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The World Health Organization's 5th edition classification of Central Nervous System (CNS) tumors differentiates diffuse gliomas into adult and pediatric variants. Pediatric-type diffuse low-grade gliomas (pDLGGs) are distinct from adult gliomas in their molecular characteristics, biological behavior, clinical progression, and prognosis. Various molecular alterations identified in pDLGGs are crucial for treatment. There are four distinct entities of pDLGGs. All four of these tumor subtypes exhibit diffuse growth and share overlapping histopathological and imaging characteristics. Molecular analysis is essential for differentiating these lesions. [ABSTRACT FROM AUTHOR]

Background: Angiocentric glioma (AG) is a rare, benign, and slow-growing tumor. First described in 2005, it is now gaining attention with respect to the possibility of being diagnosed. Even with no statistical differences between sex, it has been reported both in children and the elderly. A total of 120 cases have been described in the literature. The aim of this study is to provide new data for a new statistical assessment of the prevalence and incidence of AG in populations. Case report: An 8-year-old male patient with no history of epilepsy and no need for antiepileptic therapy underwent surgery for a left-parietal brain lesion, revealed through MRI. Imaging was acquired after his first absence episode. The lesion was completely resected. Histological findings indicated angiocentric glioma. No signs of recurrency after two years of follow-up. Conclusion: AG is usually an epilepsy-related low-grade glioma. Few cases exhibit disease progression and exitus. Surgical management should aim for a gross total resection to avoid recurrence and persisting epilepsy. Surgery represents the gold standard in diagnosis and treatment and must be performed as soon as possible in consideration of its healing properties and its useful diagnosis.

Background: Angiocentric glioma (AG) is a rare, benign, and slow-growing tumor. First described in 2005, it is now gaining attention with respect to the possibility of being diagnosed. Even with no statistical differences between sex, it has been reported both in children and the elderly. A total of 120 cases have been described in the literature. The aim of this study is to provide new data for a new statistical assessment of the prevalence and incidence of AG in populations. Case report: An 8-year-old male patient with no history of epilepsy and no need for antiepileptic therapy underwent surgery for a left-parietal brain lesion, revealed through MRI. Imaging was acquired after his first absence episode. The lesion was completely resected. Histological findings indicated angiocentric glioma. No signs of recurrency after two years of follow-up. Conclusion: AG is usually an epilepsy-related low-grade glioma. Few cases exhibit disease progression and exitus. Surgical management should aim for a gross total resection to avoid recurrence and persisting epilepsy. Surgery represents the gold standard in diagnosis and treatment and must be performed as soon as possible in consideration of its healing properties and its useful diagnosis. [ABSTRACT FROM AUTHOR]

Images were taken at 100 × magnification and the scale bars represent 200 µm Differential diagnoses included high-grade gliomas, astrocytoma, oligodendroglioma, diffuse pediatric-type high-grade glioma, H3-wildtype and IDH-wildtype, diffuse hemispheric glioma, H3 G34-mutant, and some rare neuroectodermal tumors with a diffuse growth pattern. Keywords: Oligodendroglioma; Pleomorphic xanthoastrocytoma; Ganglioglioma; Angiocentric glioma; Polymorphous low-grade neuroepithelial tumor of the young EN Oligodendroglioma Pleomorphic xanthoastrocytoma Ganglioglioma Angiocentric glioma Polymorphous low-grade neuroepithelial tumor of the young 869 876 8 08/28/23 20230901 NES 230901 The authors C. Storz and R. Sankowski contributed equally to the manuscript. Pleomorphic Xanthoastrocytoma Pleomorphic xanthoastrocytomas (PXA) are rare astrocytic tumors, classified as WHO CNS grade II-III tumors, depending on the histological features [[1]]. Dysembryoplastic Neuroepithelial Tumor Dysembryoplastic neuroepithelial tumors (DNET) are slowly growing, well-circumscribed, cortically based tumors, classified as CNS WHO grade I neoplasms [[1], [7]]. [Extracted from the article]

