low-grade glioma

低度胶质瘤
  • 文章类型: Case Reports
    磁共振成像(MRI)是神经外科不可缺少的工具,尽管它有时会面临诸如“肿瘤模仿”之类的挑战。“虽然术中MRI(iMRI)因其在神经胶质瘤手术期间实现最大安全切除的有用性而被广泛认可,iMRI上仍存在肿瘤拟态的情况。此外,关于通过iMRI观察到的肿瘤模拟物的报告,特别是在低级别胶质瘤中,保持稀缺。在这篇文章中,我们介绍了一个少突胶质细胞瘤的病例,iMRI上新出现的T2高信号强度区域需要区分肿瘤扩张。一名23岁的男子出现了新诊断的脑肿瘤,并接受了手术切除。肿瘤切除后的iMRI显示,切除腔周围有一个新出现的T2高强度区,没有扩散限制。在术前MRI中未观察到。怀疑肿瘤残留,我们进行了额外的切除.第二天的MRI证实iMRI上确定的T2高信号区域已被完全切除,但也显示了更宽区域的T2高信号区域扩大。组织病理学在额外切除的区域没有发现肿瘤细胞,表明iMRI发现是肿瘤模拟。六个月后,切除腔周围的T2高信号区域在没有任何额外治疗的情况下在MRI上消失.这种情况突出了在iMRI上看到的神经胶质瘤手术期间区分T2高强度拟态和肿瘤增大的挑战。尽管iMRI具有重要价值,我们的报告强调了在神经外科实践中需要仔细解释,特别是非对比增强肿瘤。
    Magnetic resonance imaging (MRI) is an indispensable tool in neurosurgery, though it sometimes faces challenges such as \"tumor mimicry.\" While intraoperative MRI (iMRI) is widely recognized for its usefulness in achieving maximal safe resection during glioma surgery, instances of tumor mimicry still occur on iMRI. Moreover, reports on tumor mimics observed through iMRI, particularly in low-grade gliomas, remain scarce. In this article, we present a case of oligodendroglioma, where a newly emerged T2 high-signal intensity region on iMRI necessitated differentiation from tumor expansion. A 23-year-old man presented with a newly diagnosed brain tumor and underwent surgical removal. An iMRI taken after tumor removal revealed a newly emerged T2 hyperintense area without diffusion restriction around the resection cavity, which was not observed in the preoperative MRI. Suspecting residual tumor, we performed additional resection. An MRI on the following day confirmed that the T2 hyperintense area identified on the iMRI had been completely resected but also revealed an enlarged T2 high-signal area over a wider region. Histopathology found no tumor cells in the additionally resected area, indicating that the iMRI finding was a tumor mimic. Six months later, the T2 high-signal area around the resection cavity had disappeared on MRI without any additional treatment. This case highlights the challenge of distinguishing between T2 hyperintense mimicry and tumor enlargement during glioma surgery seen on iMRI. Despite the significant value of iMRI, our report underscores the need for careful interpretation in neurosurgical practice, particularly with non-contrast-enhancing tumors.
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  • 文章类型: Case Reports
    尽管清醒手术是切除雄辩区域脑肿瘤的黄金标准,听力障碍患者在术中任务期间需要特别考虑.
    我们介绍了一名45岁的右撇子本地男性患者使用手语进行清醒手术的情况,该患者患有听力障碍和左额叶肿瘤病变,三角部(怀疑是低度胶质瘤)。患者主要通过手语和写作进行交流,但通过童年训练能够以足够听得见的水平说话。尽管患者仍然无症状,肿瘤逐渐变大。进行清醒手术切除肿瘤。开颅手术后,病人醒了,大脑功能映射是使用计数等任务进行的,图片命名,和阅读。精通手语的护士使用手语促进了交流,患者口头回应。在对肿瘤相邻区域进行电刺激期间,术中任务顺利进行,没有言语停滞或言语理解困难。肿瘤大体全切除,患者无明显并发症。病理检查显示,世界卫生组织II级少突胶质细胞瘤具有异柠檬酸脱氢酶1突变体和1p19q共缺失。
    由于这种情况下的患者从童年开始就没有因训练而引起的发音障碍,任务是用手语提出的,病人用声音回应,从而实现了安全的操作。关于听力障碍患者的清醒手术,根据听力损伤和发音障碍的程度,通过执行术中任务可以实现安全的肿瘤切除.
