multicentric

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  • 文章类型: Journal Article
    目的:神经细胞瘤占原发性脑肿瘤的0.25%至0.5%,主要见于年轻人。这些肿瘤具有神经元分化。基础治疗是神经外科。其他疗法的疗效,包括放射治疗,还不清楚。这项研究的目的是评估中枢神经细胞瘤的治疗和放射治疗的作用。
    方法:纳入了法国2006年至2015年间新诊断为组织学确诊的神经细胞瘤的所有成年患者(18岁或以上)。
    结果:研究期间有116例患者被诊断为中枢神经细胞瘤。所有患者均行手术切除,6人接受了辅助放疗。11例患者因病情进展接受放疗。在中位随访68.7个月后,29例患者发生局部衰竭。5年局部控制率为73.4%。根据单变量分析,增殖标志物Ki67指数大于2%(风险比[HR]:1.48;置信区间[CI]:1.40-1.57;P=0.027)和次全切除(HR:8.48;CI:8.01-8.99;P<0.001)与局部衰竭增加相关.总切除与癫痫后遗症(HR:3.62;CI:3.42-3.83;P<0.01)和记忆障碍(HR:1.35;CI:1.07-1.20;P<0.01)的风险较高。随访期间死亡10例(8.6%)。10年总生存率为89.0%。没有发现总生存期的预后因素。
    结论:分析表明,接受手术次全切除的患者,特别是当肿瘤的Ki67指数大于2%时,局部复发的风险增加。这些患者可以从辅助放疗中获益。
    OBJECTIVE: Neurocytomas represent 0.25 to 0.5% of primary brain tumours and are mainly found in young adults. These tumours have neuronal differentiation. The cornerstone treatment is neurosurgery. The efficacy of other therapies, including radiotherapy, is still unclear. The objective of this study was to evaluate the management of central neurocytomas and the role of radiotherapy.
    METHODS: All adult patients (age 18 years or older) newly diagnosed with a histologically confirmed neurocytoma between 2006 and 2015 in France were included.
    RESULTS: One hundred and sixteen patients were diagnosed with a central neurocytoma during the study period. All patients underwent surgical resection, and six received adjuvant radiotherapy. Eleven patients received radiotherapy due to progression. After a median follow-up of 68.7 months, local failure occurred in 29 patients. The 5-year local control rate was 73.4%. According to univariate analysis, marker of proliferation Ki67 index greater than 2% (hazard ratio [HR]: 1.48; confidence interval [CI]: 1.40-1.57; P=0.027) and subtotal resection (HR: 8.48; CI: 8.01-8.99; P<0.001) were associated with an increase in local failure. Gross total resection was associated with a higher risk of sequelae epilepsy (HR: 3.62; CI: 3.42-3.83; P<0.01) and memory disorders (HR: 1.35; CI: 1.07-1.20; P<0.01). Ten patients (8.6%) died during the follow-up. The 10-year overall survival rate was 89.0%. No prognostic factors for overall survival were found.
    CONCLUSIONS: The analysis showed that patients who underwent subtotal surgical resection, particularly when the tumour had a Ki67 index greater than 2%, had an increased risk of local recurrence. These patients could benefit from adjuvant radiotherapy.
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  • 文章类型: Case Reports
    Castleman病是一种罕见的淋巴结增生,主要影响纵隔,肠系膜局部化极为罕见。它分为孤立和多中心形式。在这个案例报告中,我们介绍了一例46岁的女性患者,在腹侧疝的检查中发现了偶然的肠系膜肿块。肿块被完全切除,组织病理学检查证实诊断为混合型Castleman病。手术是这种疾病局部形式的首选治疗方法,组织学检查对确诊至关重要。
    Castleman disease is a rare type of lymph node hyperplasia primarily affecting the mediastinum, with mesenteric localization being extremely uncommon. It is classified into solitary and multicentric forms. In this case report, we present the case of a 46-year-old female patient in whom an incidental mesenteric mass was discovered during the workup for a ventral hernia. The mass was completely excised, and the histopathological examination confirmed the diagnosis of mixed-type Castleman disease. Surgery is the treatment of choice for localized forms of this condition, and histological examination is crucial in confirming the diagnosis.
