primary CNS lymphoma

原发性中枢神经系统淋巴瘤
  • 文章类型: Journal Article
    肌肉减少症与实体癌预后恶化相关。颞肌厚度(TMT)已成为衡量肌肉减少症的指标。因此,本研究旨在评估恶性轴内肿瘤患者的TMT与预后指标之间的关系.
    我们搜索了Medline,Embase,Scopus和Cochrane数据库进行相关研究。使用RevMan5.4软件分析具有95%置信区间(CI)的事件比率。在无法进行荟萃分析的地方,投票计数用于确定TMT对结果的影响。等级框架用于确定证据的确定性。
    在涉及4430名患者的17项研究中,报告了3项疾病的4项结果。胶质母细胞瘤:较厚的TMT对总生存期(OS)具有保护作用(HR0.59;95%CI0.46-0.76)(等级低),无进展生存期(PFS)(HR0.40;95%CI0.26-0.62)(分级高),和早期停止治疗(OR0.408;95%CI0.168-0.989)(等级高);与并发症无关(HR0.82;95%CI0.60-1.10)(等级低)。脑转移:较厚的TMT对OS具有保护作用(HR0.73;95%CI0.67-0.78)(中度);与PFS无关(低等级)。原发性中枢神经系统淋巴瘤:TMT对总生存期(HR0.34;95%CI0.19-0.60)(中度)和无进展生存期(HR0.23;95%CI0.09-0.56)(高度)具有保护作用。
    TMT在轴内恶性肿瘤中具有重要的预后潜力,显示其与GRADE评估后结果的相关性具有中等到高度的确定性。这将使患者和临床医生之间的共同决策成为可能。
    UNASSIGNED: Sarcopenia is associated with worsened outcomes in solid cancers. Temporalis muscle thickness (TMT) has emerged as a measure of sarcopenia. Hence, this study aims to evaluate the relationship between TMT and outcome measures in patients with malignant intra-axial neoplasms.
    UNASSIGNED: We searched Medline, Embase, Scopus and Cochrane databases for relevant studies. Event ratios with 95% confidence intervals (CI) were analysed using the RevMan 5.4 software. Where meta-analysis was impossible, vote counting was used to determine the effect of TMT on outcomes. The GRADE framework was used to determine the certainty of the evidence.
    UNASSIGNED: Four outcomes were reported for three conditions across 17 studies involving 4430 patients. Glioblastoma: thicker TMT was protective for overall survival (OS) (HR 0.59; 95% CI 0.46-0.76) (GRADE low), progression free survival (PFS) (HR 0.40; 95% CI 0.26-0.62) (GRADE high), and early discontinuation of treatment (OR 0.408; 95% CI 0.168-0.989) (GRADE high); no association with complications (HR 0.82; 95% CI 0.60-1.10) (GRADE low). Brain Metastases: thicker TMT was protective for OS (HR 0.73; 95% CI 0.67-0.78) (GRADE moderate); no association with PFS (GRADE low). Primary CNS Lymphoma: TMT was protective for overall survival (HR 0.34; 95% CI 0.19-0.60) (GRADE moderate) and progression free survival (HR 0.23; 95% CI 0.09-0.56) (GRADE high).
    UNASSIGNED: TMT has significant prognostic potential in intra-axial malignant neoplasms, showing a moderate to high certainty for its association with outcomes following GRADE evaluation. This will enable shared decision making between patients and clinicians.
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  • 文章类型: Meta-Analysis
    目的:评估无进展生存期(PFS)作为原发性中枢神经系统淋巴瘤(PCNSL)总生存期(OS)的早期替代终点。方法:PubMed,截至2022年6月7日,搜索了Embase和Cochrane中央图书馆。通过对PFS和OS的对数风险比进行加权线性回归进行试验水平分析。在PFS率和3年或5年OS率之间进行治疗组水平分析。结果:纳入9项随机对照试验中的1471例PCNSL患者。PFS与OS相关(r=0.750;95%CI:0.228-0.937)。1-,2年和3年PFS和3年OS(r=0.896-0.928),3至6个月的PFS和3年的OS之间存在中度或弱相关性,3个月至5年PFS和5年OS。结论:PCNSL中短期PFS可以有效替代长期OS。
    Purpose: To evaluate progression-free survival (PFS) as early surrogate endpoints for overall survival (OS) in primary CNS lymphoma (PCNSL). Methods: PubMed, Embase and Cochrane Central Library were searched up to 7 June 2022. Trial-level analyses were performed by weighted linear regression of logarithmic hazard ratios for PFS and OS. Treatment arm-level analyses were performed between PFS rates and 3- or 5-year OS rates. Results: 1471 PCNSL patients in nine randomized control trials were included. PFS was associated with OS (r = 0.750; 95% CI: 0.228-0.937). Strong linear correlations existed between 1-, 2- and 3-year PFS and 3-year OS (r = 0.896-0.928), moderate or weak correlations existed between 3- to 6-month PFS and 3-year OS, 3-month to 5-year PFS and 5-year OS. Conclusion: Short-term PFS can validly substitute for long-term OS in PCNSL.
