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An invasive and diffuse cranial actinomycosis with a dura-based mass mimicking a brain tumor: illustrative case. 侵袭性弥漫性颅骨放线菌病,硬脑膜肿块模仿脑肿瘤:示例病例。
Pub Date : 2024-09-23 DOI: 10.3171/CASE24210
Dejen T Gebrewahd, Tsegazeab Laeke, Eyob Z Wendimagegnehu, Mestet Y Shiferaw, Tesfaye G Tefera, Ilili A Aliye, Taye J Robele, Bereket H Mekuria, Anteneh E Berga, Surafael M Mendere, Sebboona B Abelti

Background: Actinomycosis is a chronic suppurative infection caused by non-spore-forming, anaerobic, and filamentous gram-positive bacteria. Primary central nervous system involvement is rare, with no specific clinical features, causing a clinical diagnostic dilemma. Imaging can help in localizing and characterizing the lesion; however, a definitive diagnosis relies on culture and/or histopathology.

Observations: The authors describe a 29-year-old male farmer with a rare case of invasive and diffuse cranial actinomycosis with a dura-based mass mimicking a brain tumor. Brain magnetic resonance imaging showed a moderately enhanced right frontoparietal infiltrative dura-based mass with marked thickening of the skull and multiple scalp actinomycotic abscesses. He underwent microsurgical excision of the mass, orbital decompression, and debridement of the scalp abscess. Histopathology confirmed actinomycosis, and his postoperative course was uneventful.

Lessons: Invasive and diffuse cranial actinomycosis with a dura-based actinomycetoma is a rare presentation that poses a diagnostic challenge due to its nonspecific manifestations. Imaging is helpful in localizing and characterizing the lesion; however, histopathology remains the gold standard for diagnosing actinomycosis. A high index of suspicion is also warranted in patients with predisposing factors to promote an early diagnosis and the initiation of appropriate treatments to improve functional recovery and limit residual deficits. https://thejns.org/doi/10.3171/CASE24210.

背景:放线菌病是一种慢性化脓性感染,由非芽孢杆菌、厌氧菌和丝状革兰氏阳性菌引起。原发性中枢神经系统受累非常罕见,没有特殊的临床特征,造成临床诊断上的难题。影像学检查有助于确定病灶的位置和特征,但明确诊断还需依靠培养和/或组织病理学检查:作者描述了一名 29 岁男性农民的罕见病例,他患有侵袭性和弥漫性颅骨放线菌病,硬脑膜肿块模仿脑肿瘤。脑磁共振成像显示,右侧额顶浸润性硬脑膜肿块中度增强,颅骨明显增厚,头皮多处放线菌脓肿。他接受了显微手术切除肿块、眼眶减压和头皮脓肿清创。组织病理证实为放线菌病,术后恢复顺利:启示:伴有硬脑膜放线菌瘤的侵袭性和弥漫性颅骨放线菌病是一种罕见的病症,由于其无特异性表现,给诊断带来了挑战。影像学检查有助于确定病变的位置和特征;然而,组织病理学仍是诊断放线菌病的金标准。https://thejns.org/doi/10.3171/CASE24210。
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引用次数: 0
Intrathecal placement of percutaneous spinal cord stimulation leads: illustrative cases. 经皮脊髓刺激导线的鞘内置入:示例。
Pub Date : 2024-09-23 DOI: 10.3171/CASE24275
Zachary T Olmsted, Peter B Wu, Alireza Katouzian, Michael J Dorsi

Background: Spinal cord stimulation (SCS), including the percutaneous placement of epidural stimulation leads, has been increasingly utilized to treat chronic pain. Although lead migration is a well-characterized complication, few studies have reported on malpositioned leads in the intrathecal space. Here, the authors discuss two cases of intrathecal lead placement necessitating surgical revision.

Observations: This report is a two-case series on the inadvertent placement of percutaneous SCS leads in the intrathecal space. The authors describe the identification of malpositioned leads, describe the clinical presentation, characterize stimulation parameters, and report improvement following neurosurgical revision for each case. Two patients originally presenting with chronic low-back pain underwent percutaneous SCS lead implantation. Both patients presented with atypical pain symptoms in the acute to subacute postprocedural period, raising suspicion for malpositioned leads. Imaging was consistent with intrathecal malpositioning. Both patients underwent revision surgery resulting in symptomatic improvement.