    UNASSIGNED: Although awake surgery is the gold standard for resecting brain tumors in eloquent regions, patients with hearing impairment require special consideration during intraoperative tasks.
    UNASSIGNED: We present a case of awake surgery using sign language in a 45-year-old right-handed native male patient with hearing impairment and a neoplastic lesion in the left frontal lobe, pars triangularis (suspected to be a low-grade glioma). The patient primarily communicated through sign language and writing but was able to speak at a sufficiently audible level through childhood training. Although the patient remained asymptomatic, the tumors gradually grew in size. Awake surgery was performed for tumors resection. After the craniotomy, the patient was awake, and brain function mapping was performed using tasks such as counting, picture naming, and reading. A sign language-proficient nurse facilitated communication using sign language and the patient vocally responded. Intraoperative tasks proceeded smoothly without speech arrest or verbal comprehension difficulties during electrical stimulation of the tumor-adjacent areas. Gross total tumor resection was achieved, and the patient exhibited no apparent complications. Pathological examination revealed a World Health Organization grade II oligodendroglioma with an isocitrate dehydrogenase one mutant and 1p 19q codeletion.
    UNASSIGNED: Since the patient in this case had no dysphonia due to training from childhood, the task was presented in sign language, and the patient responded vocally, which enabled a safe operation. Regarding awake surgery in patients with hearing impairment, safe tumor resection can be achieved by performing intraoperative tasks depending on the degree of hearing impairment and dysphonia.
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  • 文章类型: Journal Article
    背景:毛细胞性星形细胞瘤(PA)是儿童/青少年中最常见的神经胶质瘤,但在成人中并不常见且研究甚少。这里,我们描述了临床表现,手术管理,和手术治疗的成人患者心室内(IV)PA的结果,并回顾文献。
    方法:确定了在三级学术中心连续25年(1997-2023年)接受IV脑肿瘤治疗的成年患者。回顾性分析成人IVPA患者的临床资料。根据PRISMA(系统审查和荟萃分析的首选报告项目)指南进行了系统的文献综述。
    结果:纳入了8例IVPA患者。年龄中位数为25岁(范围,18-69岁),4名(50%)为女性。最常见的肿瘤位置是侧脑室(5,63%),其次是第四脑室(3,37%)。次全切除和接近全切除是最常见的手术结果(6例,75%),其次是总切除2(25%)。进展或复发3例(37%),2例患者需要重复切除。5年总生存率和无进展生存率分别为67%和40%。分别。此外,在文献中确定了42例。
    结论:成人的PA很少见,静脉输液位置更不常见。研究结果表明,在照顾这些患者方面存在挑战,总体和无进展生存结局比一般成人PA人群差。研究结果支持在可能的情况下采用有利于全切的手术技术和方法。需要进一步的研究来更好地表征这种独特的表现。
    Pilocytic astrocytomas (PA) are the most common gliomas in children/adolescents but are less common and poorly studied in adults. Here, we describe the clinical presentation, surgical management, and outcomes of surgically treated adult patients with intraventricular (IV) PA and review the literature.
    Consecutive adult patients treated for IV brain tumors at a tertiary academic center over 25 years (1997-2023) were identified. Clinical data were reviewed retrospectively for adult IV PA patients. A systematic literature review was conducted following the Preferred Reporting Items for Systematic Reviews and Meta-analyses guidelines.
    Eight patients with IV PA were included. Median age was 25 years (range, 18-69 years), and 4 (50%) were female. The most common tumor location was the lateral ventricle (5, 63%), followed by the fourth ventricle (3, 37%). Subtotal and near total resection were the most common surgical outcomes (6 patients, 75%), followed by gross total resection in 2 (25%). Progression or recurrence occurred in 3 patients (37%), requiring repeat resection in 2 patients. The 5-year overall survival and progression-free survival were 67% and 40%, respectively. In addition, 42 cases were identified in the literature.