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  • 文章类型: Case Reports
    鞍区原发性结外粘膜相关淋巴组织(MALT)淋巴瘤是一种罕见的惰性B细胞淋巴瘤。
    报道了一名新诊断的源自垂体柄的MALT淋巴瘤患者。在一名24岁的男子中发现了鞍区占位性病变,该男子除了在职前体检中发现的与性激素紊乱(雌激素上升和雄激素下降)有关的症状外,没有临床症状。病理诊断为MALT淋巴瘤。术后建议进行放疗和化疗。然而,患者仅选择雄激素替代疗法而非放化疗.在接下来的3个月里,没有视觉干扰,头痛,颅神经异常,或出现其他症状。
    原发性鞍区MALT淋巴瘤是一种极为罕见的疾病。鞍区和鞍区肿块的鉴别诊断应包括原发性鞍区MALT淋巴瘤。早期发现和治疗淋巴瘤可有效改善预后。
    UNASSIGNED: Primary extranodal mucosa-associated lymphoid tissue (MALT) lymphoma in the sellar region is a rare indolent B-cell lymphoma.
    UNASSIGNED: A newly diagnosed patient with MALT lymphoma originating from the pituitary stalk is reported. A space-occupying lesion in the sellar region was found in a 24 year-old man who had no clinical symptoms except for those relating to a sex hormone disorder (rising estrogen and falling androgen) identified during a pre-employment physical examination. MALT lymphoma was diagnosed pathologically. Radiotherapy and chemotherapy were proposed after surgery. However, the patient selected androgen replacement therapy only rather than chemoradiotherapy. Over the next 3 months, no visual disturbance, headache, cranial nerve abnormality, or other symptoms occurred.
    UNASSIGNED: Primary sellar region MALT lymphoma is an extremely rare disease. The differential diagnosis of sellar and parasellar masses should include primary sellar region MALT lymphoma. Early detection and treatment of this lymphoma can effectively improve the prognosis.
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  • 文章类型: Journal Article
    背景:巩固治疗改善了新诊断的PCNSL患者的预后。全脑放疗(WBRT)是第一个使用的整合策略,并代表了多年的黄金标准,但以神经毒性的高风险为代价。因此,为了改善疾病结局和避免WBRT引起的神经认知副作用,正在研究替代策略。
    方法:我们回顾了已发表的关于使用HDC/ASCT治疗的PCNSL患者的研究,重点关注调理方案的疗效和安全性。前瞻性和回顾性研究,1992年至2022年以英语发表,在PubMed上确定了高质量的国际期刊。
    结果:用含有高度中枢神经系统渗透剂的HDC进行合并(thiotepa,白消安或BCNU),然后是ASCT,为PCNSL患者提供了长期的疾病控制和生存率。两项前瞻性随机研究,比较HDC/ASCT与WBRT,报道了相似的无进展生存期(PFS),相当比例的患者在WBRT后神经认知功能下降方面的结果相似,但在HDC-ASCT后没有.最近一项比较HDC/ASCT与非清髓性巩固的随机研究报道移植患者PFS更长。
    结论:ASCT以方案为条件,包括高度中枢神经系统渗透剂,代表,到目前为止,适用于新诊断的PCNSL患者的可用巩固策略中的最佳选择。
    BACKGROUND: Consolidation therapy has improved the outcome of newly diagnosed PCNSL patients. Whole-brain radiotherapy (WBRT) was the first consolidation strategy used and represented the gold standard for many years, but at the expense of a high risk of neurotoxicity. Thus, alternative strategies are being investigated in order to improve disease outcomes and to spare the neurocognitive side effects due to WBRT.
    METHODS: We reviewed published studies on PCNSL patients treated with HDC/ASCT, focusing on the efficacy and safety of the conditioning regimens. Prospective and retrospective studies, published in the English language from 1992 to 2022, in high-quality international journals were identified in PubMed.