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  • 文章类型: Journal Article
    背景:患有环磷酰胺失败的淋巴瘤的狗,阿霉素,长春新碱,和泼尼松化疗(CHOP)在完成方案之前通常被认为具有较差的长期结果,但之前没有研究评估早期复发对接受抢救化疗的患者的无进展间期(PFI)或总生存时间(OST)的影响.
    目的:将多中心淋巴瘤犬的抢救治疗结果与一线CHOP化疗后的结果相关联。
    方法:数据来自先前对187只接受一线CHOP化疗然后接受洛莫司汀(CCNU)的多中心淋巴瘤犬的6项回顾性或前瞻性研究,L-天冬酰胺酶和泼尼松(LAP),或rabacfosadine(RAB,Tanovea),有或没有泼尼松或L-天冬酰胺酶。
    结果:CHOP化疗开始后的PFI与进展后的反应率显着相关,PFI,以及两种救援方案的救援后生存时间(ST)。免疫表型(B-vsT细胞)与反应无显著相关,LAP的PFI或OST,但与RAB的反应和PFI显著相关。
    结论:在一线CHOP化疗期间或之后经历短PFI的狗对抢救治疗的反应率较低,较短的PFI和ST。免疫表型对LAP的预后无显著影响,但与RAB的PFI相关。
    BACKGROUND: Dogs with lymphoma that fail cyclophosphamide, doxorubicin, vincristine, and prednisone chemotherapy (CHOP) before completion of their protocol are commonly thought to have poor long-term outcome, but no previous studies have evaluated the effect of early relapse on progression-free interval (PFI) or overall survival time (OST) for patients undergoing rescue chemotherapy.
    OBJECTIVE: Correlate rescue treatment outcomes in dogs with multicentric lymphoma with outcomes after 1st-line CHOP chemotherapy.
    METHODS: Data were collected from 6 previous retrospective or prospective studies in 187 dogs with multicentric lymphoma that received 1st-line CHOP chemotherapy and then received either lomustine (CCNU), L-asparaginase and prednisone (LAP), or rabacfosadine (RAB, Tanovea), with or without prednisone or L-asparaginase.
    RESULTS: The PFI after initiation of CHOP chemotherapy was significantly associated with response rate postprogression, PFI, and postrescue survival time (ST) for both rescue protocols. Immunophenotype (B- vs T-cell) was not significantly associated with response, PFI or OST for LAP but was significantly associated with response and PFI for RAB.
    CONCLUSIONS: Dogs that experience short PFI during or after 1st-line CHOP chemotherapy had lower response rates to rescue treatment, with shorter PFI and ST. Immunophenotype did not significantly affect outcome with LAP but was associated with PFI for RAB.
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  • 文章类型: Case Reports
    虽然比较少见,淋巴瘤是马最常见的造血肿瘤,多中心淋巴瘤仍然是该疾病最常见的表现。马淋巴瘤的发病机制仍然知之甚少,诊断通常在疾病的晚期得到证实,影响预后。这项研究调查了临床,病态,1例马多中心淋巴瘤的分子特征。
    在Vairão动物繁殖中心住院的5岁杂交母马,葡萄牙,突然出现眶上水肿和下颌淋巴结肿大的临床症状,发展中的发烧,面部水肿,和全身淋巴结病。由于多系统器官衰竭,在第一个临床症状出现24天后,母马最终死亡。血液和生化分析,尸检,并对受影响的组织进行显微镜和分子评估。尸检时,主要发现是多发性多结节性病变,沿口咽浆液表面分布,气管,心包,胃肠道,和肠系膜.微观上,这些包括对CD3(T细胞)表现出免疫阳性的肿瘤圆形细胞的实体增殖.基于这些发现,诊断为中度多中心T细胞淋巴瘤.
    关于马淋巴瘤的分子表征的研究仍然很少。作为一个实体本身是相当异构的,重要的是要描述物种间的特殊性,以了解其发展和行为。
    UNASSIGNED: Although relatively uncommon, lymphoma is the most prevalent haematopoietic neoplasia in horses, and multicentric lymphoma remains the most common presentation of the disease. The pathogenesis of equine lymphoma is still poorly understood and the diagnosis is usually confirmed at an advanced stage of the disease, compromising the prognosis. This study investigated the clinical, pathological, and molecular features of a case of equine multicentric lymphoma.