Lessons: Indicators of malpositioned thoracic SCS leads in the intrathecal space include thoracoabdominal or flank pain exacerbated by movement, insufficient pain relief versus that in the SCS trial, very low electrode impedances, direct visualization on imaging, and lack of epidural lead visualization following laminectomy. Revision options include removal of the intrathecal leads and the surgical placement of a paddle electrode in the epidural space. https://thejns.org/doi/10.3171/CASE24275.

背景:脊髓刺激(SCS),包括经皮放置硬膜外刺激导线,越来越多地被用于治疗慢性疼痛。虽然导联移位是一种特征明确的并发症,但有关鞘内腔导联定位错误的研究报道却很少。在此,作者讨论了两例鞘内导联放置不当而需要手术修正的病例:本报告是两例经皮 SCS 导联无意中置入鞘内腔的系列病例。作者描述了如何识别定位错误的导线,描述了临床表现,描述了刺激参数的特征,并报告了每个病例在神经外科手术修正后的改善情况。两名最初表现为慢性腰背痛的患者接受了经皮 SCS 导联植入术。这两名患者在术后急性期至亚急性期均出现了非典型疼痛症状,这引起了对导联位置不当的怀疑。影像学检查结果与鞘内错位一致。两名患者均接受了翻修手术,症状得到改善:启示:鞘内间隙胸椎 SCS 导联定位不良的指标包括:胸腹部或侧腹疼痛因运动而加剧、疼痛缓解程度低于 SCS 试验中的缓解程度、电极阻抗极低、成像直接可见、椎板切除术后硬膜外导联不可见。翻修方案包括移除鞘内导联,并通过手术在硬膜外腔放置桨状电极。https://thejns.org/doi/10.3171/CASE24275。
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引用次数: 0
A novel technique for skull base reconstruction in craniofacial fibrous dysplasia surgery using three-dimensional printing and a dental alginate mold: illustrative case. 利用三维打印和牙科藻酸盐模具在颅面纤维发育不良手术中重建颅底的新技术:示例。
Pub Date : 2024-09-23 DOI: 10.3171/CASE24262
Kirby C Manigos, Joseph Erroll V Navarro, Kenny S Seng, Jose Carlos S Alcazaren, Oliver Ryan M Malilay, Tito Guillermo D Rejante, Kevin Paul Ferraris

Background: Fibrous dysplasia is a rare and benign skeletal lesion characterized by fibrous tissue proliferation due to an abnormal osteogenesis replacing normal bone.

Observations: An 18-year-old male with fibrous dysplasia of the left sphenoid, ethmoid, orbit, and frontal bones was managed with excision and skull base reconstruction. After complete removal of the tumor, skull base reconstruction was commenced by making a reverse temporalis flap and placing it over the opened paranasal sinuses for a robust vascularized graft, followed by an abdominal fat graft, and then a pedicled pericranal flap was added to complete the multilayer onlay graft. To recreate the skull base, a mirror image of the contralateral skull base was constructed using three-dimensional (3D) printing, and the 3D-printed model was sterilized prior to the surgery. Intraoperatively, the model was then pressed onto dental alginate gel to make a negative mold. This was used to make the definitive flap using polymethylmethacrylate. Temporoplasty was also performed using polymethylmethacrylate to fill the defect left by the temporalis graft. The patient recovered well following the procedure.

Lessons: Appropriate, personalized skull base reconstruction techniques can be successfully done with 3D printing using alternative low-cost materials and implements, especially following resection of cases like craniofacial fibrous dysplasia. https://thejns.org/doi/10.3171/CASE24262.

