Amyloid-β PET (Aβ-PET) imaging is playing an increasingly important role in the diagnosis and treatment of Alzheimer’s Disease (AD). The Centiloid (CL) scale has been developed to standardize the measurements of Aβ PET imaging. Previous CL threshold settings were based on PET/CT, while our team’s preliminary study found that CL values from PET/MRI are higher than those from PET/CT. Therefore, there is an urgent need to establish clinical interpretation cutoffs for Aβ status via PET/MRI to facilitate application in clinical practice. The clinical performance of Aβ PET/MRI and cerebrospinal fluid biomarkers were evaluated in a multisite cohort of 720 participants. Aβ-PET scans were visually read and quantified using CL method. A two-cutoff approach identified thresholds maintaining > 90

Primary central nervous system lymphoma (PCNSL) is a rare, aggressive malignancy presenting significant therapeutic challenges. Optimal maintenance therapy following initial remission remained debated. We conducted a multicentre, real-world study comparing BTK inhibitors (BTKi, n = 23) versus lenalidomide (n = 48) as maintenance in 71 newly diagnosed PCNSL patients in remission. With a median follow-up of 41.2 months, median overall survival (OS) and progression-free survival (PFS) remained unachieved for the total cohort. BTKi maintenance was associated with significantly superior PFS compared with lenalidomide (not achieved vs. 61.0 months; p = 0.016), with a substantially higher 6-year PFS rate (91.3

Central nervous system involvement by chronic lymphocyte leukemia (CNS-CLL) without Richter transformation is a rare and hard-to-diagnose condition. Nonspecific symptoms and the need for a brain biopsy complicate diagnosis. We report a 58-year-old male with Rai stage 0 CLL who had severe neurological signs. His initial MRI suggested autoimmune-related demyelinating disease. However, high-dose steroids and intravenous immunoglobulin (IVIG) did not help. His neurological status continued to worsen. A multidisciplinary team used advanced imaging (PET-CT showing a hypermetabolic lesion with SUVmax 8.2) and stereotactic brain biopsy. Immunoglobulin heavy chain (IGH) gene clonal rearrangement showed identical clones (IGHV3-64*01/02/07) in both cerebral tissue and bone marrow. This confirmed CNS-CLL. Treatment with ibrutinib stabilized the disease and led to partial cognitive recovery. The patient survived five years without progression. This case and a literature review show that CNS-CLL can have atypical imaging and occur even in early-stage disease. Under targeted therapy, this rare condition may have a better prognosis than previously reported. The report highlights the importance of molecular studies for diagnosis and recommends a multidisciplinary diagnostic approach.

Key point:IRM appears promising and well tolerated as first-line therapy for newly diagnosed PCNS DLBCL in a small pilot cohort; these hypothesis-generating results require confirmation in larger prospective studies. Background:Primary diffuse large B-cell lymphoma of the central nervous system (PCNS DLBCL) is a rare, aggressive lymphoma with rising incidence in elderly patients. Bruton tyrosine kinase (BTK) inhibitors show promise in recurrent/refractory cases, warranting exploration in newly diagnosed disease. Methods:This single-center pilot study evaluated the safety/efficacy of ibrutinib, rituximab, and high-dose methotrexate (IRM) in nine newly diagnosed PCNS DLBCL patients (2018-2019). Treatment included 4 cycles of IRM induction, consolidation (HSCT or 2 additional IRM cycles), and maintenance therapy (ibrutinib/lenalidomide). Results:After induction, overall response rate (ORR) was 100% (complete response [CR]: 77.8%, partial response [PR]: 22.2%). Post-consolidation, CR increased to 88.9%. At a median follow-up of 77.6 months, 5-year overall survival (OS) and progression-free survival (PFS) rates were both 77.8%, with 8 patients in sustained CR and one progression. No treatment-related deaths occurred; grade ≥3 adverse events were rare (2 neutropenia, 2 anemia, 1 gastrointestinal bleeding). Conclusion:In this small pilot cohort, IRM showed promising activity and tolerability as first-line therapy for PCNS DLBCL. These descriptive findings warrant confirmation in larger prospective trials (#ChiCTR1900027811).