    UNASSIGNED: An apparently healthy 5-year-old crossbreed mare hospitalized at the Centre of Animal Reproduction of Vairão, Portugal, suddenly presented clinical signs of supraorbital oedema and mandibular lymph node enlargement, developing fever, facial oedema, and generalized lymphadenopathy. The mare ended up dying twenty-four days after the first clinical signs due to multisystem organ failure. Haematological and biochemical analyses, necropsy, and microscopic and molecular evaluation of affected tissues were performed. At necropsy, the main findings were multiple multinodular lesions, distributed along the serous surface of oropharynx, trachea, pericardium, gastrointestinal tract, and mesentery. Microscopically, these consisted of solid proliferations of neoplastic round cells that exhibited immunopositivity for CD3 (T cells). Based on these findings, a medium-grade multicentric T-cell lymphoma was diagnosed.
    UNASSIGNED: There is still very little research regarding the molecular characterization of lymphoma in horses. As an entity itself is quite heterogeneous, it is important to describe the interspecies particularities to understand its development and behaviour.
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  • 文章类型: Journal Article
    背景:微创手术越来越多地用于左侧胰腺切除术。SIMPLR研究旨在比较开放,腹腔镜,和使用倾向得分匹配分析的机器人方法。
    方法:这项研究包括258例胰腺左侧肿瘤患者,他们在2016年至2020年期间在三个高容量中心接受了手术。根据手术方式将患者分为三组,并以1:1的比例进行匹配。
    结果:开放组的估计失血量明显更高(620mLvs.320毫升,p<0.001),手术时间更长(273vs.216分钟,p=0.003),和更长的住院时间(16.9vs.6.81天,p<0.001)与腹腔镜组相比。淋巴结产量或切除状态无差异。比较开放组和机器人组时,机器人程序产生了更多的淋巴结(24.9与15.2,p=0.011)没有明显更长的时间。腹腔镜组手术时间较短(210vs.340分钟,p<0.001),ICU住院时间较短(0.63vs.1.64天,p<0.001),和更短的住院时间(6.61vs.11.8天,与机器人组相比,p<0.001)。三种技术之间的发病率或死亡率没有差异。
    结论:腹腔镜方法具有短期益处。这三种技术在肿瘤安全性方面是等同的,发病率,和死亡率。
    BACKGROUND: Minimally invasive surgery is increasingly preferred for left-sided pancreatic resections. The SIMPLR study aims to compare open, laparoscopic, and robotic approaches using propensity score matching analysis.
    METHODS: This study included 258 patients with tumors of the left side of the pancreas who underwent surgery between 2016 and 2020 at three high-volume centers. The patients were divided into three groups based on their surgical approach and matched in a 1:1 ratio.
    RESULTS: The open group had significantly higher estimated blood loss (620 mL vs. 320 mL, p < 0.001), longer operative time (273 vs. 216 min, p = 0.003), and longer hospital stays (16.9 vs. 6.81 days, p < 0.001) compared to the laparoscopic group. There was no difference in lymph node yield or resection status. When comparing open and robotic groups, the robotic procedures yielded a higher number of lymph nodes (24.9 vs. 15.2, p = 0.011) without being significantly longer. The laparoscopic group had a shorter operative time (210 vs. 340 min, p < 0.001), shorter ICU stays (0.63 vs. 1.64 days, p < 0.001), and shorter hospital stays (6.61 vs. 11.8 days, p < 0.001) when compared to the robotic group. There was no difference in morbidity or mortality between the three techniques.
    CONCLUSIONS: The laparoscopic approach exhibits short-term benefits. The three techniques are equivalent in terms of oncological safety, morbidity, and mortality.
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  • 文章类型: Journal Article
    目的:双特异性抗体(BsAb)是用于复发性/难治性多发性骨髓瘤的有效治疗方法。尽管耐受性良好的安全性,感染性事件在临床试验中似乎很常见.关于流行病学的真实世界数据,特点,在接受BsAb治疗的患者中,感染的危险因素和结局仍需要考虑.
