Journal of Neuropathology & Experimental Neurology最新文献_第7页

Elevated Expression of the Cerebrospinal Fluid Disease Markers Chromogranin A and Clusterin in Astrocytes of Multiple Sclerosis White Matter Lesions 多发性硬化症白质病变星形胶质细胞中脑脊液疾病标志物嗜铬粒蛋白A和聚簇蛋白的表达升高

Journal of Neuropathology & Experimental Neurology Pub Date : 2016-01-01 DOI: 10.1093/jnen/nlv004

Marvin M. van Luijn, M. van Meurs, M. Stoop, E. Verbraak, A. Wierenga-wolf, M. Melief, K. Kreft, R. Verdijk, B. '. ’t Hart, T. Luider, J. Laman, R. Hintzen

{"title":"Elevated Expression of the Cerebrospinal Fluid Disease Markers Chromogranin A and Clusterin in Astrocytes of Multiple Sclerosis White Matter Lesions","authors":"Marvin M. van Luijn, M. van Meurs, M. Stoop, E. Verbraak, A. Wierenga-wolf, M. Melief, K. Kreft, R. Verdijk, B. '. ’t Hart, T. Luider, J. Laman, R. Hintzen","doi":"10.1093/jnen/nlv004","DOIUrl":"https://doi.org/10.1093/jnen/nlv004","url":null,"abstract":"Using proteomics, we previously identified chromogranin A (CgA) and clusterin (CLU) as disease-related proteins in the cerebrospinal fluid (CSF) of patients with multiple sclerosis (MS). CgA and CLU are involved in cell survival and are implicated in neurodegenerative disorders and may also have roles in MS pathophysiology. We investigated CgA and CLU expression in lesions and nonlesional regions in postmortem brains of MS patients and controls and in the brains of marmosets with experimental autoimmune encephalomyelitis. By quantitative PCR, mRNA levels of CgA and CLU were elevated in white matter but not in grey matter of MS patients. In situ analyses showed greater expression of CgA and CLU in white matter lesions than in normal-appearing regions in MS patients and in the marmosets, primarily in or adjacent to perivascular spaces and inflammatory infiltrates. Both proteins were expressed by glial fibrillary acidic protein-positive astrocytes. CgA was more localized in astrocytic processes and endfeet surrounding blood vessels and was abundant in the superficial glia limitans and ependyma, 2 CSF-brain borders. Increased expression of CgA and CLU in reactive astrocytes in MS white matter lesions supports a role for these molecules as neuro-inflammatory mediators and their potential as CSF markers of active pathological processes in MS patients.","PeriodicalId":16434,"journal":{"name":"Journal of Neuropathology & Experimental Neurology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2016-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"84022727","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 19

Meningiomas With Rhabdoid Features Lacking Other Histologic Features of Malignancy: A Study of 44 Cases and Review of the Literature. 缺乏其他恶性肿瘤组织学特征的横纹肌瘤：44 个病例的研究和文献综述。

Journal of Neuropathology & Experimental Neurology Pub Date : 2016-01-01 Epub Date: 2015-12-07 DOI: 10.1093/jnen/nlv006

Rachael A Vaubel, Selby G Chen, David R Raleigh, Michael J Link, Michael R Chicoine, Igor Barani, Sarah M Jenkins, Patrice Abell Aleff, Fausto J Rodriguez, Peter C Burger, Sonika Dahiya, Arie Perry, Caterina Giannini