Purpose Retrospectively analyse the 99m Tc-MDP SPECT whole-body bone scan in POEMS syndrome to explore its clinical value. Methods Twenty-four untreated patients with pathologically confirmed POEMS syndrome were included in the study. 24 of them underwent 99m Tc-MDP SPECT whole-body bone scan, 24 underwent CT examination and 18 patients underwent X-ray examination in different parts. Features of bone lesions in 99m Tc-MDP SPECT, and X-ray, CT were analysed. Three experienced radiologists read the images and gave diagnosed results for bone lesions. Results Of the 24 POEMS syndrome patients, three types of bone lesions were found: osteosclerotic lesions, osteolytic lesions and mixed lesions, of which the most common type was osteosclerotic. 54.16% (13/24) patients were found bone lesions by SPECT; 44.44% (8/18) patients underwent X-ray and 62.50% (15/24) patients underwent CT were detected bone lesions. We compared the difference of the X-ray, CT and SPECT scans of the bone lesions by chi-square and found that there was no difference ( P = 0.51) in detection of bone lesions among the three methods. Conclusion 99m Tc-MDP SPECT wholebody bone scan also useful in evaluating patients with suspected POEMS syndrome. We can use it as a supplement examination of the CT in the confirmation of one minor diagnostic criterion for POEMS syndrome: bone lesions.

Background and Significance: Primary central nervous system lymphoma (PCNSL) is a rare and aggressive form of extranodal non-Hodgkin lymphoma with an increasing incidence, particularly among the elderly. The optimal treatment strategies for newly diagnosed PCNSL remain elusive. The Bruton's tyrosine kinase (BTK) inhibitor-ibrutinib, has shown promise in recurrent/refractory CNS lymphoma due to its efficacy and ability to penetrate the blood-brain barrier. This prospective study explored the combination of ibrutinib, rituximab, and HD-MTX ( IRM regimen) as a novel therapeutic approach for newly diagnosed PCNSL, aiming to improve response rates and minimize toxicity. Study Population: Thirty-two patients with newly diagnosed PCNSL were enrolled between January 1, 2020, and May 2024.The median age of the participants was 59 years, with a Karnofsky Performance Status (KPS) score ranging from 20 to 40. Location of Participating Centers: This single-center study was conducted at the Department of Hematology, Xuanwu Hospital, Capital Medical University, Beijing, China. Inclusion Criteria: Newly diagnosed patients with PCNSL confirmed by brain biopsy; aged 18-80 years; signed informed consent; at least one measurable lesion; ECOG 0-4; leucocytes ≥5x10^9/L, hemoglobin ≥80 g/L, platelets ≥75x10^9/L, bilirubin ≤1.5 ULN, AST ≤2.5 ULN, ALT ≤2.5 ULN, creatinine ≤1.5 ULN; LEVF ≥50%; effective contraception for men or women of childbearing age; expected survival time of at least 6 months. Exclusion Criteria: Participated in other clinical trials within 3 months before enrollment. Patients who participated in non-intervention studies were eligible to participate in this study; Received blood transfusion, erythropoietin (EPO), granulocyte colony-stimulating factor (G-CSF) or granulocyte-macrophage colony-stimulating factor (GM-CSF) treatment within 14 days before enrollment; Patients who are planned to be vaccinated with live virus, or patients who have been vaccinated within 28 days (or 5 half-life of the vaccine) before enrollment; History of gastrointestinal perforation and/or fistula within 6 months before enrollment; Those who have contraindications to any of the components in the Ibutinib-R-HD-MTX scheme; The patient had previously received treatment for lymphoma, including: chemotherapy, immunotherapy, local radiotherapy for lymphoma, surgical treatment (except tumor or pathological tissue biopsy and surgical removal not for lymphoma) and any BTK inhibitor treatment before enrollment. History of other malignancies that may affect the compliance of the research protocol or the analysis of the results (there are cured skin cells that have cured and have not relapsed within 3 years before enrollment, or skin melanoma or cervical Patients with a history of carcinoma in situ can be enrolled);Severe non-malignant tumor diseases that can affect compliance with the study protocol, such as severe cardiovascular disease (such as New York Heart Association Class III or IV heart disease, myocardial infarction or unstable type within the last 6 months)，arrhythmia or unstable angina), uncontrolled diabetes and hypertension; Known uncontrolled active infectious disease or any major infection event requiring intravenous antibiotic treatment or hospitalization (excluding tumor fever) within 4 weeks before enrollment; Human immunodeficiency virus (HIV) antibody is positive; Hepatitis C virus (HCV) antigens or antibodies were positive; Hepatitis B virus (HBV) surface antigen positive, and HBV-DNA> 10^3 copy number; Researchers consider whether anyone is unsuitable for enrollment. Endpoints: Primary endpoint: ORR. Secondary endpoints: PFS, OS and safety profile. Current Status: As of June 30, 2024, the median follow-up was 25.1 months. Of the 32 enrolled patients, 8 patients were withdrawn due to drug allergies and dropout (loss to follow-up and disease progression). Among the remaining 24 patients, 13 completed consolidation therapy and entered maintenance therapy. Five patients just completed 4 cycles of induction therapy, and six are still undergoing induction therapy. No severe adverse reactions, such as atrial fibrillation or pulmonary fungal infections, were reported. Further detailed results will be available upon study completion.