    PAs in adults are rare and an IV location is even more uncommon. The findings demonstrate the challenges in caring for these patients, with overall- and progression-free survival outcomes being poorer than the general adult PA population. Findings support the employment of surgical techniques and approaches that favor gross total resection when possible. Further studies are needed to better characterize this unique presentation.
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  • 文章类型: Case Reports
    成熟囊性畸胎瘤(MCT)是一种常见的卵巢肿瘤,在极少数情况下,进行恶性转化。已经发现,由于抗N-甲基-D-天冬氨酸受体(NMDAR)抗体的存在,MCT患者可能会出现精神症状,这是自身免疫性脑炎的根本原因。这里,我们介绍了第一例记录的抗NMDAR脑炎患者,该患者在MCT中也有低度胶质瘤形态.
    一名45岁女性出现癫痫发作,改变了意识,NMDAR抗体IgG滴度异常,和异常的脑MRI检查结果证实了抗NMDAR脑炎的诊断。体格检查显示,在子宫附件的超声检查中,左附件区域有一个椭圆形的混合回声肿块,大小为54×37mm。患者接受腹腔镜左卵巢和输卵管切除术。病理大体检查发现一堆灰红色囊性实性碎裂组织,大小7×6×2.2cm。组织学检查显示MCT的特征成分。此外,大体组织的固体成分显示出增殖和密集排列的星形胶质细胞,具有细胞异型性,GFAP和Olig-2阳性,IDH1和EMA阴性。Ki67指数大约是10%,提示存在低度胶质瘤病变。该患者被诊断为MCT恶性转化为低度胶质瘤的形态,未指定。卵巢肿瘤切除后,病人的精神症状得到改善。
    MCT内的低级别神经胶质瘤很少发生,抗NMDAR脑炎患者出现这种恶性转化的情况更为罕见.
    UNASSIGNED: Mature cystic teratoma (MCT) is a common type of ovarian tumors that can, in rare cases, undergo malignant transformation. It has been discovered that MCT patients may experience psychiatric symptoms due to the presence of anti-N-methyl-D-aspartate receptor (NMDAR) antibodies, which is the underlying cause of autoimmune encephalitis. Here, we present the first documented case of a patient with anti-NMDAR encephalitis who also had a morphology of low-grade glioma within MCT.
    UNASSIGNED: A 45-year-old woman presented with seizures, altered consciousness, abnormal NMDAR antibody IgG titers, and abnormal brain MRI findings confirm the diagnosis of anti-NMDAR encephalitis. Physical examination revealed an oval mixed echo mass measuring 54 × 37 mm in the left adnexal area on ultrasound of the uterine appendage. The patient underwent laparoscopic left ovarian and fallopian tube resection. The pathological gross examination revealed a pile of grayish-red cystic and solid fragmented tissue measuring 7 × 6 × 2.2 cm. Histological examination revealed characteristic components of MCT. Furthermore, the solid component of the gross tissue showed proliferative and densely arranged astrocytes with cellular atypia, which were positive for GFAP and Olig-2, negative for IDH1 and EMA. And the Ki67 index was approximately 10%, suggesting the presence of low-grade glioma lesions. The patient was diagnosed with malignant transformation of MCT into a morphology of low-grade glioma, not otherwise specified. After the removal of the ovarian tumor, the patient\'s psychiatric symptoms improved.
    UNASSIGNED: Low-grade glioma within MCT is a rare occurrence, and the presence of this malignant transformation in patients with anti-NMDAR encephalitis is even more uncommon.
