Emerging glioneuronal and neuronal tumors: case-based review
AbstractGlioneuronal and neuronal tumors (GNTs) are rare heterogeneous central nervous system tumors characterized by slow growth and favorable outcomes, but are often associated with diagnostic difficulties. A thorough analysis of three rare and recently recognized GNTs was performed in the context of clinicopathological features and molecular genetic characterization. The current spinal diffuse leptomeningeal glioneuronal tumor (DLGNT) was characterized with oligodendroglioma-like tumor with chromosome 1p/19q codeletion withoutIDH mutations andKIAA1549:BRAF fusion. The current occipital multinodular and vacuolating neuro...
Source: Brain Tumor Pathology - January 20, 2022 Category: Neurology Source Type: research

Histopathological predictors of progression-free survival in atypical meningioma: a single-center retrospective cohort and meta-analysis
AbstractTo determine the prognostic significance of histopathological features included in the diagnostic criteria of atypical meningioma for progression-free survival (PFS). We performed a retrospective cohort study and meta-analysis. Brain invasion, mitotic index, spontaneous necrosis, sheeting, prominent nucleoli, high cellularity, and small cells were the histopathological features of interest. The data from 25 studies involving 3590 patients including our cohort (n = 262) were included. The pooled HR of mitotic index at a cutoff value of 4 showed no statical significance in the gross analysis (pooled HR, 1.09; 95%...
Source: Brain Tumor Pathology - January 15, 2022 Category: Neurology Source Type: research

A patient with two gliomas with independent oligodendroglioma and glioblastoma biology proved by DNA-methylation profiling: a case report and review of the literature
AbstractHere, we report on a patient presenting with two histopathologically distinct gliomas. At the age of 42, the patient underwent initial resection of a right temporal oligodendroglioma IDH mutated 1p/19q co-deleted WHO Grade II followed by adjuvant radiochemotherapy with temozolomide. 15  months after initial diagnosis, the patient showed right hemispheric tumor progression and an additional new left frontal contrast enhancement in the subsequent imaging. A re-resection of the right-sided tumor and resection of the left frontal tumor were conducted. Neuropathological work-up showed recurrence of the right-sided olig...
Source: Brain Tumor Pathology - January 11, 2022 Category: Neurology Source Type: research

Role of proliferative marker index and KBTBD4 mutation in the pathological diagnosis of pineal parenchymal tumors
This study included 19 cases of PPTs [3 pineocytomas (PCs), 10 PPTs of intermediate differentiation (PPTID), and 6 pineoblastomas (PBs)]. Immunohistochemistry for Ki-67, PHH3, and DICER1, as well as Sanger sequencing analysis forKBTBD4 mutations, was performed using formalin-fixed paraffin-embedded tissue specimens that were resected during surgery. Tumor cell proliferation was quantified using an image analysis software. For the PHH3 and MIB-1 indices, a significant difference was observed between the PPTIDs and PBs (P <  0.05). Loss of DICER1 was not specific for PB; 0/3 PCs (0.0%), 2/9 PPTIDs (22.2%), and 2/4 PBs...
Source: Brain Tumor Pathology - January 9, 2022 Category: Neurology Source Type: research

Timing of H3K27me3 loss in secondary anaplastic meningiomas
(Source: Brain Tumor Pathology)
Source: Brain Tumor Pathology - January 6, 2022 Category: Neurology Source Type: research

Correction to: Epithelioid inflammatory myofibroblastic sarcoma with VCL –ALK fusion of central nervous system: case report and brief review of the literature
(Source: Brain Tumor Pathology)
Source: Brain Tumor Pathology - January 1, 2022 Category: Neurology Source Type: research

Reliability of IDH1-R132H and ATRX and/or p53 immunohistochemistry for molecular subclassification of Grade 2/3 gliomas
We report the associations between histological findings, IHC, and genetic status. We performed IHC of IDH1-R132H, ATRX, and p53 in 76 lower-grade gliomas and discussed its validity based on the 2016 WHO and the upcoming 2021 WHO classification. The sensitivity and specificity of anti-ATRX, p53, and IDH1-R132H IHC were 40.9%/98.1%, 78.6%/85.4%, and 90.5%/84.6%, respectively. Among 21IDH1-mutant gliomas without 1p/19q codeletion, two gliomas (9.5%) mimicked the so-called classic for oligodendroglioma (CFO) in their morphology. Of the 42 gliomas with 1p/19q codeletion, four cases were difficult to diagnose as oligodendroglio...
Source: Brain Tumor Pathology - November 26, 2021 Category: Neurology Source Type: research