    RESULTS: Consolidation with HDC containing highly CNS-penetrating agents (thiotepa, busulfan or BCNU) followed by ASCT provided long-term disease control and survival in PCNSL patients. Two prospective randomized studies, comparing HDC/ASCT versus WBRT, reported similar progression-free survival (PFS) and similar results on the decline in neurocognitive functions in a substantial proportion of patients after WBRT but not after HDC-ASCT. A recent randomized study comparing HDC/ASCT versus non-myeloablative consolidation reported a longer PFS in transplanted patients.
    CONCLUSIONS: ASCT conditioned with regimens, including highly CNS-penetrating agents, represents, to date, the best choice among the available consolidation strategies for fit newly diagnosed PCNSL patients.
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  • 文章类型: Systematic Review
    复发/难治性中枢神经系统(CNS)淋巴瘤,无论是主要的还是次要的,与目前可用的治疗方式的不良预后相关,包括大剂量化疗-自体干细胞移植。关键的ZUMA-1和JULIET试验导致FDA批准Axicabtageneciloleucel和Tisagenlecleucel用于复发性难治性大细胞淋巴瘤,由于担心毒性增加,排除了中枢神经系统受累的患者。然而,Lisocabtagenemaraleucel在复发性难治性大细胞淋巴瘤中的TRANSCEND研究允许中枢神经系统受累的患者,并报告了这些患者可控制的中枢神经系统毒性。在现实世界的经验,嵌合抗原受体T细胞(CAR-T)治疗对于这些预后不良的患者被认为是安全有效的.在这次系统审查中,我们分析了现有文献,以评估使用Embase的CART细胞疗法在原发性和继发性CNS淋巴瘤中的作用,科克伦,和PubMed数据库。共14项研究,包括8项回顾性分析和6项前瞻性研究/临床试验,包括在定性合成中,以研究CART的安全性和有效性。基于我们的分析,CART细胞疗法似乎与原发性和继发性CNS淋巴瘤的合理疗效和可控制的安全性相关。
    Relapsed/refractory central nervous system (CNS) lymphoma, whether primary or secondary, is associated with poor prognosis with currently available treatment modalities, including high-dose chemotherapy-autologous stem cell transplantation. The pivotal ZUMA-1 and JULIET trials that led to FDA approval of Axicabtagene ciloleucel and Tisagenlecleucel for relapsed refractory large cell lymphoma excluded patients with CNS involvement due to concerns of increased toxicity. However, TRANSCEND study for Lisocabtagene maraleucel in relapsed refractory large cell lymphoma allowed patients with CNS involvement and reported manageable CNS toxicities in these patients. In the real-world experience, chimeric antigen receptor T-cell (CAR T) therapy has been deemed safe and effective for these patients with poor prognosis. In this systematic review, we analyzed available literature to evaluate the role of CAR T-cell therapy in both primary and secondary CNS lymphoma using Embase, Cochrane, and PubMed databases. A total of 14 studies, including 8 retrospective analyses and 6 prospective studies/clinical trials, were included in the qualitative synthesis to study the safety and efficacy of CAR T. Based on our analysis, CAR T-cell therapy appears to be associated with reasonable efficacy and a manageable safety for primary and secondary CNS lymphoma.
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  • 文章类型: Meta-Analysis
    新诊断的原发性中枢神经系统淋巴瘤(PCNSL)的治疗包括给予基于大剂量甲氨蝶呤的方案,然后进行巩固治疗,以最大程度地降低复发风险。然而,最佳巩固策略(自体造血细胞移植[auto-HCT]与全脑放疗[WBRT])是有争议的。因此,我们对所有随机对照试验进行了系统评价和荟萃分析,比较了一线治疗后PCNSL患者的auto-HCT与WBRT巩固治疗.主要结果是总生存期(OS),而次要结局包括无进展生存期(PFS),缓解率(总体缓解率[ORR]和完全缓解率[CR]),复发率,治疗相关死亡率(TRM),和神经心理学不良事件。我们对纳入自动HCT或WBRT巩固的单臂研究进行了汇总分析,并评估了神经认知结果。只有两项研究符合纳入标准(n=240)。OS差异无统计学意义(HR=1.50;95%CI=0.95-2.36),PFS(HR=0.99;95%CI=0.44-2.22),ORR(RR=1.48;95%CI=0.90-2.44),CR率(RR=1.21;95%CI=0.90-1.63),复发率(RR=0.46;95%CI=0.05-4.28),和TRM(RR=5.67;95%CI=1.01-31.91)。在两项研究中,用于评估神经认知领域的神经心理学测试是不同的,并且报告不一致,因此我们无法进行荟萃分析,但提供了描述性评估。两项研究均显示,与自动HCT相比,接受WBRT的人的注意力/执行功能(基于跟踪测试A和跟踪测试B)显着下降。我们发现了9项单臂II期研究,报告了与自动HCT(5项研究)或WBRT(4项研究)合并相关的结果数据。其中,两项研究(n=43)报道了自动HCT巩固后神经认知功能下降的数据。在这些研究中,接受auto-HCT的神经认知能力下降患者的合并比例为6%(95%CI,0%-17%),并且研究之间没有异质性(I2=0%)。三项研究(n=122)报道了WBRT巩固后神经认知能力下降的数据。在这些研究中,接受WBRT的神经认知能力下降患者的合并比例为43%(95%CI,11%-78%),并且研究之间存在高度异质性(I2=94%)。亚组之间存在显著异质性(p=0.035)。接受自动HCT或WBRT巩固治疗的PCNSL患者的结果没有显着差异。然而,与自动HCT巩固相比,与WBRT相关的神经认知能力下降程度更高.选择整合策略的决定需要根据年龄进行个性化,脆弱,和合并症。