    方法:回顾性研究,2020年12月至2023年2月,在14个法国中心对BsAb治疗的MM患者进行了多中心研究。主要目的是描述需要住院治疗的感染发生率,具体治疗,或BsAb施用中的适应。
    结果:在229例接受BsAb治疗的MM患者中,153(67%)接受了testlistamab,47(20%)接受了elranatamab和29(13%)talquetamab。我们共报告了234例感染,包括123名(53%)≥3级。主要感染影响呼吸道(n=116,50%),其次是菌血症(n=36,15%)。住院率为56%(n=131),20例(9%)感染导致死亡。在所有患者中,首次感染的全球累积发生率为70%,73%的患者用BCMA靶向治疗,51%的患者用GPRC5D靶向BsAb治疗。在单变量分析中,用于CRS/ICANS的皮质类固醇与较高的首次感染风险相关(HR=2.13;95CI:1.38-3.28),而GPRC5D靶向BsAb和抗菌药物预防与较低的风险相关(HR=0.53;95CI:0.3-0.94和HR=0.65;95CI:0.46-0.9).精细和灰色多变量模型发现,只有用于CRS/ICANS的皮质类固醇与较高的首次感染风险相关(HR=2.01;95CI:1.27-3.19)。
    结论:实施旨在降低BsAb感染风险的预防措施至关重要,特别是在接受CRS/ICANS糖皮质激素治疗的患者中。
    OBJECTIVE: Bispecific antibodies (BsAbs) are an effective treatment used in relapsed or refractory multiple myeloma. Despite a well-tolerated safety profile, infectious events appear to be frequent in clinical trials. Real-world data on epidemiology, characteristics, risk factors, and outcomes of infections in patients treated with BsAb are still needed.
    METHODS: A retrospective, multicentre study in BsAb-treated patients with multiple myeloma was performed in 14 French centres from December 2020 to February 2023. The primary objective was to describe the incidence of infections that required hospitalization, specific treatment, or adaptation in BsAb administration.
    RESULTS: Among 229 patients with multiple myeloma treated with BsAb, 153 (67%) received teclistamab, 47 (20%) received elranatamab, and 29 (13%) talquetamab. We reported a total of 234 infections, including 123 (53%) of grade of ≥3. Predominant infections affected the respiratory tract (n = 116, 50%) followed by bacteraemias (n = 36, 15%). The hospitalization rate was 56% (n = 131), and 20 (9%) infections resulted in death. Global cumulative incidence of the first infection was 70% in all patients, 73% in patients treated with B-cell maturation antigen-targeting, and 51% with GPRC5D-targeting BsAb. In univariate analyses, corticosteroids for cytokine release syndrome (CRS)/immune effector cell-associated neurotoxicity syndrome (ICANS) were associated with a higher risk of first infection (HR = 2.13; 95% CI, 1.38-3.28), whereas GPRC5D-targeting BsAb and anti-bacterial prophylaxis were associated with a lower risk (HR = 0.53; 95% CI, 0.3-0.94 and HR = 0.65; 95% CI, 0.46-0.9). Fine and Gray multivariate model found that only corticosteroids for CRS/ICANS were correlated with a higher risk of first infection (HR = 2.01; 95% CI, 1.27-3.19).
    CONCLUSIONS: The implementation of preventive measures that aim to mitigate the risk of infection under BsAb is pivotal, notably in patients who received corticosteroids for CRS/ICANS.
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  • 文章类型: Case Reports
    背景:淋巴瘤是马的常见肿瘤,但在驴中的报道较少。在这个案例报告中,我们描述了宏观的,肠和骨髓受累的多中心淋巴瘤的显微镜和免疫组织化学特征。
    方法:发现有慢性跛行病史的老年母驴死亡。验尸显示身体极度消瘦,牙周病,左前足椎板炎和多发性,软,白色至黄褐色肠透壁肿块,直径可达12厘米。细胞学提示肠肿瘤为圆形细胞。左侧提示肢的股骨大小是正常对侧的两倍,怀疑肿瘤浸润和骨髓和骨置换。组织学上,我们诊断为肠和左股骨淋巴瘤。免疫组织化学,肿瘤细胞显示CD3免疫标记,支持多中心T细胞淋巴瘤的诊断。
    结论:据作者所知,这是多中心淋巴瘤首次在驴中被诊断出来。进一步研究遗传背景,临床,实验室,组织病理学,和免疫组织化学,以及淋巴瘤的发病机制,需要更好地了解这种肿瘤在驴中的独特低频率。
    BACKGROUND: Lymphoma is a common neoplasm in horses but is reported much less commonly in donkeys. In this case report, we describe the macroscopic, microscopic and immunohistochemical features of a multicentric lymphoma with intestinal and bone marrow involvement.