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  • 文章类型: Journal Article
    背景:激光间质热疗法(LITT)已成为低级别神经胶质瘤(LGG)和放射性坏死(RN)消融的微创治疗方式。
    目的:为了评估疗效,安全,影像学推测的复发性或新诊断的LGG和RN患者接受LITT治疗的生存结局。
    方法:对2013年1月1日至2020年12月31日期间接受LITT治疗LGG的所有患者的四级中心神经肿瘤数据库进行了审查。包括人口统计学在内的临床数据,病变特征,收集临床和影像学结果.Kaplan-Meier分析包括总生存期(OS)和无进展生存期(PFS)。
    结果:9名患者(7名男性,2名女性;平均[SD]年龄50[16]岁)。患者在诊断后平均11.6(8.5)年接受LITT。两名(22%)患者在没有事先治疗的情况下在影像学检查中出现了新的病变。在其他7名患者中,所有(78%)都接受了手术切除,6人(67%)接受了调强放疗和化疗,分别,和4(44%)的立体定向放射外科。两名(22%)患者的病变为野生型IDH1状态。术前T1加权对比增强和T2加权液体衰减反转恢复(FLAIR)序列的体积评估得出的平均(SD)病变体积分别为4.1(6.5)cm3和26.7(27.9)cm3。三名(33%)患者在LITT后有影像学进展的证据。该队列的汇总中位数(IQR)PFS为52(56)个月,诊断后的中位(IQR)OS为183(72)个月,LITT后的中位(IQR)OS为52(60)个月。在研究的时候,2例(22%)患者死亡。
    结论:LITT是治疗LGG和RN的一种安全有效的治疗选择,然而,深部皮质下病变的治疗可能会增加永久性并发症的风险.
    BACKGROUND: Laser interstitial thermal therapy (LITT) has emerged as a minimally invasive treatment modality for ablation of low-grade glioma (LGG) and radiation necrosis (RN).
    OBJECTIVE: To evaluate the efficacy, safety, and survival outcomes of patients with radiographically presumed recurrent or newly diagnosed LGG and RN treated with LITT.
    METHODS: The neuro-oncological database of a quaternary center was reviewed for all patients who underwent LITT for management of LGG between 1 January 2013 and 31 December 2020. Clinical data including demographics, lesion characteristics, and clinical and radiographic outcomes were collected. Kaplan-Meier analyses comprised overall survival (OS) and progression-free survival (PFS).
    RESULTS: Nine patients (7 men, 2 women; mean [SD] age 50 [16] years) were included. Patients underwent LITT at a mean (SD) of 11.6 (8.5) years after diagnosis. Two (22%) patients had new lesions on radiographic imaging without prior treatment. In the other 7 patients, all (78%) had surgical resection, 6 (67%) had intensity-modulated radiation therapy and chemotherapy, respectively, and 4 (44%) had stereotactic radiosurgery. Two (22%) patients had lesions that were wild-type IDH1 status. Volumetric assessment of preoperative T1-weighted contrast-enhancing and T2-weighted fluid-attenuated inversion recovery (FLAIR) sequences yielded mean (SD) lesion volumes of 4.1 (6.5) cm3 and 26.7 (27.9) cm3, respectively. Three (33%) patients had evidence of radiographic progression after LITT. The pooled median (IQR) PFS for the cohort was 52 (56) months, median (IQR) OS after diagnosis was 183 (72) months, and median (IQR) OS after LITT was 52 (60) months. At the time of the study, 2 (22%) patients were deceased.
    CONCLUSIONS: LITT is a safe and effective treatment option for management of LGG and RN, however, there may be increased risk of permanent complications with treatment of deep-seated subcortical lesions.
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  • 文章类型: Review
    年轻人的多形性低度神经上皮肿瘤(PLNTY),小儿型弥漫性低度胶质瘤之一,其特征在于少突胶质细胞瘤样肿瘤细胞的弥漫性浸润模式,显示CD34阳性并带有丝裂原活化蛋白激酶(MAPK)改变,如vRAF鼠肉瘤病毒癌基因同源物B1(BRAF)p.V600E或成纤维细胞生长因子基因融合。由于颞叶的主要位置,它主要发生在癫痫发作的儿科和青少年中。然而,在成年患者中有少数PLNTY病例,这表明肿瘤的范围很广。这里,我们描述了两个PLNTY案例,一个是14岁的女性,另一个是66岁的女性。小儿肿瘤表现出典型的临床病程和组织病理学,BRAFp.V600E突变,而老年肿瘤是罕见的,因为临床上是非癫痫发作和室管膜分化在组织病理学上包含KIAA1549-BRAF融合。可能有不寻常但可能有PLNTY,就像我们的老人一样。我们还通过回顾文献比较了典型的儿科和不寻常的老年肿瘤。
    Polymorphous low-grade neuroepithelial tumor of the young (PLNTY), one of the pediatric-type diffuse low-grade gliomas, is characterized by a diffuse infiltrating pattern of oligodendroglioma-like tumor cells showing CD34 positivity and harbors mitogen-activated protein kinase (MAPK) alteration, such as vRAF murine sarcoma viral oncogene homolog B1 (BRAF) p.V600E or fibroblast growth factor fusion genetically. It occurs mainly in pediatric and adolescents with seizures due to the dominant location of the temporal lobe. However, there have been a few cases of PLNTY in adult patients, suggesting the wide range of this tumor spectrum. Here, we describe two cases of PLNTY, one in a 14-year-old female and the other in a 66-year-old female. The pediatric tumor showed typical clinical course and histopathology with BRAF p.V600E mutation, whereas the elderly tumor was unusual because of non-epileptic onset clinically and ependymal differentiation histopathologically harboring KIAA1549-BRAF fusion. There might be unusual but possible PLNTY, as in our elderly case. We also compared typical pediatric and unusual elderly tumors by reviewing the literature.