背景:纤维性发育不良是一种罕见的良性骨骼病变,其特点是由于异常成骨作用导致纤维组织增生,取代正常骨骼:一名 18 岁男性的左侧蝶骨、乙状骨、眼眶和额骨纤维性发育不良患者接受了切除术和颅底重建术。完全切除肿瘤后,开始进行颅底重建,首先制作反向颞肌皮瓣,并将其置于打开的副鼻窦上,以形成稳固的血管移植物,然后进行腹部脂肪移植物,最后再加上有蒂的颅周皮瓣,以完成多层嵌合移植物。为了再造颅底,使用三维(3D)打印技术构建了对侧颅底的镜像,并在手术前对3D打印模型进行了消毒。术中,模型被压在牙科藻酸盐凝胶上,制成阴模。然后使用聚甲基丙烯酸甲酯制作最终皮瓣。此外,还使用聚甲基丙烯酸甲酯进行了颞成形术,以填补颞肌移植后留下的缺损。术后患者恢复良好:https://thejns.org/doi/10.3171/CASE24262。
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引用次数: 0
A rare case of central nervous system pancreatoblastoma metastasis: illustrative case. 一例罕见的中枢神经系统胰母细胞瘤转移病例:示例。
Pub Date : 2024-09-16 DOI: 10.3171/CASE23764
Vincenzo Mastropasqua, Marco Obersnel, Vito Stifano, Marco Gessi, Domenico Cristiano Corsi, Francesco Doglietto

Background: Pancreatoblastoma is a malignant neoplasm of the pancreas, occurring usually in children and rarely in adults. Treatment consists of surgery with a variable combination of adjuvant therapies. Liver metastases are common, whereas brain diffusion is exceptionally rare.

Observations: The authors report the case of a 42-year-old man with a 16-year history of metastatic pancreatoblastoma, previously treated with surgery, chemotherapy, and radiotherapy, demonstrating a partial response. He presented with headache and dizziness, and brain magnetic resonance imaging (MRI) showed a cerebellar lesion. A craniotomy was performed with complete tumor removal, and the postoperative course was uneventful. Brain MRI showed gross-total resection of the lesion, and the patient was discharged with an improvement of the preoperative symptoms. Histopathological analysis confirmed the diagnosis of metastasis from pancreatoblastoma. The patient received adjuvant stereotactic radiotherapy and showed further clinical improvement at the last follow-up.

Lessons: Brain metastases from pancreatoblastoma are exceptionally rare and poorly described in the literature. There is no standard therapy for this condition; hence, patients usually undergo treatments similar to those for other central nervous system metastases. All the described patients have had good clinical outcomes yet short-term follow-ups; therefore, further investigations are needed to better understand the best treatments for this condition. https://thejns.org/doi/10.3171/CASE23764.

背景:胰母细胞瘤是一种胰腺恶性肿瘤:胰母细胞瘤是一种胰腺恶性肿瘤,通常发生在儿童身上,成人很少见。治疗方法包括手术和多种辅助疗法。肝转移很常见,而脑扩散则异常罕见:作者报告了一例 42 岁男性患者的病例,该患者有 16 年的转移性胰腺母细胞瘤病史,曾接受过手术、化疗和放疗,并取得了部分疗效。他出现头痛和头晕,脑磁共振成像(MRI)显示小脑病变。他接受了开颅手术,肿瘤被完全切除,术后恢复顺利。脑部核磁共振成像显示病灶已被完全切除,患者术前症状有所改善后出院。组织病理分析确诊为胰母细胞瘤转移。患者接受了辅助立体定向放射治疗,最后一次随访时临床症状进一步改善:启示:胰母细胞瘤脑转移异常罕见,文献中的描述也很少。目前还没有针对这种情况的标准疗法;因此,患者通常会接受与其他中枢神经系统转移瘤类似的治疗。https://thejns.org/doi/10.3171/CASE23764。
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引用次数: 0
Covered stent deployment for a recurrent cervical internal carotid artery aneurysm referencing angioscopy: illustrative case. 参考血管造影,为复发性颈内动脉瘤部署覆盖支架:示例病例。
Pub Date : 2024-09-16 DOI: 10.3171/CASE24383
Hiromichi Hayami, Kenji Fukutome, Shuta Aketa, Junji Fukumori, Takaaki Mitsui, Yuki Shiraishi, Ryuta Matsuoka, Naoki Mori, Rinsei Tei, Yasushi Shin, Yasushi Motoyama

Background: Extracranial carotid artery aneurysms (ECAAs) are rare, and treatment guidelines are lacking. Few reports on endovascular treatments performed for ECAAs exist.