{"title":"Meningiomas With Rhabdoid Features Lacking Other Histologic Features of Malignancy: A Study of 44 Cases and Review of the Literature.","authors":"Rachael A Vaubel, Selby G Chen, David R Raleigh, Michael J Link, Michael R Chicoine, Igor Barani, Sarah M Jenkins, Patrice Abell Aleff, Fausto J Rodriguez, Peter C Burger, Sonika Dahiya, Arie Perry, Caterina Giannini","doi":"10.1093/jnen/nlv006","DOIUrl":"10.1093/jnen/nlv006","url":null,"abstract":"The behavior of rhabdoid meningiomas otherwise lacking malignant features remains unknown as most of the originally reported aggressive cases showed anaplastic histologic features independently of rhabdoid phenotype. We studied 44 patients with rhabdoid meningiomas lacking anaplastic features. Median age at diagnosis was 48.6 years (range 10-79). Location was supratentorial in 28 (63.6%), skull base in 15 (34.1%), and spinal in 1 (2.3%). Tumor grade was otherwise World Health Organization grade I (n = 22, 50%) or II (n = 22, 50%). Rhabdoid cells represented <20% of the tumor in 12 cases (27.3%), 20% to 50% in 18 (40.9%), and >50% in 14 (31.8%). Median clinical follow-up, available for 38 patients, was 5.0 years (range 0.17-14.2). Recurrence occurred in 9 patients (5-year recurrence-free survival, 73.7%) with a significantly higher risk in subtotally resected tumors (p = 0.043). Rhabdoid cell percentage was not associated with recurrence. Six patients died (4 of disease, 2 of unclear causes); 5-year overall survival was 86.7%, a mortality in excess of that expected in grade I-II meningiomas but much lower than originally reported. Review of 50 similar previously reported cases confirmed our findings. We suggest that rhabdoid meningiomas be graded analogously to nonrhabdoid tumors, with caution that some may still behave aggressively and close follow-up is recommended. ","PeriodicalId":16434,"journal":{"name":"Journal of Neuropathology & Experimental Neurology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2016-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5009417/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"86430045","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

The Diagnostic Use of Immunohistochemical Surrogates for Signature Molecular Genetic Alterations in Gliomas 免疫组织化学替代物在胶质瘤中诊断特征分子遗传改变的应用

Journal of Neuropathology & Experimental Neurology Pub Date : 2016-01-01 DOI: 10.1093/jnen/nlv009

J. Tanboon, E. Williams, D. Louis

引用次数: 82

Simultaneous Quantification of Unmyelinated Nerve Fibers in Sural Nerve and in Skin 同时定量测定腓肠神经和皮肤无髓神经纤维

Journal of Neuropathology & Experimental Neurology Pub Date : 2016-01-01 DOI: 10.1093/jnen/nlv005

M. Duchesne, L. Magy, L. Richard, P. Ingrand, J. Neau, S. Mathis, J. Vallat

{"title":"Simultaneous Quantification of Unmyelinated Nerve Fibers in Sural Nerve and in Skin","authors":"M. Duchesne, L. Magy, L. Richard, P. Ingrand, J. Neau, S. Mathis, J. Vallat","doi":"10.1093/jnen/nlv005","DOIUrl":"https://doi.org/10.1093/jnen/nlv005","url":null,"abstract":"Peripheral polyneuropathies are common and their diagnosis may be challenging. We compared the results from sural-nerve and skin biopsies in 33 patients with a polyneuropathy and neuropathic pain examined in our hospital over a 6-year period. The biopsies were all from the same lower limb of each patient. Intraepidermal nerve fiber (IENF) densities in the skin were determined by fluorescence microscopy; unmyelinated fiber densities in sural-nerve biopsies (UFNB) were determined by electron microscopy. There was no correlation with age or gender in either biopsy type; there was a weak trend to correlation between UFNB density and IENF density, possibly because of the small sample size. The sensitivity of detection of quantitative abnormalities of unmyelinated fibers was better in the skin than in the nerves. Proximal and distal IENF densities were strongly correlated; and counts of UFNB were highly reproducible. Thus, quantification of unmyelinated fibers in sural-nerve and skin biopsies seem to be complementary. Sural-nerve biopsy may be required to confirm a specific diagnosis, to identify lesion mechanisms, and to devise therapeutic strategies, whereas skin biopsy seems to be more efficient in the follow-up of length-dependent polyneuropathies and in the diagnosis of neuropathic pain.","PeriodicalId":16434,"journal":{"name":"Journal of Neuropathology & Experimental Neurology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2016-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"87571962","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 10

Increased Expression and Cellular Localization of P2X7R in Cortical Lesions of Patients With Focal Cortical Dysplasia P2X7R在局灶性皮质发育不良患者皮质病变中的表达和细胞定位增加

Journal of Neuropathology & Experimental Neurology Pub Date : 2016-01-01 DOI: 10.1093/jnen/nlv003

Yujia Wei, W. Guo, Fei-Ji Sun, W. Fu, Dahai Zheng, Xin Chen, Song Li, Zhen-le Zang, Chun-Qing Zhang, Shiyong Liu, Hui Yang