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  • 文章类型: Journal Article
    目的:在一些研究中,癌症的风险与身体或器官的大小有关。我们试图研究颅内体积(ICV)(作为终生最大脑大小的替代)与IDH突变低度神经胶质瘤风险之间的关系。
    方法:在基于人群数据的多中心病例对照研究中,我们纳入了154例IDH突变型WHO2级胶质瘤患者和995例健康对照.两组的ICV是使用自动反向脑掩模方法从3DMRI脑部扫描中计算得出的,然后使用二项逻辑回归模型进行比较。
    结果:我们发现ICV与神经胶质瘤风险之间存在非线性关联,高于和低于1394ml阈值的风险增加(p<0.001)。调整ICV后,性别不是胶质瘤的危险因素.
    结论:颅内体积可能是IDH突变型低度胶质瘤的危险因素,但这种关系似乎与颅内容量阈值以上和以下的风险增加呈非线性关系.
    OBJECTIVE: Risk of cancer has been associated with body or organ size in several studies. We sought to investigate the relationship between intracranial volume (ICV) (as a proxy for lifetime maximum brain size) and risk of IDH-mutant low-grade glioma.
    METHODS: In a multicenter case-control study based on population-based data, we included 154 patients with IDH-mutant WHO grade 2 glioma and 995 healthy controls. ICV in both groups was calculated from 3D MRI brain scans using an automated reverse brain mask method, and then compared using a binomial logistic regression model.
    RESULTS: We found a non-linear association between ICV and risk of glioma with increasing risk above and below a threshold of 1394 ml (p < 0.001). After adjusting for ICV, sex was not a risk factor for glioma.
    CONCLUSIONS: Intracranial volume may be a risk factor for IDH-mutant low-grade glioma, but the relationship seems to be non-linear with increased risk both above and below a threshold in intracranial volume.
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  • 文章类型: Case Reports
    目的:累及后岛的低级别胶质瘤(LGG)的手术方法具有挑战性,尤其是在左半球,感觉运动的风险很高,语言,或视觉恶化。在这项研究中,据报道,有5例右利手患者患有左后岛叶LGG,通过详细介绍一种经皮质皮质下皮质入路。方法:使用皮质和轴突电刺激标测在清醒的患者中完成了五项手术。神经胶质瘤是通过左罗兰和/或顶叶手术切除的,保留皮层下的连通性。结果:5例患者的皮质图呈阳性,能够选择最佳的经皮质入路,4例患者通过前外侧上脑回和/或3例患者通过中央后外侧回(1例患者通过左颞上回)。此外,在所有情况下都确定了白质束,即,上纵束的外侧部分(5例),弓形束(四例),丘脑皮质体感通路(4例),运动路径(一例),语义通路(三种情况),和视神经束(一例)。两名患者实现了LGG的完全切除,三名患者几乎完全切除。术后没有永久性感觉运动,语言,或视觉缺陷。结论:在清醒患者中,通过parietoolandic手术的经皮质入路代表了安全有效的进入左后岛叶LGG的途径。在该交叉大脑区域中需要使用白质束的直接电刺激来检测和保留功能连通性,以防止其他可预测的手术后损伤。
    Objective: Surgical approach to low-grade glioma (LGG) involving the posterior insula is challenging, especially in the left hemisphere, with a high risk of sensorimotor, language, or visual deterioration. In this study, a case series of 5 right-handed patients harboring a left posterior insular LGG is reported, by detailing a transcorticosubcortical approach. Method: The five surgeries were achieved in awake patients using cortical and axonal electrostimulation mapping. The glioma was removed through the left rolandic and/or parietal opercula, with preservation of the subcortical connectivity. Results: The cortical mapping was positive in the five patients, enabling the selection of an optimal transcortical approach, via the anterolateral supramarginal gyrus in four patients and/or via the lateral retrocentral gyrus in three cases (plus through the left superior temporal gyrus in one case). Moreover, the white matter tracts were identified in all cases, i.e., the lateral part of the superior longitudinal fasciculus (five cases), the arcuate fasciculus (four cases), the thalamocortical somatosensory pathways (four cases), the motor pathway (one case), the semantic pathway (three cases), and the optic tract (one case). Complete resection of the LGG was achieved