术切除后的胸椎脊髓损伤:示例病例
Pub Date : 2024-04-15 DOI: 10.3171/CASE249
Kohei Ishikawa, Hideki Endo, Yasufumi Ohtake, Toshiichi Watanabe, Hirohiko Nakamura
BACKGROUND Thoracic spinal cord injury after posterior cranial fossa surgery in younger patients is a rare complication. There have been reports of this complication in tumor and spine fields but not in vascular surgery. OBSERVATIONS A 22-year-old-man experienced cerebellar arteriovenous malformation rupture, and the malformation was surgically removed with the man in the Concorde position. After surgery, the man had severe paraplegia, and a thoracic spinal cord injury was diagnosed. LESSONS In younger patients, cervical hyperflexion in the Concorde position can cause thoracic spinal cord injury even in surgery for cerebrovascular disease.
背景年轻患者在后颅窝手术后出现胸椎脊髓损伤是一种罕见的并发症。在肿瘤和脊柱领域曾有过这种并发症的报道,但在血管外科领域却没有。观察 一名 22 岁的男子发生小脑动静脉畸形破裂,在协和体位下手术切除了畸形。术后,该男子严重截瘫,被诊断为胸椎脊髓损伤。启示 对于年轻患者,即使在脑血管疾病手术中,协和体位下的颈椎过度屈曲也可能导致胸椎脊髓损伤。
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引用次数: 0
Metastatic intradural primary spinal osteosarcoma: illustrative case 转移性硬膜内原发性脊柱骨肉瘤:示例病例
Pub Date : 2024-04-15 DOI: 10.3171/CASE2480
Shreyas Thiruvengadam, Mark Lam, Stephen Honeybul
BACKGROUND Osteosarcomas are a common primary bone neoplasm among adolescents but represent 0.2% of all malignancies with an incidence of two to four cases per million persons annually worldwide. Although known to have significant metastatic potential, its rare incidence, treatment resistance, and poor prognosis have rendered it a poorly understood and infrequently documented pathology. OBSERVATIONS Herein the authors present the first documented case of lumbosacral intradural metastasis of a primary osteosarcoma in a young patient, possibly via intradural dissemination following pinhole durotomy in a prior thoracic surgery. LESSONS Osteosarcomas remain a difficult pathology to treat, particularly upon metastatic dissemination. The utility of adjuvant radiotherapy after resection of an osteosarcoma is increasingly evident in the reduction of local recurrence. In the context of intraoperative pinhole durotomies in resections of high-grade lesions, due consideration should be given to whole-spine radiation, although this remains an evidence-free zone.
背景骨肉瘤是青少年中常见的原发性骨肿瘤,但占所有恶性肿瘤的 0.2%,全球每年每百万人中有 2 到 4 例发病。虽然众所周知骨肉瘤有很大的转移潜力,但其罕见的发病率、耐药性和不良的预后使其成为一种鲜为人知且鲜有记录的病理现象。观察 作者在此介绍了第一例记录在案的年轻患者腰骶部原发性骨肉瘤硬膜内转移病例,该病例可能是在之前的胸腔手术中进行针孔穹隆切开术后发生硬膜内播散。启示 骨肉瘤仍然是一种难以治疗的病变,尤其是在转移扩散后。骨肉瘤切除术后辅助放疗在减少局部复发方面的作用越来越明显。在切除高级别病灶的术中针孔穿刺术中,应适当考虑全脊柱放射治疗,尽管这仍是一个无证据区。
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引用次数: 0
Primary intracranial peripheral primitive neuroectodermal tumor: lessons from an exceptionally rare neoplasm. Illustrative case 原发性颅内外周原始神经外胚层肿瘤:从一种异常罕见的肿瘤中汲取的教训。示例病例
Pub Date : 2024-04-15 DOI: 10.3171/CASE24133
Jhon E. Bocanegra-Becerra, Luis Felipe Novoa-Ramírez, Alan Jesús Latorre-Zúñiga, Norka Tacas-Gil, Rolando V Rojas-Apaza
BACKGROUND The primary intracranial peripheral primitive neuroectodermal tumor (pPNET) is a lesion subtype within the Ewing sarcoma family of tumors. pPNETs are extremely uncommon pathologies, accounting for 0.03% of intracranial tumors and 1% to 2% of Ewing sarcoma cases. Given its histological aspect similar to other highly proliferative malignant neuroectodermal neoplasms, pPNET merits extensive workup for accurate diagnosis and treatment. OBSERVATIONS A 36-year-old male presented to the emergency department with a 1-year history of headaches in the right frontoparietal area, generalized tonic-clonic seizures, and a history of the resection of a tumor labeled as a meningioma 5 years before admission. He was neurologically intact. Brain magnetic resonance imaging revealed a heterogeneous focal lesion of 25 × 35 × 23 mm with a necrotic center and neoformative appearance in the right frontal cortex. The patient underwent multimodal treatment with gross-total resection, radiotherapy, and chemotherapy. Histopathological examination results supported the diagnosis of pPNET. At the 2-year follow-up, the patient had no new-onset symptoms, and brain imaging revealed absent signs of tumor recurrence. LESSONS The present case describes an extraordinary pPNET case, initially confounded as a clear cell meningioma. Managing pPNET requires thorough investigation, careful differentiation from similar neuroectodermal lesions, and multimodal treatment to improve the patient’s prognosis.