    The management of newly diagnosed primary central nervous system lymphoma (PCNSL) includes administration of high-dose methotrexate based regimens followed by consolidation therapy to minimize the risk of relapse. However, the best consolidation strategy (autologous hematopoietic cell transplant [auto-HCT] vs. whole-brain radiotherapy [WBRT]) is controversial. Hence, we performed a systematic review and meta-analysis of all randomized controlled trials that compared auto-HCT versus WBRT consolidation for patients with PCNSL after first-line treatment.The primary outcome was overall survival (OS), while the secondary outcomes included progression-free survival (PFS), response rates (overall response rate [ORR] and complete remission [CR]), relapse rate, treatment-related mortality (TRM), and neuropsychological adverse events. We performed a pooled analysis of the single-arm studies that incorporated auto-HCT or WBRT consolidation and evaluated neurocognitive outcomes. Only two studies met the inclusion criteria (n = 240). There was no significant difference in OS (HR = 1.50; 95% CI = 0.95-2.36), PFS (HR = 0.99; 95% CI = 0.44-2.22), ORR (RR = 1.48; 95% CI = 0.90-2.44), CR rate (RR = 1.21; 95% CI = 0.90-1.63), relapse rate (RR = 0.46; 95% CI = 0.05-4.28), and TRM (RR = 5.67; 95% CI = 1.01-31.91). The neuropsychological tests to assess neurocognitive domains were different and inconsistently reported in the two studies and therefore we were unable to perform a meta-analysis but provide a descriptive assessment. Both the studies showed a significant decline in the attention/executive function (based on the trail making test A and trail making test B) in those receiving WBRT compared to auto-HCT. We found 9 single-arm phase II studies that reported data on outcomes associated with either auto-HCT (5 studies) or WBRT (4 studies) consolidation. Of these, two studies (n = 43) reported data on neurocognitive decline following auto-HCT consolidation. Pooled proportion of patients with neurocognitive decline in these studies was 6% (95% CI, 0%-17%) for those receiving auto-HCT and there was no heterogeneity between studies (I2  = 0%). Three studies (n = 122) reported data on neurocognitive decline following WBRT consolidation. Pooled proportion of patients with neurocognitive decline in these studies was 43% (95% CI, 11%-78%) for those receiving WBRT and there was high heterogeneity between studies (I2  = 94%). There was significant heterogeneity between subgroups (p = 0.035). The outcomes were not significantly different in patients with PCNSL receiving auto-HCT or WBRT consolidation therapies, however, there is a higher degree of neurocognitive decline associated with WBRT compared to auto-HCT consolidation. The decision to choose a consolidation strategy needs to be individualized based on age, frailty, and co-morbidities.
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  • 文章类型: Journal Article
    原发性中枢神经系统淋巴瘤(PCNSL)约占所有原发性脑肿瘤的3%,占所有非霍奇金淋巴瘤的近1-3%。近年来,原发性CNS淋巴瘤在免疫功能正常的患者中的发病率正在增加。由于PCNSL对化学敏感和放射敏感,它的早期和准确的诊断是必要的优化管理。对比增强磁共振成像(MRI)是PCNSL的推荐成像模式;然而,对比增强计算机断层扫描(CE-CT)是在MRI是禁忌的情况下进行。先进的成像技术,如DWI(扩散加权成像),MRS(MR光谱),MR灌注,DTI(扩散张量成像)在诊断和帮助其与其他肿瘤的鉴别中很重要。
    Primary central nervous system lymphoma (PCNSL) constitutes about 3% of all primary brain tumors and nearly 1 to 3% of all Non Hodgkin Lymphomas. In the recent years the incidence of primary CNS lymphoma is increasing in immunocompetent patients. As PCNSL are chemosensitive as well as radiosensitive, its early and accurate diagnosis is imperative for optimal management. Contrast enhanced Magnetic Resonance Imaging (MRI) is the recommended imaging modality for PCNSL; however, contrast enhanced Computed Tomography (CE-CT) is done in cases where MRI is contraindicated. Advanced imaging techniques like DWI (diffusion weighted imaging), MRS (MR Spectroscopy), MR perfusion, DTI (Diffusion tensor imaging) are important in diagnosis and help in its differentiation from other tumors.