Objectives This study aims to evaluate the subgroup consisting of uncommon pathological entities presenting with temporal lobe epilepsy (TLE). Methodology Twenty-four consecutively operated patients of lesional temporal lobe epilepsy were included in the study. Eleven cases were identified that had pathologies implicated as “uncommon” in previously done studies on the pathology of TLE. A subgroup analysis consisting of these uncommon lesions was done for clinical presentation and seizure outcome. The seizure outcome was assessed in terms of Engel and International League Against Epilepsy (ILAE) classification. Results Nine different pathological entities were identified within the study group. The mean age at surgery was 18.6 years (range 2–30 years). The mean duration of epilepsy was 48.3 months and the average duration of follow-up was 39.2months. All patients had seizures as the only complaint except three. Three patients had focal-aware seizures, two had focal motor onset, and the rest all had focal-unaware seizures. Seven patients were seizure free and the average age at diagnosis for these patients was 15.4 years (range 2–24 years.). The duration of seizure in the postoperative seizure-free group was 29.7 months and it was 81 months for the other group. All the patients with persistent postoperative seizures had focal-unaware seizures preoperatively. Conclusion Despite the small and heterogeneous nature of this subgroup of uncommon lesions causing temporal lobe epilepsy, the clinical presentation, prognostic factors, and seizure outcome are similar to the cohort of common pathologies, including mesial temporal sclerosis.

Introduction: Angiocentric gliomas (AG) in brainstem location are exceedingly rare and might cause differential diagnostic problems and uncertainty regarding the best therapeutic approach. Hereby, we describe the clinicopathological findings in a brainstem AG presenting in a toddler child and review the literature.Case report: A 2-year-old boy presented with 5 weeks history of gait disturbances, frequent falls, left-sided torticollis and swallowing problems. MRI head showed a T2-hyperintense, partly exophytic mass lesion centred in the pontomedullary region, raising the possibility of diffuse midline glioma. The exophytic component was partially resected by suboccipital craniotomy, leaving intact the infiltrative component. Ventriculoperitoneal shunt was implanted due to postoperative hydrocephalus. Histological examination revealed a moderately cellular tumour consisted of bland glial cells infiltrating the brain parenchyma and radially arranged around the blood vessels. By immunohistochemistry, the tumour strongly expressed S100 and GFAP in addition to intense nestin positivity, while OLIG2 was negative in the perivascular tumour cells. DNA methylation array profiled the tumour as “methylation class diffuse astrocytoma, MYB or MYBL1-altered subtype B (infratentorial)” and an in-frame MYB::QKI fusion was identified by RNA sequencing, confirming the diagnosis of angiocentric glioma. The patient has been initially treated with angiogenesis inhibitor and mTOR inhibitor, and now he is receiving palliative vinblastine. He is clinically stable on 9 months follow-up.Conclusion: Brainstem AG may cause a diagnostic problem, and the surgical and oncological management is challenging due to unresectability and lack of response to conventional chemo-radiation. In the future, genetically-tailored therapies might improve the prognosis.

Objectives  This study aims to evaluate the subgroup consisting of uncommon pathological entities presenting with temporal lobe epilepsy (TLE). Methodology  Twenty-four consecutively operated patients of lesional temporal lobe epilepsy were included in the study. Eleven cases were identified that had pathologies implicated as "uncommon" in previously done studies on the pathology of TLE. A subgroup analysis consisting of these uncommon lesions was done for clinical presentation and seizure outcome. The seizure outcome was assessed in terms of Engel and International League Against Epilepsy (ILAE) classification. Results  Nine different pathological entities were identified within the study group. The mean age at surgery was 18.6 years (range 2–30 years). The mean duration of epilepsy was 48.3 months and the average duration of follow-up was 39.2months. All patients had seizures as the only complaint except three. Three patients had focal-aware seizures, two had focal motor onset, and the rest all had focal-unaware seizures. Seven patients were seizure free and the average age at diagnosis for these patients was 15.4 years (range 2–24 years.). The duration of seizure in the postoperative seizure-free group was 29.7 months and it was 81 months for the other group. All the patients with persistent postoperative seizures had focal-unaware seizures preoperatively. Conclusion  Despite the small and heterogeneous nature of this subgroup of uncommon lesions causing temporal lobe epilepsy, the clinical presentation, prognostic factors, and seizure outcome are similar to the cohort of common pathologies, including mesial temporal sclerosis. [ABSTRACT FROM AUTHOR]