背景原发性颅内外周原始神经外胚层肿瘤(pPNET)是尤文肉瘤家族肿瘤中的一种病变亚型。pPNET 是一种极为罕见的病变,占颅内肿瘤的 0.03%,占尤文肉瘤病例的 1%至 2%。鉴于其组织学方面与其他高度增生性恶性神经外胚层肿瘤相似,pPNET 值得广泛检查以进行准确诊断和治疗。观察 一名 36 岁的男性患者因右侧额顶区头痛 1 年、全身强直阵挛性发作和入院前 5 年曾切除脑膜瘤肿瘤而到急诊科就诊。他的神经系统完好无损。脑磁共振成像显示,右侧额叶皮层有一个 25 × 35 × 23 毫米的异质性病灶,中心坏死,呈新形。患者接受了大体全切除、放疗和化疗等多模式治疗。组织病理学检查结果支持 pPNET 的诊断。随访两年,患者没有出现新的症状,脑部影像学检查也没有发现肿瘤复发的迹象。启示 本病例描述了一个特殊的 pPNET 病例,起初被误认为是透明细胞脑膜瘤。处理 pPNET 需要进行彻底检查、仔细与类似的神经外胚层病变进行鉴别,并采取多模式治疗以改善患者的预后。
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引用次数: 0
Epidural lipomatosis with foci of hemorrhage and acute compression of the spinal cord in a child with CLOVES syndrome: illustrative case 硬膜外脂肪瘤病伴有出血灶和脊髓急性压迫,患儿患有 CLOVES 综合征:示例病例
Pub Date : 2024-04-15 DOI: 10.3171/CASE23772
Dmytro Ishchenko, Iryna Benzar, Andrii Holoborodko
BACKGROUND Congenital lipomatous overgrowth, vascular malformations, epidermal nevi, spinal/skeletal anomalies, and/or scoliosis (CLOVES) syndrome is the most recently described combined vascular anomaly characterized by congenital excessive growth of adipose tissue, vascular malformations, epidermal nevi, and skeletal deformities. This condition exhibits a significant variability in clinical manifestations and a tendency for rapid progression and affects extensive anatomical regions. Information regarding the association of epidural lipomatosis with low-flow venous lymphatic malformations is rare, with few reports in the literature. OBSERVATIONS The authors present a case of a 6-year-old girl who was admitted to the emergency department complaining of rapidly progressing weakness in her lower extremities and partial loss of sensation in the inguinal area. Radiologically, an extradural mass was identified at the T2–6 level, causing acute spinal cord compression. Urgent decompression and partial resection of the mass were performed. Despite satisfactory intraoperative hemo- and lymphostasis, postoperative lymphorrhea/seroma leakage was encountered as a delayed complication and was managed conservatively. LESSONS CLOVES syndrome is characterized by the combination of various clinical symptoms, not all of which are included in the abbreviation, as well as a progressively deteriorating course, the emergence of new symptoms, and complications throughout the patient’s life. This necessitates ongoing monitoring of such patients.
背景 先天性脂肪瘤过度生长、血管畸形、表皮痣、脊柱/骨骼畸形和/或脊柱侧弯(CLOVES)综合征(CLOVES Syndrome)是最近描述的一种合并血管畸形,其特征是先天性脂肪组织过度生长、血管畸形、表皮痣和骨骼畸形。这种疾病的临床表现差异很大,病情发展迅速,影响的解剖区域也很广。硬膜外脂肪瘤病与低流量静脉淋巴畸形相关的信息很少见,文献中也鲜有报道。观察 作者介绍了一例 6 岁女孩的病例,她因下肢无力和腹股沟区部分感觉丧失而急诊入院。放射学检查发现,T2-6水平有硬膜外肿块,导致急性脊髓压迫。医生紧急对肿块进行了减压和部分切除。尽管术中血液和淋巴止血效果令人满意,但术后淋巴液/血清瘤渗漏仍是一个迟发性并发症,因此采取了保守治疗。教训 克罗维斯综合征的特点是合并各种临床症状,但并非所有症状都包含在缩写中,而且病程会逐渐恶化,出现新的症状,并在患者的整个生命过程中出现并发症。因此,有必要对这类患者进行持续监测。
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引用次数: 0
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Journal of Neurosurgery: Case Lessons
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