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  • 文章类型: Journal Article
    动脉自旋标记(ASL)是一种非侵入性神经成像技术,可以在没有静脉造影的情况下量化脑血流量。各种神经血管疾病和肿瘤具有脑血流改变。通过ASL识别这些灌注变化可以帮助诊断,尤其是在具有正常结构成像的实体中。此外,也可以使用ASL监测肿瘤治疗和肿瘤进展的并发症。在这个基于案例的审查中,我们展示了ASL在诊断和监测脑肿瘤和治疗并发症方面的临床应用。
    Arterial spin labeling (ASL) is a noninvasive neuroimaging technique that allows for quantifying cerebral blood flow without intravenous contrast. Various neurovascular disorders and tumors have cerebral blood flow alterations. Identifying these perfusion changes through ASL can aid in the diagnosis, especially in entities with normal structural imaging. In addition, complications of tumor treatment and tumor progression can also be monitored using ASL. In this case-based review, we demonstrate the clinical applications of ASL in diagnosing and monitoring brain tumors and treatment complications.
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  • 文章类型: Case Reports
    UNASSIGNED: Primary central nervous system (CNS) lymphoma is an uncommon non-Hodgkin disease limited to the CNS, and most cases are diffuse large B-cell lymphomas. Other pathologies, including lymphoplasmacytic lymphoma (LPL), are exceedingly rare and poorly understood. The clinical presentation of primary CNS LPL is diverse. It depends on the original site and the tumor\'s extension. There is currently no consensus on a treatment strategy for this uncommon manifestation. To our knowledge, no previously published case was successfully treated with radiation therapy alone.
    UNASSIGNED: We present here a case of primary CNS LPL. A 46-year-old, previously healthy woman was presented with a worsening headache and lower extremity numbness. Multifocal enhanced masses were detected in an MRI with biopsy results consistent with LPL. A complete staging workup was performed with no evidence of systemic disease. The patient received external-beam radiotherapy alone and had a complete remission. After 2 years of follow-up, she remains disease-free.
    UNASSIGNED: Radiation alone is a promising treatment option for primary CNS lymphoplasmacytic lymphoma.
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  • 文章类型: Case Reports
    Primary pituitary lymphoma (PPL) represents an extremely rare entity. Here, we have reported two recently identified cases of immunocompetent PPL having diffuse large B-cell lymphoma by surgical biopsy. Both patients had hypopituitarism, with one patient developing right ptosis. In both patients, MRI and FDG-PET/CT depicted sellar mass that extended into the cavernous sinus with the right sphenoid also present in one of the patients. No systemic disease was found in these two patients. Surprisingly, we found that both patients had infiltrative lesions in sphenoid sinus mucosa pathologically, but the sphenoid bones that composed the sellar base were visually intact during the biopsy procedure. Chemotherapy was administered to both patients, where one patient achieved remission at the recent follow-up, whereas the other one did not respond to the treatment. The diagnosis of PPL is usually difficult if solely dependent on history, clinical presentation, biochemical indexes, and radiographic findings. We have also updated and reviewed the epidemiologic features, clinical presentations, pathological characteristics, potential mechanisms, therapeutic orientation, and prognostic advances of PPL. A total of 40 cases (including ours and four pediatric patients), histologically diagnosed, were analyzed in terms of clinical presentation, endocrine abnormality, radiological features, pathology, treatment, and follow-up. Hypopituitarism and headache were the most common presentation of PPL, while diabetes insipidus was reported in 13 patients (43.3%). B cell lymphoma was the most common type of pathology, followed by T-cell and NK/T cell. PPL was more invasive in nature at the suprasellar region (72.5%), cavernous sinus (52.5%), and sphenoidal sinus (27.5%) in 29, 21, and 11 patients, respectively. Pediatric patients with PPL seem to be different compared to their adult counterparts in terms of pathogenesis, clinical presentation, and radiological features. The management of PPL usually follows the treatment protocols for PCNSL but has a poor prognosis compared to the pituitary involvement of systemic lymphoma.
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