Background and Purpose: Angiocentric gliomas (AGs) are epileptogenic low‐grade gliomas in young patients. We aimed to investigate the MRI findings of AGs and systematically review previous publications and three new cases. Methods: We searched PubMed, Elsevier's abstract and citation database, and Embase databases and included 50 patients with pathologically proven AGs with analyzable preoperative MRI including 3 patients from our institution and 47 patients from 38 publications (median age, 13 years [range, 2‐83 years]; 35 men). Two board‐certified radiologists reviewed all images. The relationships between seizure/epilepsy history and MRI findings were statistically analyzed. Moreover, clinical and imaging differences were evaluated between supratentorial and brainstem AGs. Results: Intratumoral T1‐weighted high‐intensity areas, stalk‐like signs, and regional brain parenchymal atrophy were observed in 23 out of 50 (46.0%), 10 out of 50 (20.0%), and 14 out of 50 (28.0%) patients, respectively. Intratumoral T1‐weighted high‐intensity areas were observed significantly more frequently in patients with stalk‐like signs (positive, 9/10 vs. negative, 14/40, p =.0031) and regional atrophy (13/14 vs. 10/36, p =.0001). There were significant relationships between the length of seizure/epilepsy history and presence of intratumoral T1‐weighted high‐intensity area (median 3 years vs. 0.5 years, p =.0021), stalk‐like sign (13.5 vs. 1 year, p <.0001), and regional atrophy (14 vs. 0.5 years, p <.0001). Patients with brainstem AGs (n = 7) did not have a seizure/epilepsy history and were significantly younger than those with supratentorial AGs (median, 5 vs. 13 years, p <.0001, respectively). Conclusions: Intratumoral T1‐weighted high‐intensity areas, stalk‐like signs, and regional brain atrophy were frequent imaging features in AG. We also found that affected age was different between supratentorial and brainstem AGs. [ABSTRACT FROM AUTHOR]

We herein report a case of a 12-year-old girl who presented with a superficial mass in the right temporal lobe and a 7-month history of seizures. Intraoperative consultation with squash smear cytology was done. It showed low-grade glioma with slender, bipolar cells with angiocentric features. The surgical resection specimen revealed features of angiocentric glioma with extensive schwannoma-like areas and typical EMA “dot-like” positivity.

Keywords: angiocentric glioma; DNA methylation; epilepsy; glioma EN angiocentric glioma DNA methylation epilepsy glioma 1 3 3 01/16/23 20230101 NES 230101 CLINICAL HISTORY A 64-year-old woman with a 29-year history of drug resistant epilepsy presented to the NIH Clinical Center for surgical evaluation. UMAP clustering analysis of DNA methylation profile data classified this tumor as low-grade glioma (F). gl DNA methylation microarrays classifier analysis (V11) of this case indicated a match to low-grade glioma, MYB/MYBL1 (calibrated score 0.99). According to the 5th edition of the WHO international classification of human tumors, angiocentric glioma is classified under glioma, glioneuronal tumors and neuronal tumors, and is a subtype of pediatric-type diffuse low-grade glioma. [Extracted from the article]

Angiocentric glioma (AG) is a rare, low-grade glioma with slow growth. In 2007, AG was first classified as a solid tumor according to the WHO classification of the central nervous system (WHO class I). The outcome and prognosis of most of the cases are very good, but a few cases with tumor metastasis and disease progression, even death, have been reported. We report a case and systematically analyze previous literature to increase our understanding of the disease and determine the factors that may affect disease progression to make prognostic judgments. A young male patient complained of a 3-year history of epilepsy. Anti-epileptic drug treatment was ineffective. An imaging examination revealed a lesion in the left parietal cortex area. Thus, the lesion was completely resected. The pathological diagnosis was angiocentric glioma. During a follow-up of two years, the patient had epilepsy relief controlled by sodium valproate and a disease-free period. AG is an epilepsy-related low-grade glioma that heals after complete resection in most reported cases. However, few reported cases have had disease progression and death. This result may be due to the pathological complexity of the diseased tissue. In addition, AG is usually found to have an MYB–QKI rearrangement on genetic analysis. Due to the small number of reported cases and studies, our understanding and knowledge of this disease are still lacking. The potential malignant changes and prognostic factors need to be verified in more than clinical cases and basic research in the future. [ABSTRACT FROM AUTHOR]