in two patients and near-total resection in three patients. There were no postoperative permanent sensorimotor, language, or visual deficits. Conclusion: A transcortical approach through the parietorolandic operculum in awake patients represents safe and effective access to the left posterior insular LGG. Detection and preservation of the functional connectivity using direct electrostimulation of the white matter bundles are needed in this cross-road brain region to prevent otherwise predictable postsurgical impairments.
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  • 文章类型: Journal Article
    Infratentorial oligodendrogliomas, a rare pathological entity, are generally considered metastatic lesions from supratentorial primary tumors. Here, we report the case of a 23-year-old man presenting with a histopathologically confirmed right precentral gyrus grade 2 oligodendroglioma and a concurrent pontine grade 3 oligodendroglioma. The pontine lesion was biopsied approximately a year after the biopsy of the precentral lesion due to disease progression despite 4 cycles of procarbazine-CCNU-vincristine (PCV) chemotherapy and stable supratentorial disease. Histology and genetic analysis of the pontine biopsy were consistent with grade 3 oligodendroglioma, and comparison of the two lesions demonstrated common 1p/19q co-deletions and TERT promoter mutations but distinct IDH1 mutations, with a non-canonical IDH1 R132G mutation identified in the infratentorial lesion and a R132H mutation identified in the cortical lesion. Initiation of Temozolomide led to complete response of the supratentorial lesion and durable disease control, while Temozolomide with subsequent radiation therapy of 54 Gy in 30 fractions resulted in partial response of the pontine lesion. This case report supports possible distinct molecular pathogenesis in supratentorial and infratentorial oligodendrogliomas and raises questions about the role of different IDH1 mutant isoforms in explaining treatment resistance to different chemotherapy regimens. Importantly, this case suggests that biopsies of all radiographic lesions, when feasible and safe, should be considered in order to adequately guide management in multicentric oligodendrogliomas.
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  • 文章类型: Case Reports
    松果体区毛细胞星形细胞瘤极为罕见,关于它们的放射学特征的信息有限。我们报告了一名22岁的女性,其松果体区域有囊性病变。在诊断后的两年里,病变沿双侧大脑内静脉和call体下外侧表面不规则延伸。实现了总切除,组织病理学研究显示病变是毛细胞星形细胞瘤。病变表现出罕见的生长,导致难以建立准确的术前诊断。应当注意,松果体区毛细胞星形细胞瘤可以表现出非典型外观。
    Pineal region pilocytic astrocytomas are extremely rare, and there is limited information about their radiological features. We report the case of a 22-year-old woman with a cystic lesion in the pineal region. In the 2 years after diagnosis, the lesion irregularly extended along the bilateral internal cerebral veins and the inferolateral surface of the corpus callosum. Gross total resection was achieved, and the histopathological study revealed that the lesion was a pilocytic astrocytoma. The lesion exhibited uncommon growth, leading to difficulty in establishing an accurate preoperative diagnosis. It should be noted that pineal region pilocytic astrocytomas can demonstrate atypical appearances.
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