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ganglioglioma

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https://www.readbyqxmd.com/read/28408259/pediatric-basal-ganglia-region-tumors-clinical-and-radiological-features-correlated-with-histopathological-findings
#1
Wei Fu, Yan Ju, Si Zhang, Chao You
PURPOSE: To summarize the clinical and radiological features of pediatric basal ganglia region tumors (PBGRT) in correlation with their histopathological findings to reduce inappropriate surgery and identify tumors that can benefit from maximal safe resection. METHODS: The records of 35 children with PBGRT treated in our hospital from December 2011 to December 2015 were retrospectively analyzed. The clinical and radiological features of these tumors were summarized in correlated with their histopathological diagnosis...
April 10, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28406082/intraventricular-ganglioglioma-with-extensive-hemorrhage
#2
William Warnica, John P Provias
Gangliogliomas represent a rare form of neuroepithelial tumor (up to 1.3% of brain tumors [1]), which even more rarely present with hemorrhage or localize intraventricularly. To date, only two cases of ganglioglioma with both of these features have been reported. Our patient is a 23-year-old woman who presented with signs and symptoms of increased intracranial pressure (ICPI), with a post-subtotal resection diagnosis of WHO Grade I ganglioglioma localizing bilaterally to the lateral ventricles. One year following the operation, the tumor showed radiologic evidence of interval hemorrhage, which was verified histopathologically following a second subtotal resection...
April 13, 2017: Clinical Neuropathology
https://www.readbyqxmd.com/read/28395087/retrospective-analysis-of-molecular-and-immunohistochemical-characterization-of-381-primary-brain-tumors
#3
Leomar Y Ballester, Gregory N Fuller, Suzanne Z Powell, Erik P Sulman, Keyur P Patel, Rajyalakshmi Luthra, Mark J Routbort
The classification of brain tumors has traditionally depended on microscopic examination of hematoxylin and eosin-stained tissue sections. The increased understanding of clinically relevant genetic alterations has led to the incorporation of molecular signatures as part of the diagnosis of brain malignancies. Advances in sequencing technologies have facilitated the use of next-generation sequencing (NGS) assays in clinical laboratories. We performed a retrospective analysis of sequencing results for 381 brain tumors tested by NGS at our institution using a validated, commercially available panel...
March 1, 2017: Journal of Neuropathology and Experimental Neurology
https://www.readbyqxmd.com/read/28293736/5ala-in-pediatric-brain-tumors-is-not-routinely-beneficial
#4
Jonathan Roth, Shlomi Constantini
PURPOSE: Over recent years, 5-aminoluvolinic acid (5ALA) has been increasingly used for resection guidance in adult high-grade gliomas. However, amongst pediatric patients, publication of intraoperative fluorescence has been limited, with inconsistent outcomes. We describe our experience and intraoperative finding amongst children with various brain tumors that were given 5ALA prior to tumor resection. METHODS: Since October 2014, data regarding intraoperative findings amongst children that received 5ALA prior to tumor resection were prospectively collected...
March 14, 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/28293477/braf-v600e-immunohistochemistry-in-a-large-series-of-glial-and-glial-neuronal-tumors
#5
Quentin Breton, Hélène Plouhinec, Delphine Prunier-Mirebeau, Blandine Boisselier, Sophie Michalak, Philippe Menei, Audrey Rousseau
INTRODUCTION: Some glial-neuronal tumors (GNT) (pleomorphic xantho-astrocytoma [PXA], ganglioglioma [GG]) display BRAF-V600E mutation, which represents a diagnostic clue to these entities. Targeted therapies against BRAF-V600 protein have shown promising results in GNT. The aim of this study was to assess the utility of BRAF-V600E immunohistochemistry (IHC, clone VE1) in daily practice in a series of 140 glial, and GNT compared to molecular biology (MB) techniques. METHODS: We performed BRAF-V600E IHC on all 140 cases...
March 2017: Brain and Behavior
https://www.readbyqxmd.com/read/28222441/unusual-occurrence-of-multifocal-desmoplastic-infantile-astrocytoma-a-case-report-and-review-of-the-literature
#6
Vinayak Narayan, Amey R Savardekar, Anita Mahadevan, A Arivazhagan, Lingegowda Appaji
Desmoplastic infantile gliomas are rare, benign tumors of the early infancy period. Two histological subtypes - desmoplastic infantile astrocytoma (DIA) and desmoplastic infantile ganglioglioma - have been described. The characteristic features of DIAs are lobar location, glial histology, and excellent prognosis after complete surgical excision. DIAs usually present as solitary, cortical-surfacing, solid-cystic neoplasms; however, atypical, aggressive, and multifocal variants of DIA have been reported in the literature...