The World Health Organization (WHO) Central Nervous System (CNS) Tumors Classification 5 th edition (2021) integrates both molecular and histopathological criteria for diagnosing glial tumors. This updated classification highlights significant differences between pediatric and adult gliomas in terms of molecular characteristics and prognostic implications. The 5 th edition comprises a new category of pediatric-type diffuse low-grade glioma (PDLGG) and pediatric-type diffuse high-grade glioma (PDHGG), classified mainly based on genetic alterations and histopathological features. We reviewed the microscopy, diagnostic molecular pathology, and prognosis of various tumors under the categories PDLGG and PDHGG. The review also addresses the need for clarification concerning overlapping diagnostic features. PDLGG are characterized by diffuse growth, low-grade morphology, and MYB/MYBL1(MYB Proto-Oncogene Like 1) gene fusion or mitogen-activated protein kinase (MAPK) pathway alterations. In contrast, PDHGG is described by diffuse growth, high-grade morphology, and increased mitosis and often shows alterations of histone gene resulting in epigenetic alterations, which contrasts with common isocitrate dehydrogenase (IDH) mutation and epidermal growth factor receptor (EGFR) amplification seen in adult-type high-grade glioma., Competing Interests: The authors declare no conflict of interest., (© 2024 The Author(s).)

Background: Angiocentric glioma (AG) is an extremely rare intracranial tumor that was first described in 2005 and identified as a special type of intracranial tumor in 2007 by the WHO, which mainly affects children and young adolescents. Epilepsy is the main presentation; therefore, it was recognized as a seizure-related tumor in the past. Here, we report a case of AG with acute intracerebral hemorrhage (ICH) as the first symptom who never had a seizure onset. Case Description: A 3-year-old girl with the right limb weakness was admitted to our hospital 4 h after onset in 2018. Computed tomography showed a hematoma of about 20 ml accompanied by a hyper/iso-dense spheroid lesion located in the sub-cortex of the left parietal lobe. Magnetic resonance image (MRI) showed signs of hypointense signal in T1, T2, and fluid-attenuated inversion recovery sequence, distinct enhancement of this tumefactive lesion in the contrast-enhanced sequence. Thus, the admission diagnosis was neoplasm with acute ICH. A gross total resection of the tumor was achieved by parietal craniotomy. The histopathological diagnosis was AG. No signs showed tumor recurrence after 36 months of follow-up. Conclusion: This is the sole case of AGs with acute intracranial hemorrhage as the first symptom without any kind of epilepsy by far. This case had unique MRI signs that were different from the previous description. This case enriches the clinical and radiological manifestations of AG and reveals that further investigations are needed to further understand AG. [ABSTRACT FROM AUTHOR]

Angiocentric glioma (AG) is a rare form of brain tumor characterized by cortical epileptogenic lesions that show angiocentric patterns upon histopathological examination. Cystoid degeneration is a rare radiological manifestation of this condition. We report a case involving the left eloquent cortical areas and review the clinical features of angiocentric glioma. A 10-year-old girl presented with a history of refractory drug-resistant epilepsy. Imaging revealed a cystic-solid neoplasm within the left frontal lobe. Surgical procedures were performed, and it was observed that the pathological manifestations were consistent with those of AG. Angiocentric glioma should be considered for diagnosis when the supratentorial cortical neoplasm contains a cystic component. The diagnosis of AG can be confirmed by histopathological examination. Gross total resection is the recommended strategy for controlling seizures elicited by this condition. [ABSTRACT FROM AUTHOR]

We herein report a case of a 12-year-old girl who presented with a superficial mass in the right temporal lobe and a 7-month history of seizures. Intraoperative consultation with squash smear cytology was done. It showed low-grade glioma with slender, bipolar cells with angiocentric features. The surgical resection specimen revealed features of angiocentric glioma with extensive schwannoma-like areas and typical EMA "dot-like" positivity. [ABSTRACT FROM AUTHOR]