Observations: A 73-year-old woman with a left giant cervical internal carotid artery aneurysm was treated with overlapping closed-cell stents. The aneurysm regrew 1 year after the treatment, and then a covered stent was deployed. Angioscopy was performed to confirm neointimal development to determine the appropriate stent position before the retreatment, and it revealed that the stent struts were embedded in thick neointima for the most part but that the neointima was thin around the aneurysm neck. Multiple holes connecting to the aneurysm were observed between the stent struts. A covered stent overlapped inside the closed-cell stents, and blood flow into the aneurysm completely disappeared.

Lessons: When deploying the covered stent for recurrent aneurysms, angioscopy is useful for confirming neointimal development and determining the appropriate stent length and position. Angioscopic observations suggest that using stents with a higher mesh density and smaller pore size can reduce the neck hole size of the aneurysm and may achieve complete occlusion of the aneurysm. https://thejns.org/doi/10.3171/CASE24383.

背景:颅外颈动脉动脉瘤(ECAA)非常罕见,而且缺乏治疗指南。关于 ECAA 的血管内治疗的报道也很少:一位 73 岁的女性患有左侧颈内动脉巨大动脉瘤,她接受了重叠式闭孔支架治疗。治疗 1 年后,动脉瘤重新生长,随后又植入了一个覆盖支架。血管造影检查确认了新内膜的发育情况,以确定再治疗前支架的适当位置,结果显示支架支柱大部分嵌入厚厚的新内膜中,但动脉瘤颈周围的新内膜较薄。在支架支柱之间观察到多个与动脉瘤相连的孔。覆盖支架重叠在闭孔支架内,流入动脉瘤的血流完全消失:启示:在使用覆盖支架治疗复发性动脉瘤时,血管造影有助于确认新血管的发育情况,并确定适当的支架长度和位置。血管造影观察结果表明,使用网眼密度较高、孔径较小的支架可以缩小动脉瘤颈部的孔径,并可能实现动脉瘤的完全闭塞。https://thejns.org/doi/10.3171/CASE24383。
{"title":"Covered stent deployment for a recurrent cervical internal carotid artery aneurysm referencing angioscopy: illustrative case.","authors":"Hiromichi Hayami, Kenji Fukutome, Shuta Aketa, Junji Fukumori, Takaaki Mitsui, Yuki Shiraishi, Ryuta Matsuoka, Naoki Mori, Rinsei Tei, Yasushi Shin, Yasushi Motoyama","doi":"10.3171/CASE24383","DOIUrl":"10.3171/CASE24383","url":null,"abstract":"<p><strong>Background: </strong>Extracranial carotid artery aneurysms (ECAAs) are rare, and treatment guidelines are lacking. Few reports on endovascular treatments performed for ECAAs exist.</p><p><strong>Observations: </strong>A 73-year-old woman with a left giant cervical internal carotid artery aneurysm was treated with overlapping closed-cell stents. The aneurysm regrew 1 year after the treatment, and then a covered stent was deployed. Angioscopy was performed to confirm neointimal development to determine the appropriate stent position before the retreatment, and it revealed that the stent struts were embedded in thick neointima for the most part but that the neointima was thin around the aneurysm neck. Multiple holes connecting to the aneurysm were observed between the stent struts. A covered stent overlapped inside the closed-cell stents, and blood flow into the aneurysm completely disappeared.</p><p><strong>Lessons: </strong>When deploying the covered stent for recurrent aneurysms, angioscopy is useful for confirming neointimal development and determining the appropriate stent length and position. Angioscopic observations suggest that using stents with a higher mesh density and smaller pore size can reduce the neck hole size of the aneurysm and may achieve complete occlusion of the aneurysm. https://thejns.org/doi/10.3171/CASE24383.</p>","PeriodicalId":94098,"journal":{"name":"Journal of neurosurgery. Case lessons","volume":"8 12","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-09-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11418031/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142304904","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Letter to the Editor Response. 致编辑的信的回复。
Pub Date : 2024-09-16 DOI: 10.3171/CASE24305
Daniel D Cummins, Hyun Joo Park, Jenna Tosto-Mancuso, David Putrino, Fedor E Panov, Christopher P Kellner, Roshini Kalagara, Margaret H Downes
{"title":"Letter to the Editor Response.","authors":"Daniel D Cummins, Hyun Joo Park, Jenna Tosto-Mancuso, David Putrino, Fedor E Panov, Christopher P Kellner, Roshini Kalagara, Margaret H Downes","doi":"10.3171/CASE24305","DOIUrl":"10.3171/CASE24305","url":null,"abstract":"","PeriodicalId":94098,"journal":{"name":"Journal of neurosurgery. Case lessons","volume":"8 12","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-09-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11418029/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142304905","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Ventral epidural stimulation for motor recovery after spinal cord injury: illustrative case. 用于脊髓损伤后运动恢复的腹侧硬膜外刺激:示例。
Pub Date : 2024-09-16 DOI: 10.3171/CASE24155
Abdullah K Afridi, Alexander G Steele, Catherine Martin, Dimitry G Sayenko, Sean M Barber