{"title":"Increased Expression and Cellular Localization of P2X7R in Cortical Lesions of Patients With Focal Cortical Dysplasia","authors":"Yujia Wei, W. Guo, Fei-Ji Sun, W. Fu, Dahai Zheng, Xin Chen, Song Li, Zhen-le Zang, Chun-Qing Zhang, Shiyong Liu, Hui Yang","doi":"10.1093/jnen/nlv003","DOIUrl":"https://doi.org/10.1093/jnen/nlv003","url":null,"abstract":"Focal cortical dysplasias (FCDs) are major brain malformations that commonly lead to medically intractable epilepsy. The purinergic ionotropic P2X7 receptor (P2X7R) is an atypical P2X subtype that gates calcium and sodium ions. Previous animal studies have suggested that P2X7R is a contributing factor in epileptogenesis. This study aimed to define the distribution and expression of P2X7R in 35 FCD patient-surgical-resection specimens relative to autopsy control samples (n = 8). Immunohistochemical colocalization assays revealed that P2X7R was primarily expressed in neurons, astrocytes, and microglia. In FCD samples, P2X7R protein levels were increased in abnormal cell types such as dysmorphic neurons and balloon cells, which are characteristic of FCD. By real-time PCR and Western blotting, P2X7R mRNA and protein expression levels were elevated in FCD patient samples vs control samples; P2X7R expression was also higher in FCDII vs FCDIa patient samples. Because interleukin-1&bgr; is a downstream factor of the P2X7R signaling pathway, we determined that there was also moderate-to-strong interleukin-1&bgr; expression in the dysmorphic neurons, balloon cells, and microglia in FCD patient lesions. These results indicate that increasing P2X7R levels may contribute to the pathogenesis of human FCD and that P2X7R represents a potential anti-epileptogenic target.","PeriodicalId":16434,"journal":{"name":"Journal of Neuropathology & Experimental Neurology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2016-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"91551913","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 11

Characterization of Early Pathological Tau Conformations and Phosphorylation in Chronic Traumatic Encephalopathy 慢性创伤性脑病早期病理性Tau构象和磷酸化的特征

Journal of Neuropathology & Experimental Neurology Pub Date : 2016-01-01 DOI: 10.1093/jnen/nlv001

N. Kanaan, K. Cox, Victor E. Alvarez, T. Stein, Sharra Poncil, A. Mckee

{"title":"Characterization of Early Pathological Tau Conformations and Phosphorylation in Chronic Traumatic Encephalopathy","authors":"N. Kanaan, K. Cox, Victor E. Alvarez, T. Stein, Sharra Poncil, A. Mckee","doi":"10.1093/jnen/nlv001","DOIUrl":"https://doi.org/10.1093/jnen/nlv001","url":null,"abstract":"Chronic traumatic encephalopathy (CTE) is a neurodegenerative tauopathy that develops after repetitive head injury. Several lines of evidence in other tauopathies suggest that tau oligomer formation induces neurotoxicity and that tau oligomer-mediated neurotoxicity involves induction of axonal dysfunction through exposure of an N-terminal motif in tau, the phosphatase-activating domain (PAD). Additionally, phosphorylation at serine 422 in tau occurs early and correlates with cognitive decline in patients with Alzheimer disease (AD). We performed immunohistochemistry and immunofluorescence on fixed brain sections and biochemical analysis of fresh brain extracts to characterize the presence of PAD-exposed tau (TNT1 antibody), tau oligomers (TOC1 antibody), tau phosphorylated at S422 (pS422 antibody), and tau truncated at D421 (TauC3 antibody) in the brains of 9-11 cases with CTE and cases of nondemented aged controls and AD (Braak VI) (n = 6, each). All 3 early tau markers (ie, TNT1, TOC1, and pS422) were present in CTE and displayed extensive colocalization in perivascular tau lesions that are considered diagnostic for CTE. Notably, the TauC3 epitope, which is abundant in AD, was relatively sparse in CTE. Together, these results provide the first description of PAD exposure, TOC1 reactive oligomers, phosphorylation of S422, and TauC3 truncation in the tau pathology of CTE.","PeriodicalId":16434,"journal":{"name":"Journal of Neuropathology & Experimental Neurology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2016-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"78690855","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 86