Angiocentric glioma (AG) is a rare subtype of neuroepithelial tumor in children and young adults that commonly presents with seizures. To study the clinical characteristics, treatment and prognosis of patients with AG, the features of two cases of AG were described and 108 cases reported in the literature were assessed. The cases of the present study were two males aged 8 and 16 years, who mainly presented with seizures. MRI revealed superficial, non-enhanced lesions in the left temporal and right frontal lobe, respectively. The two patients underwent gross total resection (GTR) and remained seizure-free without neurological deficits after 3.5 and 2.5 years, respectively. Histopathological examination revealed that the tumors consisted of monomorphous cells that surrounded the blood vessels and neurons in the cerebral cortex, and formed concentric sleeves or pseudorosettes. Furthermore, immunostaining indicated that the diffuse infiltrative neoplastic cells were positive for glial fibrillary acidic protein and a dot-like pattern of epithelial membrane antigen was observed. AG mostly appeared similar to low-grade gliomas on MRI. GTR of the lesions was curative and radiation or chemotherapy were not required. AG typically has a favorable prognosis, with low mortality and incidence of disability. [ABSTRACT FROM AUTHOR]

Angiocentric glioma (AG) is an epileptogenic low grade (World Health Organization grade I) glial tumor with astrocytic and ependymal differentiation, most commonly affecting the pediatric and adolescent population. Despite its infiltrating histological growth kinetics, it is widely accepted that AG has a low potential for aggressive behavior. We present the case of a 42-year-old man who represents the first documented case of not only extracranial manifestation of AG, but also spinal metastatic dissemination. Our patient initially presented with a generalized tonic clonic seizure; following a biopsy, he was diagnosed with a low-grade supratentorial astrocytoma and subsequently received fractionated radiotherapy. He presented 10 months later with worsening dorsal column symptoms and was found to have a contrast-enhancing intradural extramedullary lesion that was surgically resected and histologically confirmed as an AG. Further research is required to examine the microenvironment and potential for malignant change in this tumor. [ABSTRACT FROM AUTHOR]

Background: Angiocentric glioma (AG) is a relatively uncommon clinico-pathological entity that presents in childhood. Angiocentric glioma displays various histopathological features which resemble cortical ependymoma, astroblastoma, and pilomyxoid astrocytoma and schwannoma. The astrocytes in angiocentric glioma appear peculiarly elongated, bipolar in shape, and characteristically arranged around blood vessels. They resemble radial glia and tanycytes morphologically. Unlike ependymomas, AG is a diffusely infiltrating lesion and perivascular processes are often much thicker than those in classic ependymomas. Case presentation: AG usually present clinically as seizures, often as medically intractable epilepsy. In the indexed case, apart from unusual presentation with features of raised intra-cranial tension, an unusual histological picture of a more cellular oligodendroglioma like component was also seen. Conclusion: The appropriate diagnosis is critical as AG is usually slowly growing and treatable by surgical excision alone. [ABSTRACT FROM AUTHOR]

Highlights from the article: Gangliogliomas are uncommon tumors of the central nervous system (CNS) predominantly seen in children and young patients presenting with temporal lobe epilepsy, as these tumors are the most common neoplastic cause of chronic temporal lobe epilepsy [[1]]. Initially, it remained unclear whether MVNTs were true neoplastic processes or malformed dysplastic lesions; however, according to the revised WHO classification of CNS tumors, these tumors have been recognized as low-grade mixed glial neuronal lesions since 2016 [[3], [11]]. Angiocentric gliomas, also known as angiocentric neuroepithelial tumor, are very rare neuroepithelial tumors with low proliferation rates, which are classified as WHO grade I neoplasms [[3], [13]]. Pilocytic astrocytoma, Ganglioglioma, Dysembryoplastic neuroepithelial tumors, Angiocentric glioma, Multinodular and vacuolating neuronal tumor, Hypersalivatory seizures.

Angiocentric glioma (AG), first described in 2005, was included as a distinct entity in the 2007 World Health Organization Classification of Tumors of the Central Nervous System. It is a very rare cerebrocortical tumor mainly affecting children and young adults with a history of intractable partial seizures. The histopathological features of this entity are perivascular arrangement of monomorphic, bipolar spindled cells with subpial aggregation of tumor cells and variable neuroparenchymal colonization. Of uncertain histogenesis, this is a stable/slowly growing tumor. Prognosis following total surgical resection is favorable. We describe an AG in a 16-year-old, intellectually disabled, male patient, with psychosis. This is a rare presentation with only one such case in literature. Patient's symptoms ameliorated following surgery. [ABSTRACT FROM AUTHOR]