Background: Spinal cord stimulation (SCS) has demonstrated potential as a therapy to enhance motor functional recovery after spinal cord injury (SCI). Epidural SCS for motor recovery is traditionally performed via the dorsal electrode. While ventral epidural stimulation may provide more direct and specific stimulation of the ventral motor neurons involved in motor control, it is largely unstudied, and its role in motor recovery after SCI is unclear. In order to profile the safety and feasibility of ventral epidural spinal stimulation (VSS), the authors present a patient who underwent VSS following a corpectomy to treat SCI related to metastatic epidural cord compression.

Observations: A patient underwent transpedicular corpectomy for spinal cord decompression, as well as the placement of 2 ventral epidural electrodes, followed by concurrent physical therapy and ventral epidural stimulation. He was nonambulatory preoperatively but was able to walk over 300 feet with the assistance of a rolling walker at the conclusion of the 3-week study period. VSS was noted to produce improvements in muscle contraction when stimulation was on.

Lessons: VSS appears to be safe, feasible, and well tolerated. VSS, as compared to standard-of-care therapy for SCI, can be used in conjunction with physical therapy and may lead to improvements in motor function. https://thejns.org/doi/10.3171/CASE24155.

背景:脊髓刺激疗法(SCS)已被证明具有促进脊髓损伤(SCI)后运动功能恢复的潜力。用于运动功能恢复的硬膜外脊髓刺激疗法传统上通过背侧电极进行。虽然腹侧硬膜外刺激可对参与运动控制的腹侧运动神经元提供更直接、更特异的刺激,但这种刺激在很大程度上尚未得到研究,其在 SCI 后运动功能恢复中的作用也不明确。为了了解腹侧硬膜外脊髓刺激(VSS)的安全性和可行性,作者介绍了一名患者,该患者在接受脊髓切除术治疗与转移性硬膜外脊髓压迫相关的 SCI 后接受了 VSS:观察结果:一名患者接受了经椎间孔镜脊髓减压术,并放置了 2 个腹侧硬膜外电极,随后同时接受了物理治疗和腹侧硬膜外刺激。术前他无法行走,但在为期三周的研究结束时,他可以在滚动助行器的帮助下行走 300 多英尺。据悉,VSS 在刺激时能改善肌肉收缩:启示:VSS 看来是安全、可行且耐受性良好的。https://thejns.org/doi/10.3171/CASE24155。
{"title":"Ventral epidural stimulation for motor recovery after spinal cord injury: illustrative case.","authors":"Abdullah K Afridi, Alexander G Steele, Catherine Martin, Dimitry G Sayenko, Sean M Barber","doi":"10.3171/CASE24155","DOIUrl":"10.3171/CASE24155","url":null,"abstract":"<p><strong>Background: </strong>Spinal cord stimulation (SCS) has demonstrated potential as a therapy to enhance motor functional recovery after spinal cord injury (SCI). Epidural SCS for motor recovery is traditionally performed via the dorsal electrode. While ventral epidural stimulation may provide more direct and specific stimulation of the ventral motor neurons involved in motor control, it is largely unstudied, and its role in motor recovery after SCI is unclear. In order to profile the safety and feasibility of ventral epidural spinal stimulation (VSS), the authors present a patient who underwent VSS following a corpectomy to treat SCI related to metastatic epidural cord compression.</p><p><strong>Observations: </strong>A patient underwent transpedicular corpectomy for spinal cord decompression, as well as the placement of 2 ventral epidural electrodes, followed by concurrent physical therapy and ventral epidural stimulation. He was nonambulatory preoperatively but was able to walk over 300 feet with the assistance of a rolling walker at the conclusion of the 3-week study period. VSS was noted to produce improvements in muscle contraction when stimulation was on.