Background Developing biomarkers for early stage AD patients is crucial. Glucose metabolism measured by 18 F-FDG PET is the most common biomarker for evaluating cellular energy metabolism to diagnose AD. Arterial spin labeling (ASL) MRI can potentially provide comparable diagnostic information to 18 F-FDG PET in patients with neurodegenerative disorders. However, the conclusions about the diagnostic performance of AD are still controversial between 18 F-FDG PET and ASL. This study aims to compare quantitative cerebral blood flow (CBF) and glucose metabolism measured by 18 F-FDG PET diagnostic values in patients with Alzheimer’s disease (AD) and amnestic mild cognitive impairment (aMCI) using integrated PET/MR. Results Analyses revealed overlapping between decreased regional rCBF and 18 F-FDG PET SUVR in patients with AD compared with NC participants in the bilateral parietotemporal regions, frontal cortex, and cingulate cortex. Compared with NC participants, patients with aMCI exclusively demonstrated lower 18 F-FDG PET SUVR in the bilateral temporal cortex, insula cortex, and inferior frontal cortex. Comparison of the rCBF in patients with aMCI and NC participants revealed no significant difference ( P > 0.05). The ROC analysis of rCBF in the meta-ROI could diagnose patients with AD (AUC, 0.87) but not aMCI (AUC, 0.61). The specificity of diagnosing aMCI has been improved to 75.56% when combining rCBF and 18 F-FDG PET SUVR. Conclusion ASL could detect similar aberrant patterns of abnormalities compared to 18 F-FDG PET in patients with AD compared with NC participants but not in aMCI. The diagnostic efficiency of 18 F-FDG-PET for AD and aMCI patients remained higher to ASL. Our findings support that applying 18 F-FDG PET may be preferable for diagnosing AD and aMCI.