Neuropathological Characteristics of Brachial Plexus Avulsion Injury With and Without Concomitant Spinal Cord Injury. 伴有或不伴有脊髓损伤的臂丛神经撕脱伤的神经病理学特征

Journal of Neuropathology & Experimental Neurology Pub Date : 2016-01-01 DOI: 10.1093/jnen/nlv002

Zarina S Ali, Victoria E Johnson, William Stewart, Eric L Zager, Rui Xiao, Gregory G Heuer, Maura T Weber, Arka N Mallela, Douglas H Smith

{"title":"Neuropathological Characteristics of Brachial Plexus Avulsion Injury With and Without Concomitant Spinal Cord Injury.","authors":"Zarina S Ali, Victoria E Johnson, William Stewart, Eric L Zager, Rui Xiao, Gregory G Heuer, Maura T Weber, Arka N Mallela, Douglas H Smith","doi":"10.1093/jnen/nlv002","DOIUrl":"10.1093/jnen/nlv002","url":null,"abstract":"Neonatal brachial plexus avulsion injury (BPAI) commonly occurs as a consequence of birth trauma and can result in lifetime morbidity; however, little is known regarding the evolving neuropathological processes it induces. In particular, mechanical forces during BPAI can concomittantly damage the spinal cord and may contribute to outcome. Here, we describe the functional and neuropathological outcome following BPAI, with or without spinal cord injury, in a novel pediatric animal model. Twenty-eight-day-old piglets underwent unilateral C5–C7 BPAI with and without limited myelotomy. Following avulsion, all animals demonstrated right forelimb monoparesis. Injury extending into the spinal cord conferred greater motor deficit, including long tract signs. Consistent with clinical observations, avulsion with myelotomy resulted in more severe neuropathological changes with greater motor neuron death, progressive axonopathy, and persistent glial activation. These data demonstrate neuropathological features of BPAI associated with poor functional outcome. Interestingly, in contrast to adult small animal models of BPAI, a degree of motor neuron survival was observed, even following severe injury in this neonatal model. If this is also the case in human neonatal BPAI, repair may permit functional restoration. This model also provides a clinically relevant platform for exploring the complex postavulsion neuropathological responses that may inform therapeutic strategies.","PeriodicalId":16434,"journal":{"name":"Journal of Neuropathology & Experimental Neurology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2016-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6322589/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"84409080","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

Transmission of Soluble and Insoluble &agr;-Synuclein to Mice 可溶性和不溶性&agr;-Synuclein在小鼠体内的传递

Journal of Neuropathology & Experimental Neurology Pub Date : 2015-12-01 DOI: 10.1097/NEN.0000000000000262

D. Jones, M. Delenclos, Ann-Marie T Baine, M. DeTure, M. Murray, D. Dickson, P. McLean

{"title":"Transmission of Soluble and Insoluble &agr;-Synuclein to Mice","authors":"D. Jones, M. Delenclos, Ann-Marie T Baine, M. DeTure, M. Murray, D. Dickson, P. McLean","doi":"10.1097/NEN.0000000000000262","DOIUrl":"https://doi.org/10.1097/NEN.0000000000000262","url":null,"abstract":"Abstract The neurodegenerative synucleinopathies, which include Parkinson disease, multiple-system atrophy, and Lewy body disease, are characterized by the presence of abundant neuronal inclusions called Lewy bodies and Lewy neurites. These disorders remain incurable, and a greater understanding of the pathologic processes is needed for effective treatment strategies to be developed. Recent data suggest that pathogenic misfolding of the presynaptic protein, &agr;-synuclein (&agr;-syn), and subsequent aggregation and accumulation are fundamental to the disease process. It is hypothesized that the misfolded isoform is able to induce misfolding of normal endogenous &agr;-syn, much like what occurs in the prion diseases. Recent work highlighting the seeding effect of pathogenic &agr;-syn has largely focused on the detergent-insoluble species of the protein. In this study, we performed intracerebral inoculations of the sarkosyl-insoluble or sarkosyl-soluble fractions of human Lewy body disease brain homogenate and show that both fractions induce CNS pathology in mice at 4 months after injection. Disease-associated deposits accumulated both near and distal to the site of the injection, suggesting a cell-to-cell spread via recruitment of &agr;-syn. These results provide further insight into the prion-like mechanisms of &agr;-syn and suggest that disease-associated &agr;-syn is not homogeneous within a single patient but might exist in both soluble and insoluble isoforms.","PeriodicalId":16434,"journal":{"name":"Journal of Neuropathology & Experimental Neurology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2015-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"78022501","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 31