</p><p><strong>Lessons: </strong>VSS appears to be safe, feasible, and well tolerated. VSS, as compared to standard-of-care therapy for SCI, can be used in conjunction with physical therapy and may lead to improvements in motor function. https://thejns.org/doi/10.3171/CASE24155.</p>","PeriodicalId":94098,"journal":{"name":"Journal of neurosurgery. Case lessons","volume":"8 12","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-09-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11418034/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142304907","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Letter to the Editor. The need for a documented therapeutic effect before recommending vagus nerve stimulation for recovery after cerebral bleeding. 致编辑的信。在推荐使用迷走神经刺激术促进脑出血后的恢复之前,需要有治疗效果的证明文件。
Pub Date : 2024-09-16 DOI: 10.3171/CASE24263
Josef Finsterer
{"title":"Letter to the Editor. The need for a documented therapeutic effect before recommending vagus nerve stimulation for recovery after cerebral bleeding.","authors":"Josef Finsterer","doi":"10.3171/CASE24263","DOIUrl":"10.3171/CASE24263","url":null,"abstract":"","PeriodicalId":94098,"journal":{"name":"Journal of neurosurgery. Case lessons","volume":"8 12","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-09-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11418030/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142304906","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Acute peritumoral brainstem hemorrhage of a clival chondrosarcoma successfully treated via endoscopic endonasal surgery: illustrative case. 通过内窥镜腔内手术成功治疗的簇状软骨肉瘤急性瘤周脑干出血:示例病例。
Pub Date : 2024-09-16 DOI: 10.3171/CASE2460
Koki Mitani, Masahiro Tanji, Yuji Kitada, Masahiro Kikuchi, Akihiko Sugimoto, Masakazu Fujimoto, Yoshiki Arakawa

Background: Chondrosarcoma is typically a slow-growing tumor, and intratumoral hemorrhage is rare. Acute brainstem hemorrhage due to chondrosarcoma has rarely been reported.

Observations: A 77-year-old man presented with the sudden onset of headache and vomiting followed by a declining level of consciousness, progressive right hemiparesis, and left ophthalmoplegia. Magnetic resonance imaging showed pontine hemorrhage and a mass in the retroclival space compressing the brainstem. Emergency endoscopic endonasal surgery was performed. Intraoperative observation revealed that a hematoma was located in the pons and subdural space around the tumor mass, suggesting that the hematoma had likely been caused by the rupture of small vessels around the pons, not by intratumoral hemorrhage. The pathological diagnosis was chondrosarcoma. The patient recovered well and underwent radiotherapy.

Lessons: This report describes a case of sudden neurological deterioration due to hemorrhage in a patient with chondrosarcoma of the skull base. An emergency endoscopic endonasal approach for mass reduction and hematoma removal was effective in the acute setting. This approach revealed the suspected etiology of peritumoral hemorrhage, not intratumoral hemorrhage. https://thejns.org/doi/10.3171/CASE2460.