Objective:To investigate the clinical efficacy of adjuvant radiotherapy after surgical resection of extraventricular neurocytoma (EVNs).Methods:A retrospective study was conducted on the clinical data of 10 EVN patients (accounting for 6.8% of intraventricular and extraventricular neurocytomas treated in the same period) who underwent tumor resection with or without postoperative adjuvant radiotherapy (RT) from January 2001 to December 2020. Among them, 5 cases were admitted to the Department of Neurosurgery and Department of Radiology, Beijing Tiantan Hospital, Capital Medical University, and 5 cases were admitted to the Department of Neurosurgery and Department of Radiation Oncology of Xuanwu Hospital, Capital Medical University. The tumor was located in the sellar region in 2 cases, in the frontal lobe in 2 cases, in the thalamus in 3 cases, in the parietal lobe in 1 case and in the spinal cord in 2 cases. Six patients underwent three-dimensional conformal radiotherapy or adjuvant intensity-modulated radiation therapy with a median dose of 54 Gy (range: 50.4-58.0 Gy). Follow-up was performed postoperatively by out-patient clinic or telephone. Kaplan-Meier survival analysis was used to evaluate the survival of patients, and tumor recurrence was determined by imaging follow-up.Results:Except 1 case undergoing biopsy, the remaining 9 cases underwent tumor resection, including gross total resection in 3 cases, subtotal resection in 3 cases, and partial resection in 3 cases. All patients were followed up for a median of 69.5 months (range: 16-142 months). Among the 10 patients, a total of 3 relapsed, 2 of which were located in the cervical spinal cord and included 1 patient who underwent biopsy and 1 patient who underwent total resection. The former died of tumor recurrence 16 months after the operation; the latter underwent operation and radiotherapy after recurrence and still survived with tumor at the last follow-up. One patient developed orthotopic recurrence in the sellar region at 7 months post partial resection, underwent surgical resection and radiotherapy, and remained alive with residual tumor at the last follow-up. None of the 5 patients who received incomplete resection (subtotal resection or partial resection) plus adjuvant radiotherapy had a recurrence. Among the 3 patients who received total resection, 1 of the 2 patients who received no adjuvant radiotherapy had a recurrence, while the patient who received adjuvant radiotherapy had no recurrence. The 5-year and 10-year overall survival rates of the 10 patients were both 90%, and the 5-year and 10-year progression-free survival rates were 80% and 60%, respectively.Conclusion:Preliminary studies suggest that adjuvant radiotherapy after incomplete resection in patients with EVN may achieve progression-free survival comparable to total resection.

患者 男,39岁.因"右上肢、右前胸和右后背疼痛麻木20余天"入我院.查体:脊柱侧弯,右侧T2～T10水平浅感觉减退,右上肢发作性疼痛过敏.影像学检查:CT平扫矢状位:C2～T1水平脊髓内条形低密度影(图1).MRI矢状位:平扫C2～L2水平脊髓增粗,髓内可见条形异常信号,T1WI以低信号为主,内伴条状、不规则高信号,T2WI呈不均匀高信号(图2～4),T1WI反相位原T1WI高信号范围减小,T1WI脂肪抑制原T1WI高信号影消失;增强扫描病变在C5～T2水平结节状明显强化(图5),T3～T11水平边缘环形强化,L2水平边缘线状轻度强化(图6),其余部分未见强化.术前诊断:颈胸腰髓内占位性病变,畸胎瘤或脂肪瘤可能性大.

BACKGROUND AND PURPOSE:The presence of apolipoprotein E ε4 (APOE ε4) is associated with an increased risk of developing Alzheimer disease (AD). The aim of this study was to assess the effects of APOE ε4 on amyloid-β (Aβ) pathology, glucose metabolism, and gray matter (GM) volume and their longitudinal changes in healthy control (HC) and amnestic mild cognitive impairment (aMCI).METHODS:We included 50 HCs and 109 aMCI patients from the Alzheimer's Disease Neuroimaging Initiative phase 2/GO based on availability of baseline T1-weighted magnetic resonance imaging, 18 F-florbetapir positron emission tomography (PET), and 18 F-fluorodeoxyglucose (FDG) PET. Of these, 35 HCs and 67 aMCI patients who underwent 24-month scans were included for follow-up study.RESULTS:Voxelwise analysis revealed that APOE ε4 carriers exhibited greater baseline Aβ deposition than APOE ε4 noncarriers in both diagnostic groups. However, there was no significant difference between APOE ε4 noncarriers and APOE ε4 carriers in terms of 18 F-FDG PET standardized uptake value ratio and GM volume. Region of interest-based analysis showed statistically significant greater Aβ deposition in APOE ε4 carriers than APOE ε4 noncarriers only in aMCI patients. Furthermore, APOE ε4 carriers generally exhibited a greater magnitude and spatial extent of longitudinal changes in Aβ deposition than APOE ε4 noncarriers in both diagnostic groups.CONCLUSIONS:Our findings suggest a differential effect of APOE ε4 on Aβ pathology, glucose metabolism, and GM volume. Studying APOE ε4-related brain changes with neuroimaging biomarkers in preclinical AD offers an opportunity to further our understanding of the pathophysiology of AD at an early stage.