February 22, 2017: Pediatric Neurosurgery
https://www.readbyqxmd.com/read/28144989/dentatorubral-pallidoluysian-atrophy-drpla-with-a-small-ganglioglioma-component-containing-neurofibrillary-tangles-and-polyglutamine-aggregation
#7
Seiji Yamada, Tatsuya Yamazaki, Satoshi Nakata, Sumihito Nobusawa, Hayato Ikota, Munenori Ide, Kazuyuki Mizushima, Yasuo Harigaya, Junko Hirato, Hideaki Yokoo
Dentatorubral-pallidoluysian atrophy (DRPLA), one of the polyglutamine diseases, has not been reported in combination with ganglioglioma (GG). Herein, we report an autopsy case of a 72-year-old man with DRPLA with a small GG component harboring neurofibrillary tangles (NFTs) and polyglutamine aggregates. NFTs, cytoplasmic accumulations of hyper-phosphorylated tau, are mainly observed in Alzheimer's disease (AD) and other tau-associated neurodegenerative disorders. NFTs can also be present in normal aging, and are occasionally observed in low-grade central nervous system (CNS) neoplasms such as GG...
January 31, 2017: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/28111355/epigenetics-in-epilepsy
#8
REVIEW
K Kobow, I Blümcke
Approximately 50 million people have epilepsy, making it the most common chronic and severe neurological disease worldwide, with increased risk of mortality and psychological and socioeconomic consequences impairing quality of life. More than 30% of patients with epilepsy have inadequate control of their seizures with drug therapy. Any structural brain lesion can provoke epilepsy. However, progression of seizure activity as well as the development of drug-resistance remains difficult to predict, irrespective of the underlying epileptogenic condition, i...
January 19, 2017: Neuroscience Letters
https://www.readbyqxmd.com/read/28110204/new-classification-of-epilepsy-related-neoplasms-the-clinical-perspective
#9
REVIEW
Burkhard S Kasper, Ekkehard M Kasper
Neoplastic CNS lesions are a common cause of focal epilepsy refractory to anticonvulsant treatment, i.e. long-term epilepsy-associated tumors (LEATs). Epileptogenic tumors encompass a variety of intriguing lesions, e.g. dysembryoplastic neuroepithelial tumors or gangliogliomas, which differ from more common CNS neoplasms in their clinical context as well as on histopathology. Long-term epilepsy-associated tumor classification is a rapidly evolving issue in surgical neuropathology, with new entities still being elucidated...
February 2017: Epilepsy & Behavior: E&B
https://www.readbyqxmd.com/read/28098941/posterior-cortex-epilepsy-surgery-in-childhood-and-adolescence-predictors-of-long-term-seizure-outcome
#10
Georgia Ramantani, Angeliki Stathi, Armin Brandt, Karl Strobl, Susanne Schubert-Bast, Gert Wiegand, Rudolf Korinthenberg, Vera van Velthoven, Josef Zentner, Andreas Schulze-Bonhage, Thomas Bast
OBJECTIVE: We aimed to investigate the long-term seizure outcome of children and adolescents who were undergoing epilepsy surgery in the parietooccipital cortex and determine their predictive factors. METHODS: We retrospectively analyzed the data of 50 consecutive patients aged 11.1 (mean) ± 5.1 (standard deviation) years at surgery. All patients but one had a magnetic resonance imaging (MRI)-visible lesion. Resections were parietal in 40%, occipital in 32%, and parietooccipital in 28% cases; 24% patients additionally underwent a resection of the posterior border of the temporal lobe...
March 2017: Epilepsia
https://www.readbyqxmd.com/read/28069802/genomic-profiling-of-a-large-set-of-diverse-pediatric-cancers-identifies-known-and-novel-mutations-across-tumor-spectra
#11
Juliann Chmielecki, Mark Bailey, Jie He, Julia Elvin, Jo-Anne Vergilio, Shakti Ramkissoon, James Suh, Garrett M Frampton, James X Sun, Samantha Morley, Daniel Spritz, Siraj Ali, Laurie Gay, Rachel L Erlich, Jeffrey S Ross, Joana Buxhaku, Hilary Davies, Vinny Faso, Alexis Germain, Blair Glanville, Vincent A Miller, Philip J Stephens, Katherine A Janeway, John M Maris, Soheil Meshinchi, Trevor J Pugh, Jack F Shern, Doron Lipson
Pediatric cancers are generally characterized by low mutational burden and few recurrently mutated genes. Recent studies suggest that genomic alterations may help guide treatment decisions and clinical trial selection. Here, we describe genomic profiles from 1,215 pediatric tumors representing sarcomas, extracranial embryonal tumors, brain tumors, hematologic malignancies, carcinomas, and gonadal tumors. Comparable published datasets identified similar frequencies of clinically relevant alterations, validating this dataset as biologically relevant...
January 15, 2017: Cancer Research
https://www.readbyqxmd.com/read/28041673/epilepsy-related-brain-tumors
#12
REVIEW
Özdem Ertürk Çetin, Cihan İşler, Mustafa Uzan, Çiğdem Özkara
Seizures are among the most common presentations of brain tumors. Several tumor types can cause seizures in varying rates; neuroglial tumors and the gliomas are the most common ones. Brain tumors are the second most common cause of focal intractable epilepsy in epilepsy surgery series, with the highest frequency being dysembryoplastic neuroepithelial tumors and gangliogliomas. Seizure management is an important part of the treatment of patients with brain tumors. This review discusses clinical features and management of seizures in patients with brain tumors, including, neuroglial tumors, gliomas, meningioma and metastases; with the help of recent literature data...