Molecular subtyping of ependymoma and prognostic impact of Ki-67
In this study, we propose a cost-effective schematic diagnostic flow of EPNs by the anatomical location, three biomarkers (L1CAM, H3K27me3, and EZHIP), and a cut-off of a 7% Ki-67 labeling index. (Source: Brain Tumor Pathology)
Source: Brain Tumor Pathology - November 23, 2021 Category: Neurology Source Type: research

Central nervous system ALK-negative anaplastic large cell lymphoma with IRF4/DUSP22 rearrangement
We report what may be the first case of ALK-negative ALCL withIRF4/DUSP22 rearrangement involving the brain in a 55-year-old man. Magnetic resonance imaging demonstrated signal abnormalities in the periventricular region, corpus callosum and cingulate gyrus. Biopsy revealed a diffuse parenchymal and angiocentric infiltrate of CD30-positive cells that showedIRF4/DUSP22 rearrangement by fluorescence in situ hybridization. We also review the clinical and pathologic features of primary CNS ALK-negative ALCLs in the literature and highlight the need for awareness of this entity to optimize appropriate management. (Source: Brain Tumor Pathology)
Source: Brain Tumor Pathology - November 18, 2021 Category: Neurology Source Type: research

Epithelioid inflammatory myofibroblastic sarcoma with VCL –ALK fusion of central nervous system: case report and brief review of the literature
AbstractEpithelioid inflammatory myofibroblastic sarcomas are an aggressive variant of inflammatory myofibroblastic tumor described primarily in the abdomen and less commonly in pulmonary location. The anaplastic lymphoma kinase (ALK) fusion partners described in this tumor includeRANB2, RRBP1and EML4. While rare examples of inflammatory myofibroblastic tumors have been described in the central nervous system, the epithelioid variant has never been described. TheALK –VCL fusion has been described in renal cell carcinoma, high-grade glioma and epithelioid fibrous histiocytoma but has not been described in epithelioid infl...
Source: Brain Tumor Pathology - November 6, 2021 Category: Neurology Source Type: research

Insights in primary central nervous system lymphoma: a role for glymphatics?
(Source: Brain Tumor Pathology)
Source: Brain Tumor Pathology - September 17, 2021 Category: Neurology Source Type: research

Revisiting vimentin: a negative surrogate marker of molecularly defined oligodendroglioma in adult type diffuse glioma
AbstractVimentin is a marker of epithelial-mesenchymal transformation and indicates poor prognosis in various cancers, but its role in diffuse gliomas remains unknown. We investigated the vimentin expression of diffuse gliomas according to the upcoming 2021 WHO classification, its variations due to mutational status, and its prognostic effects. We analyzed vimentin immunohistochemistry in 315 gliomas: a test set (n = 164) and a validation set (n = 151). RNA-seq and mutational information from The Cancer Genome Atlas (TCGA,n = 422) were also used for validation. Vimentin was diffusely positive in astrocytic tumo...
Source: Brain Tumor Pathology - August 2, 2021 Category: Neurology Source Type: research

Spinal cord astroblastoma with EWSR1-BEND2 fusion classified as HGNET-MN1 by methylation classification: a case report
AbstractThe most recurrent fusion of central nervous system high-grade neuroepithelial tumor withMN1 alteration (HGNET-MN1) isMN1 rearrangement. Here, we report the case of a 36-year-old man with spinal cord astroblastoma showingEwing Sarcoma breakpoint region 1/EWS RNA-binding protein 1 (EWSR1)-BEN domain-containing 2 (BEND2) fusion. The patient presented with back pain, gait disturbance and dysesthesia in the lower extremities and trunk. Magnetic resonance imaging showed an intramedullary tumor at the T3 –5 level, displaying homogeneous gadolinium enhancement. Partial tumor removal was performed with laminectomy. Histo...
Source: Brain Tumor Pathology - July 27, 2021 Category: Neurology Source Type: research

Preface
(Source: Brain Tumor Pathology)
Source: Brain Tumor Pathology - July 23, 2021 Category: Neurology Source Type: research

Association between IDH mutational status and tumor-associated epilepsy or venous thromboembolism in patients with grade II and III astrocytoma
This study demonstrated that the IDH mutational status was associated with TAE but not with VTE. Therefore, a future large-scale study is needed to provide sufficient evidence. TA E was more common in young patients, while VTE was more common in the elderly. (Source: Brain Tumor Pathology)
Source: Brain Tumor Pathology - July 16, 2021 Category: Neurology Source Type: research