BACKGROUND:Alzheimer's disease (AD) is a progressive neurodegenerative disease characterized by cognitive decline and memory impairment. Amnestic mild cognitive impairment (aMCI) is the intermediate stage between normal cognitive aging and early dementia caused by AD. It can be challenging to differentiate aMCI patients from healthy controls (HC) and mild AD patients.OBJECTIVE:To validate whether the combination of 18F-fluorodeoxyglucose positron emission tomography (18F-FDG PET) and diffusion tensor imaging (DTI) will improve classification performance compared with that based on a single modality.METHODS:A total of thirty patients with AD, sixty patients with aMCI, and fifty healthy controls were included. AD was diagnosed according to the National Institute of Neurological and Communicative Diseases and Stroke/Alzheimer's Disease and Related Disorders Association (NINCDS-ADRDA) criteria for probable. aMCI diagnosis was based on Petersen's criteria. The 18F-FDG PET and DTI measures were each used separately or in combination to evaluate sensitivity, specificity, and accuracy for differentiating HC, aMCI, and AD using receiver operating characteristic analysis together with binary logistic regression. The rate of accuracy was based on the area under the curve (AUC).RESULTS:For classifying AD from HC, we achieve an AUC of 0.96 when combining two modalities of biomarkers and 0.93 when using 18F-FDG PET individually. For classifying aMCI from HC, we achieve an AUC of 0.79 and 0.76 using the best individual modality of biomarkers.CONCLUSION:Our results show that the combination of two modalities improves classification performance, compared with that using any individual modality.

患儿男,8岁,半年前始被发现无明显诱因出现愣神,呼之不应,持续数秒后自行缓解,无其他伴随症状,发作前无预兆, 1～2个月发作1次,未予特殊治疗;近2个月发作频繁,1～2次/日,外院以"癫痫发作"予丙戊酸钠治疗,症状未缓解.既往无其他特殊病史.入院查体及实验室检查未见明显异常.

患儿男,5岁,反复右侧外耳道出血23天,伴声嘶、进食呛咳22天;既往体健.查体:右耳部触痛,咽部充血.纤维喉镜示右侧声带麻痹.实验室检查无特殊.颅底M RI:右咽旁间隙见团块状肿物,长径约 6.59 cm ,T1WI呈稍低信号(图 1A) , T2WI呈高信号(图1B) ,边界清,可见包膜,向前推移右翼外肌,向后推移右茎突、右颈内动脉,向外推移右腮腺,向内压迫咽腔,向上破坏右颞骨岩部;增强 T1WI示病灶呈不均匀强化(图1C );M RI诊断:(右咽旁间隙)良性病变可能,腮腺多形性腺瘤?行内镜下经口、经鼻咽旁间隙病变切除术.术后病理:肿瘤呈鱼肉样;光镜下见肿瘤细胞呈星芒状、卵圆形及多角形巢片状生长,核染色质深,核分裂象易见(图1D );免疫组织化学:Vim(少许+) ,Des(散在+) ,Myo(部分+) ,MyoD1(+);特殊染色:AB/PAS (+).病理诊断:(右咽旁间隙)胚胎性横纹肌肉瘤(embryonal rhabdomyosarcoma ,ERMS).

患者男,29岁,无明显诱因间歇性头痛3月余;既往体健.查体及实验室检查均未见明显异常.头颅MRI:右额叶5.5 cm×5.2 cm×5.7 cm 肿块,呈 T1WI 低信号(图 1A)、T2WI稍高信号,内见囊变,瘤周见水肿(图1B),弥散加权成像(diffusion weighted imaging,DWI)呈稍高信号,表观弥散系数(apparent diffusion coefficient,ADC)为 0.88×10-3 mm2/s(图1C),增强T1WI肿块呈明显不均匀强化,与大脑镰关系密切(图1D);右侧额顶颞部硬膜下血肿T1WI(图1A、1D)、DWI(图1C)均呈高信号;诊断:右额叶占位,硬脑膜起源恶性肿瘤可能.行全麻下颅内肿瘤切除术,术中见右额叶类圆形肿瘤,血供极丰富,伴出血,其右侧与脑组织分界清楚,左侧与大脑镰紧密粘连.