    METHODS: A geriatric female donkey with history of chronic lameness was found dead. Post-mortem examination revealed advanced emaciation, periodontal disease, left front foot laminitis and multiple, soft, white to yellow tan intestinal transmural masses, up to 12 cm in diameter. Cytology suggested a round cell intestinal neoplasm. The femur of the left hint limb was double the size of the normal contralateral, with suspected neoplastic infiltration and replacement of bone marrow and bone. Histologically we diagnosed a lymphoma in the intestine and left femur. Immunohistochemically, the neoplastic cells showed CD3 immunolabelling, supporting a diagnosis of a multicentric T-cell lymphoma.
    CONCLUSIONS: To the authors\' knowledge, this is the first time multicentric lymphoma is diagnosed in donkeys. Further studies of the genetic background, clinical, laboratory, histopathologic, and immunohistochemical, as well as the pathogenesis of lymphoma, is needed to better understand the uniquely low frequency of this neoplasm in donkeys.
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  • 文章类型: Journal Article
    梭形细胞病变包括大量的良性和恶性病变,具有相似的临床和影像学特征。它们重叠的组织病理学特征确保了诊断困境。
    当前的多中心研究旨在根据细胞形态学和综合免疫组织化学分析来描绘成纤维细胞和成肌纤维的口腔梭形细胞病变。
    实验研究是在MSRamaiah应用科学大学进行的,班加罗尔,全印度应用科学研究所,德里。
    综合组织学评分标准和免疫组织化学标记组(STAT6,CD31,CD34,S100,SMA,波形蛋白,pan-CK,HHF-35,Ki67,ALK,desmin,HMB-45,SATB2,ERG,EMA和CD99)首次同时用于纤维母细胞和肌纤维母细胞口腔梭形细胞病变。将获得的数据制成表格并进行研究。
    NA。结果:使用细胞学评分标准和免疫组织化学标记组,分析和表征的病例为纤维增生性纤维瘤,纤维肉瘤,平滑肌肉瘤,结节性筋膜炎,神经纤维瘤和上皮样炎性肌纤维母细胞肉瘤(EIMS)。
    需要升级诊断策略,以诊断梭形细胞病变。重点必须放在细胞形态学上,免疫组织化学(IHC)标记物组对于准确诊断成纤维细胞和成肌纤维的口腔梭形细胞病变至关重要。
    UNASSIGNED: Spindle cell lesions comprise a vast plethora of benign and malignant lesions with similar clinical and radiographic features. Their overlapping histopathologic features ensure a diagnostic dilemma.
    UNASSIGNED: The current multicentric study aims to delineate fibroblastic and myofibroblastic oral spindle cell lesions based on cytomorphology and comprehensive immunohistochemical analysis.
    UNASSIGNED: The experimental study was conducted at MS Ramaiah University of Applied Sciences, Bangalore, and All India Institute of Applied Sciences, Delhi.
    UNASSIGNED: A comprehensive histological scoring criteria and panel of immunohistochemical makers (STAT6, CD31, CD34, S100, SMA, vimentin, pan-CK, HHF-35, Ki67, ALK, desmin, HMB-45, SATB2, ERG, EMA and CD99) were employed concurrently for the first time for fibroblastic and myofibroblastic oral spindle cell lesions. The data obtained was tabulated and studied.
    UNASSIGNED: NA. Results: Using cytological scoring criteria and panel of immunohistochemical makers, the cases analysed and characterized were desmoplastic fibroma, fibrosarcoma, leiomyosarcoma, nodular fasciitis, neurofibroma and epithelioid inflammatory myofibroblastic sarcoma (EIMS).