A New Mouse Model of Limb-Girdle Muscular Dystrophy Type 2I Homozygous for the Common L276I Mutation Mimicking the Mild Phenotype in Humans 模拟人类轻度表型的常见L276I突变的2I型四肢带状肌营养不良小鼠纯合模型

Journal of Neuropathology & Experimental Neurology Pub Date : 2015-12-01 DOI: 10.1097/NEN.0000000000000260

T. Krag, J. Vissing

{"title":"A New Mouse Model of Limb-Girdle Muscular Dystrophy Type 2I Homozygous for the Common L276I Mutation Mimicking the Mild Phenotype in Humans","authors":"T. Krag, J. Vissing","doi":"10.1097/NEN.0000000000000260","DOIUrl":"https://doi.org/10.1097/NEN.0000000000000260","url":null,"abstract":"Abstract Limb-girdle muscular dystrophy type 2I (LGMD2I) is caused by mutations in the Fukutin-related protein (FKRP) gene, leading to inadequate glycosylation of &agr;-dystroglycan, an important protein linking the extracellular matrix to the cytoskeleton. We created a mouse model of the common FKRP L276I mutation and a hemizygous FKRP L276I knockout model. We studied histopathology and protein expression in the models at different ages and found that homozygous FKRP L276I mice developed a mild progressive myopathy with increased muscle regeneration and fibrosis starting from 1 year of age. This was likely caused by progressive loss of &agr;-dystroglycan–specific glycosylation, which was decreased by 78% at 20 months. The homozygous FKRP knockout was embryonic lethal, but the hemizygous L276I model resembled the homozygous FKRP L276I model at comparable ages. These models emphasize the importance of FKRP in maintaining proper glycosylation of &agr;-dystroglycan. The mild progression in the homozygous FKRP L276I model resembles that in patients with LGMD2I who are homozygous for the L276I mutation. This animal model could, therefore, be relevant for understanding the pathophysiology of and developing a treatment strategy for the human disorder.","PeriodicalId":16434,"journal":{"name":"Journal of Neuropathology & Experimental Neurology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2015-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"91472506","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 19

Primary Central Nervous System Histiocytic Sarcoma Arising After Precursor B-Cell Acute Lymphoblastic Leukemia 前体b细胞急性淋巴母细胞白血病后发生的原发性中枢神经系统组织细胞肉瘤

Journal of Neuropathology & Experimental Neurology Pub Date : 2015-12-01 DOI: 10.1097/NEN.0000000000000258

Alan F Brown, H. Fan, J. Floyd, J. Henry, Russell A Higgins

{"title":"Primary Central Nervous System Histiocytic Sarcoma Arising After Precursor B-Cell Acute Lymphoblastic Leukemia","authors":"Alan F Brown, H. Fan, J. Floyd, J. Henry, Russell A Higgins","doi":"10.1097/NEN.0000000000000258","DOIUrl":"https://doi.org/10.1097/NEN.0000000000000258","url":null,"abstract":"Abstract Histiocytic sarcomas (HSs) are rare malignant neoplasms derived from histiocytes that may be associated with other hematolymphoid neoplasms. Histiocytic sarcomas rarely occur in the CNS and have not previously been reported in conjunction with prior B-cell lymphoblastic leukemia. We report the case of a 23-year-old man who presented with primary CNS HS 7 years after achieving remission for precursor B-cell acute lymphoblastic leukemia (B-ALL). Molecular studies revealed clonal immunoglobulin heavy-chain (IGH) gene rearrangement within the HS, suggesting linkage to his previous B-ALL. Previously reported post-ALL HSs show a strong predilection for young males (male-to-female ratio, 20:1), whereas cases of primary CNS HS without previous ALL affected older adults with balanced sex predilection. The patient's survival at 60 months exceeds expectations when compared with that of other reported cases of de novo primary CNS HS (n = 18) and post-ALL HS at all sites (n = 19). In addition, we discuss the potential relationship between B-ALL and HS posed by other authors.","PeriodicalId":16434,"journal":{"name":"Journal of Neuropathology & Experimental Neurology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2015-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"76680244","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 11