背景:软骨肉瘤通常是一种生长缓慢的肿瘤,瘤内出血非常罕见。由软骨肉瘤引起的急性脑干出血鲜有报道:一名 77 岁的男子突然出现头痛和呕吐,随后意识水平下降,右侧偏瘫和左侧眼球震颤。磁共振成像显示桥脑出血,蝶骨后间隙有肿块压迫脑干。医生紧急进行了内窥镜手术。术中观察发现,血肿位于肿瘤肿块周围的桥脑和硬膜下间隙,这表明血肿可能是由桥脑周围的小血管破裂引起的,而非瘤内出血。病理诊断为软骨肉瘤。患者恢复良好,并接受了放疗:本报告描述了一例颅底软骨肉瘤患者因出血导致神经系统突然恶化的病例。在紧急情况下,采用紧急内窥镜鼻内镜方法进行肿块缩小和血肿清除是有效的。这种方法揭示了瘤周出血而非瘤内出血的可疑病因。https://thejns.org/doi/10.3171/CASE2460。
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引用次数: 0
KEAP1-mutant atypical meningioma: illustrative case. KEAP1突变的非典型脑膜瘤:说明性病例。
Pub Date : 2024-09-09 DOI: 10.3171/CASE24387
Paul M Harary, Yusuke S Hori, Amit R L Persad, Armine Tayag, Louisa Ustrzynski, Sara C Emrich, Elham Rahimy, David J Park, Gordon Li, Steven D Chang

Background: While genetic testing of tumors is commonly used to inform the selection of systemic therapies, there is limited evidence for the application of radiotherapy for brain cancer. Recent studies have shown that Kelch-like ECH-associated protein 1 (KEAP1), a key regulator of cellular responses to oxidative and electrophilic stress, is associated with radioresistance in multiple cancer types. Several studies have reported the clinical significance of KEAP1 mutation in brain metastasis; however, the effect of KEAP1 mutations on radioresponse in meningioma has never been reported.

Observations: The authors present the case of a 40-year-old female with a KEAP1 mutation-positive atypical meningioma that was initially treated with resection followed by intensity-modulated radiation therapy (IMRT). Recurrence was observed at 15 months, requiring reoperation and adjuvant stereotactic radiosurgery (SRS). An excellent treatment response was observed at 7 months post-SRS with an improvement in reported symptoms, although bevacizumab was required for the resolution of radiation necrosis observed 2 months post-SRS.

Lessons: To the authors' knowledge, this is the first report of KEAP1-mutant meningioma, including its clinical course after comprehensive management. Notably, treatment included multimodal radiotherapy with IMRT followed by SRS. SRS led to an excellent treatment response at the 7-month follow-up. However, radiation necrosis developed after both radiotherapy treatments, suggesting that radiological modification can be beneficial in patients with KEAP1 mutations. https://thejns.org/doi/10.3171/CASE24387.

背景:虽然肿瘤基因检测通常用于为选择系统疗法提供依据,但应用于脑癌放疗的证据却很有限。最近的研究表明,Kelch 样 ECH 相关蛋白 1(KEAP1)是细胞对氧化应激和亲电应激反应的关键调节因子,与多种癌症类型的放射抗性有关。有几项研究报道了 KEAP1 突变在脑转移中的临床意义;然而,KEAP1 突变对脑膜瘤放射反应的影响却从未报道过:作者介绍了一例 KEAP1 突变阳性的 40 岁女性非典型脑膜瘤患者的病例,患者最初接受了切除术,随后接受了调强放射治疗(IMRT)。15 个月后发现复发,需要再次手术和辅助立体定向放射手术(SRS)。SRS术后7个月观察到极佳的治疗反应,报告的症状有所改善,但SRS术后2个月观察到的放射性坏死需要贝伐单抗来缓解:据作者所知,这是第一篇关于KEAP1突变脑膜瘤的报道,包括综合治疗后的临床过程。值得注意的是,治疗包括使用 IMRT 进行多模式放疗,然后进行 SRS。在7个月的随访中,SRS取得了很好的治疗效果。然而,两次放疗后都出现了放射性坏死,这表明放射学改变对KEAP1突变患者有益。https://thejns.org/doi/10.3171/CASE24387。
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引用次数: 0
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Journal of neurosurgery. Case lessons
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