    UNASSIGNED: The diagnostic strategies need to be upgraded for the diagnosis of spindle cell lesions. Emphasis must be placed on cytomorphology, an immunohistochemistry (IHC) panel of markers is imperative for the accurate diagnosis of fibroblastic and myofibroblastic oral spindle cell lesions.
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  • 文章类型: Journal Article
    在当前全球对视网膜母细胞瘤(RB)的健康意识时代,摆在我们面前的挑战是为不发达国家受RB影响的儿童提供最佳护理。对世界各国之间的相似性和差异的理解有助于实现可比的结果。随着地理障碍的消除和合作的发展,关于RB的全球合作研究变得越来越普遍。他们提供现实世界,关于RB几个方面的有力证据。这篇综述讨论了从全球RB研究中获得的关于人口统计学的见解,某些方面的病因和流行病学,国际旅行负担,基于国民收入水平的临床表现差异,管理协议,病理学,治疗结果,以及COVID-19对世界各地RB护理的影响。这些见解可能会影响个人实践,并为政策改革提供信息。
    In the current era of global health awareness for retinoblastoma (RB), the challenge that lies ahead of us is providing optimal care for children affected with RB in underdeveloped nations. The understanding of similarities and disparities between various nations across the world aids in achieving comparable outcomes. With dissolving geographic barriers and evolving collaboration, global collaborative studies on RB are becoming increasingly common. They provide real-world, robust evidence on several aspects of RB. This review discusses insights gained from global RB studies regarding the demographics, certain aspects of etiopathogenesis and epidemiology, international travel burden, disparities in clinical presentations based on national income levels, management protocols, pathology, treatment outcomes, and the effect of COVID-19 on RB care across the world. These insights are likely to impact individual practice as well as inform policy reforms.
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  • 文章类型: Case Reports
    胶质母细胞瘤可以表现为多发性,同时,非邻接病变。我们在本报告中对多个胶质母细胞瘤进行了基因分析,并讨论了它们的病因。
    我们介绍了一名47岁女性的病例,她表现为记忆障碍并留下部分瘫痪。影像学检查显示,右侧颞叶和顶叶有三个明显不连续的病变,延伸到call体,导致多中心胶质母细胞瘤的诊断。切除所有三个病变。病变的遗传分析显示TERT启动子C228T突变,大致相当的EGFR扩增,和CDKN2A/B的纯合缺失仅在两个对比增强病变中。此外,对比增强的病变表现出相同的PTEN双碱基对突变,而未增强的病变显示出部分明显的13个碱基对突变。其他遗传特征一致。而不是每个人都从头出现,我们认为它们是通过浸润发展的,因此最好归类为多灶性胶质母细胞瘤.
    我们的发现再次强调了胶质母细胞瘤浸润的可能性,即使在T2加权图像或流体衰减反转恢复图像上没有信号变化的区域内。遗传分析在区分多发性胶质母细胞瘤是多灶性还是多中心性中起着至关重要的作用。
    UNASSIGNED: Glioblastomas can manifest as multiple, simultaneous, noncontiguous lesions. We genetically analyzed multiple glioblastomas and discuss their etiological origins in this report.
    UNASSIGNED: We present the case of a 47-year-old woman who presented with memory impairment and left partial paralysis. Radiographic imaging revealed three apparently noncontiguous lesions in the right temporal and parietal lobes extending into the corpus callosum, leading to diagnosis of multicentric glioblastomas. All three lesions were excised. Genetic analysis of the lesions revealed a TERT promoter C228T mutation, a roughly equivalent amplification of EGFR, and homozygous deletion of CDKN2A/B exclusively in the two contrast-enhanced lesions. Additionally, the contrast-enhanced lesions exhibited the same two-base pair mutations of PTEN, whereas the non-enhanced lesion showed a partially distinct 13-base pair mutation. The other genetic characteristics were consistent. Rather than each having arisen de novo, we believe that they had developed by infiltration and are therefore best classified as multifocal glioblastomas.
    UNASSIGNED: Our findings underscore anew the possibility of infiltration by glioblastomas, even within regions devoid of signal alterations on T2-weighted images or fluid-attenuated inversion recovery images. Genetic analysis can play a crucial role in differentiating whether multiple glioblastomas are multifocal or multicentric.
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