Angiocentric features are uncommon in high-grade World Health Organisation (WHO) brain tumours, whilst they are typical for WHO grade I tumours, e.g. angiocentric gliomas. We present an unusual glial tumour that occurred in a 59-year-old man. The tumour had equivocal radiologic and histopathologic features, especially a characteristic angiocentric pattern, low-to-moderate Ki67, and dot-like epithelial membrane antigen expression. The tumour did not show features characteristic for glioblastoma; however, it recurred as glioblastoma four months later. Based on this case, we show that high-grade WHO brain tumours may show an angiocentric pattern typical for low-grade WHO brain tumours, such as angiocentric gliomas. [ABSTRACT FROM AUTHOR]

Angiocentric glioma (AG) is a low grade glioma, that was first described in 2002. Since this description, 83 patients with AG have been described, including ours. AG typically presents in childhood with medically refractory seizures that are cured with gross surgical resection. Whilst the natural history is that of a benign tumour, there have been reports of recurrence, transformation, and malignant features that suggest that AG is potentially malignant. We add to the literature a case of a 16-year-old girl who presented in May 2011 with a 3-month history of complex partial seizures, with MRI showing a T2-weighted hyperintense lesion in the left insula and inferior frontal lobe. This was confirmed on biopsy as AG and was followed with surveillance imaging. In April 2012, she presented with disease progression and underwent a left temporal lobectomy, with histology showing both AG and grade II astrocytoma. Adjuvant radiotherapy of 50 Gray in 28 fractions was administered. A small area of contrast enhancement appeared in the left parietal lobe in December 2012, which progressed over subsequent months. In June 2013, she underwent a near total excision, with histology showing anaplastic ependymoma. She received six cycles of adjuvant temozolamide. Despite this, the tumour continued to progress, with her seizure control deteriorating, and the development of a right hemiparesis. The patient died in January 2014, aged 19 years. [ABSTRACT FROM AUTHOR]

The MYB/MYBL1::QKI fusion induces the protooncogene, MYB, and deletes the tumor suppressor gene, QKI. MYB/MYBL1::QKI rearrangement was previously reported only in angiocentric glioma (AG) and diffuse low-grade glioma. This report compares 2 tumors containing the MYB/MYBL1::QKI fusion: a diffuse pediatric-type high-grade glioma (DPedHGG) in an 11-year-old boy and an AG in a 46-year-old woman. We used immunohistochemistry, next-generation sequencing, and methylation profiling to characterize each tumor and compare our findings to the literature on AG and tumors with the MYB/MYBL1::QKI rearrangement. Both tumors were astrocytic with angiocentric patterns. The MYB::QKI fusion-positive DPedHGG, which recurred once, was accompanied by TP53 mutation and amplification of CDK6 and KRAS, suggesting malignant transformation secondary to additional genetic aberrations. The second case was the adult AG with MYBL1::QKI fusion, which mimicked ependymoma based on histopathology and its dot- and ring-like epithelial membrane antigen positivity. Combined with a literature review, our results suggest that MYB/MYBL1 alterations are not limited to low-grade gliomas, including AG. AG is most common in the cerebra of children and adolescents but exceptional cases occur in adults and the acquisition of additional genetic mutations may contribute to high-grade glioma. These cases further demonstrate that molecular characteristics, morphologic features, and clinical context are essential for diagnosis., (© The Author(s) 2023. Published by Oxford University Press on behalf of American Association of Neuropathologists, Inc.)

Aims Angiocentric glioma ( AG) is a rare, slow-growing tumour of the central nervous system. It is often associated with refractory epilepsy and occurs most commonly in children and young adults. We herein report nine cases of AG, including four with atypical histological findings. Methods The clinical data and clinicopathological findings of nine cases with AG histological features were described. Results All nine patients had a history of refractory epilepsy with a mean history of 4.4 years and a median age of 17.6 years at surgery. The AG lesions were located in the superficial cerebrocortical region. Histological examination of these cases revealed characteristic structural features of AG, including bipolar spindle-shaped cells with an angiocentric growth pattern. However, four cases also exhibited atypical histological features: one had astroblastoma-like characteristics, two had a distinct cystic region with an onion-like structure and myxoid changes, and the other one had a region involving many abnormal neurones reminiscent to ganglioglioma. All were positive for glial fibrillary acidic protein and vimentin. Eight cases were positive for epithelial membrane antigen ( EMA), with a dot-like staining pattern. A diffuse D2-40 staining was visible in these cases, with two having similar staining pattern to EMA. All cases were immunonegative for BRAF V600 E and isocitrate dehydrogenase-1 R132 H mutations. Conclusions Our results demonstrate that atypical histological features can be present in AG. A collection of more cases and further molecular analyses are required to confirm our findings. [ABSTRACT FROM AUTHOR]