目的 探讨蝶窦异位垂体瘤的临床表现及影像特征,旨在提高诊断准确率.方法 搜集2010年1月至2020年5月本院手术证实的12例蝶窦异位垂体瘤.分析其临床特征,化验检查特征及CT、MRI特征.结果 12例蝶窦异位垂体瘤中2例患者无症状,偶然发现,其余10例临床表现不典型,部分症状与肿瘤的大小、对邻近结构的侵犯相关.血激素检查12例病例均出现2种以上激素异常.CT平扫12例均表现肿块周围骨质压迫性骨质吸收、骨质破坏,未见骨质增生硬化征象.MRI上12例肿瘤T1WI/T2 WI上表现为不均匀等信号,中度不均匀强化.12例中2例出现空泡蝶鞍.结论蝶窦异位垂体瘤具有典型的CT及MRI表现,术前血激素检查多能发现一种或多种激素水平异常.认识这些特征,可显著提高术前诊断准确率.

女性患儿,9个月,发现搀扶下无法站立20天;泌尿系统感染20余天,于外院接受对症治疗;足月顺产,第一胎,无家族及遗传病史.查体:双下肢活动力弱.实验室检查:白细胞计数13.72×109/L,淋巴细胞百分率49.3％.腰椎MRI:L2～S1水平椎管内硬膜外类椭圆形异常信号,边界清,最大径5.7 cm,T1WI、T2WI呈等信号(图1A);L2～5双侧椎间孔扩大,肿物经椎间孔向椎旁突出(图1B),L3附件见异常信号,周围见软组织信号,L2～5水平马尾神经明显受压聚拢向腹侧移位,与肿物分界尚清;增强扫描椎管内肿物明显较均匀强化,L3附件及周围病变呈明显均匀强化.腰椎CT:L2～5左侧椎弓根、横突和棘突骨质呈侵蚀性骨质破坏(图1C).影像学诊断:腰骶椎管神经源性肿瘤.行椎管内外肿瘤切除术,术中硬膜外肿物与周围组织粘连严重,侵犯周围骨、横纹肌及脂肪组织,硬膜囊受压向腹侧移位.术后病理:灰白色肿物,切面呈鱼肉状,质地软,部分质韧,血供丰富;光镜下见小圆细胞排列紧密,易见核分裂象(图1D).免疫组织化学:Vim(部分+),LGA(+),Nkx2.2(+),Fli-1(+).病理诊断:(腰骶椎管)尤因肉瘤/原始神经外层肿瘤 (Ewing sarcoma/primary neuroectodermal tumor,EWS/PNET).

MR扩散加权成像(DWI)可反映组织水分子的布朗运动.近年来,胸部DWI成像参数日趋规范,图像质量明显提高,对鉴别诊断胸部良、恶性肿瘤,评价纵隔淋巴结转移及放射和化学治疗、射频消融治疗肺癌效果具有重要价值.本文对DWI在胸部疾病中的应用进展进行综述.

Primary tumors of the spinal cord are rare, accounting for 3–6% of tumors in the central nervous system, particularly in children. KIAA1549-BRAF fusion is more common in pilocytic astrocytoma (PA) and IDH1 R132H mutation is rare in infratentorial tumors. Here, we report a 10-year-old male patient who presented with weakness in lower limbs that progressed to difficulty walking. Magnetic resonance imaging (MRI) revealed an intramedullary solid-cystic lesion from the medulla oblongata to the thoracic spin 4 level, with the expansion of the spinal cord. The lesion exhibited patchy enhancement at C4-T1, indicating a tentative diagnosis of astrocytoma. The patient underwent resection of the lesion in the spinal canal from the cervical 6 level to the thoracic 2 level. Histopathology confirmed diagnosis of astrocytoma, WHO grade 2. Genetic analysis showed both IDH1 R132H mutation and KIAA1549-BRAF fusion. Therefore, our integrated diagnosis was astrocytoma, IDH mutation, WHO grade 2. Its molecular analyses include IDH1 R132H mutation and KIAA1549-BRAF fusion. After the operation, the patient did not receive chemo- or radiotherapy, and underwent an aggressive rehabilitation regiment. Follow up 10 months later, symptoms improved. To our best knowledge, this is the first case of concomitant IDH mutation and BRAF fusion in pediatric spinal cord astrocytoma.