The WHO 2021 classification of central nervous system cancers distinguishes diffuse gliomas that arise in adults (referred to as the "adult type") and those that arise in children (defined as "paediatric") based on clinical and molecular characteristics."). However, paediatric-type gliomas may occasionally be present in younger adults and occasionally adult-type gliomas may occur in children. Diffuse low-grade paediatric glioma includes diffuse astrocytoma altered by MYB or MYBL1, low-grade polymorphic juvenile neuroepithelial tumour, angiocentric glioma, and diffuse low-grade glioma with an altered MAPK pathway. Here, we examine these newly recognised entities according to WHO diagnostic criteria and propose an integrated diagnostic approach that can be used to separate these clinically and biologically distinct tumor groups., (Copyright © 2022 Società Italiana di Anatomia Patologica e Citopatologia Diagnostica, Divisione Italiana della International Academy of Pathology.)

Angiocentric glioma is a low-grade tumour that occurs in children and young adults with a long-standing epilepsy. The typical histopathological features of this tumour is the presence of spindle-shaped cells, radially oriented around the cortical blood vessels. We present two teenage cases of the angiocentric variant of glioma: 1) a 15-year-old girl with a chronic and intractable partial epilepsy with cystic tumour located in the right temporal lobe and 2) a 14-year-old boy with intractable seizures and an extensive cortical lesion in the left parieto-occipital area. In both cases, the total tumours excision was performed. The histopathological findings revealed a characteristic angiocentric pattern that was composed of elongated cells arranging in pseudorosette-like structures around blood vessels. Moreover, schwannoma-like areas and subpial neoplastic infiltration with palisading of tumour cells at the brain surface were seen. The neoplastic cells displayed immunoreactivity for GFAP, S-100 protein and vimentin. A slight "dot-like" EMA staining, suggesting ependymal differentiation, was detected. The clinical and pathological picture allowed to establish the diagnosis of angiocentric gliomas. The patients were discharged home in a good condition and without seizures. During the 4-year follow-up, the tumour recurrence and seizures were not observed. The appropriate diagnosis of this peculiar type of usually low-grade glial tumour is important for adequate and successful treatment. The differential diagnosis requires the exclusion of other tumours with an angiocentric pattern, i.e. ependymoma, astroblastoma, which are associated with more aggressive biology. [ABSTRACT FROM AUTHOR]

The aim of this case report and minireview was to investigate the diagnosis of and therapeutic approaches for angiocentric glioma (AG) and to summarize the clinical manifestations and the pathological and imaging characteristics of the disease. Intraoperative cortical electroencephalogram (ECoG) monitoring was performed to locate the epileptic foci in a child with AG who presented with intractable epilepsy, prior to the total resection of the tumor being performed under the microscope. The clinical features, imaging characteristics, intraoperative conditions, surgical methods and pathological results were analyzed and compared with the literature. The review revealed that to date, the clinical features of the 52 reported cases of AG (including this case) have been mainly characterized by epilepsy. High T2-weighted image (WI) and fuid-attenuated inversion recovery (FLAIR) signals may be detected with magnetic resonance imaging (MRI) scanning of the cranium; however, no enhancement signals are detected by enhanced scanning. The prognosis following surgical resection is favorable. The lesions in the present case demonstrated clear boundaries with a central cystic affection accompanied by an arachnoid cyst on the left temporal pole. Pathological examination revealed that the lesion was positive for glial fibrillary acidic protein (GFAP), S-100 protein, vimentin, epithelial membrane antigen (EMA), cluster of differentiation 99 (CD99) and D2-40. The Ki-67/MIBk-1 labeling index was ~1%. In conclusion, AG exhibits characteristic features in imaging; however, its diagnosis depends on histopathological examination. The prognosis of total surgical resection is good and intraoperative ECoG may be used to assist positioning. [ABSTRACT FROM AUTHOR]