January 2017: Seizure: the Journal of the British Epilepsy Association
https://www.readbyqxmd.com/read/28017579/seizures-caused-by-brain-tumors-in-children
#13
REVIEW
Iván Sánchez Fernández, Tobias Loddenkemper
PURPOSE: To review the epidemiology, clinical features, and treatment of seizures secondary to pediatric brain tumors. METHOD: Literature review. RESULTS: Pediatric brain tumors are the most common solid pediatric tumor and the most common cause of death in pediatric cancer. Seizures are one of the most common symptoms of pediatric brain tumors. Factors associated with increased risk of seizures include supratentorial location, gray matter involvement, low-grade, and certain histological features-especially dysembryoplastic neuroepithelial tumor, ganglioglioma, and oligodendroglioma...
January 2017: Seizure: the Journal of the British Epilepsy Association
https://www.readbyqxmd.com/read/27992000/refractory-epilepsy-in-children-with-brain-tumors-the-urgency-of-neurosurgery
#14
Marília Rosa Abtibol Bernardino, Carolina Funayama, Ana Paula Andrade Hamad, Hélio Machado, Américo Sakamoto, Ursula Thome, Vera Cristina Terra, Antonio Carlos Dos Santos
In order to verify indications for surgery, 27 patients with refractory epileptic seizures and brain tumor, aged up to 19 years at the time of surgery, were studied between 1996 and 2013 and followed up for at least one year. The mean interval between the onset of seizures and the diagnosis of the tumor was 3.6 years, and from diagnosis to the surgery, 18 months. The location of the tumor was in the temporal lobe in 16, with ganglioglioma and dysembryoplastic neuroepithelial tumors being the most frequent. Among the patients, 92...
December 2016: Arquivos de Neuro-psiquiatria
https://www.readbyqxmd.com/read/27984807/braf-v600e-mutation-in-epilepsy-associated-glioneuronal-tumors-prevalence-and-correlation-with-clinical-features-in-a-chinese-population
#15
Yin-Xi Zhang, Chun-Hong Shen, Yi Guo, Yang Zheng, Jun-Ming Zhu, Yao Ding, Ye-Lei Tang, Shuang Wang, Mei-Ping Ding
PURPOSE: Glioneuronal tumors (GNTs) are the most common histological type of brain tumors in patients who received epilepsy surgery, and part of them presented with BRAF V600E mutation. We aimed to verify the presence of the BRAF V600E mutation in epilepsy-associated GNTs from Chinese population and evaluate the association with clinical features. METHODS: Data from 35 patients diagnosed with GNTs, including 24 gangliogliomas and 11 dysembryoplastic neuroepithelial tumors, were retrospectively collected...
February 2017: Seizure: the Journal of the British Epilepsy Association
https://www.readbyqxmd.com/read/27984673/co-occurrence-of-histone-h3-k27m-and-braf-v600e-mutations-in-paediatric-midline-grade-i-ganglioglioma
#16
Mélanie Pagès, Kevin Beccaria, Nathalie Boddaert, Raphaël Saffroy, Aurore Besnard, David Castel, Frédéric Fina, Doriane Barets, Emilie Barret, Ludovic Lacroix, Franck Bielle, Felipe Andreiuolo, Arnault Tauziède-Espariat, Dominique Figarella-Branger, Stéphanie Puget, Jacques Grill, Fabrice Chrétien, Pascale Varlet
Ganglioglioma (GG) is a grade I tumor characterized by alterations in the MAPK pathway, including BRAF V600E mutation. Recently, diffuse midline glioma with an H3 K27M mutation was added to the WHO 2016 classification as a new grade IV entity. As co-occurrence of H3 K27M and BRAF V600E mutations has been reported in midline tumors and anaplastic GG, we searched for BRAF V600E and H3 K27M mutations in a series of 54 paediatric midline grade I GG (midline GG) to determine the frequency of double mutations and its relevance for prognosis...
December 16, 2016: Brain Pathology
https://www.readbyqxmd.com/read/27936528/infratentorial-ganglioglioma-mimicking-pilocytic-astrocytoma
#17
Kirti Gupta, Madhivanan Karthigeyan, Pravin Salunke
Majority of tumors arising in posterior fossa in children are pilocytic astrocytoma. However, a small subset of these have a neuronal component that may either be admixed throughout with the glial component or may be segregated to one or two regions within the glial element. Interestingly, the radiological features of this subgroup are also distinctive, showing midline location, frequent cyst formation, hemorrhage, and circumscribed nature. Taken together, both radiological and pathological features can fairly predict the subgroup categorization within infratentorial gangliogliomas (GG), as has been recently elucidated...
March 2017: Clinical Neuropathology
https://www.readbyqxmd.com/read/27921213/pediatric-intracranial-primary-anaplastic-ganglioglioma
#18
Wolf Lüdemann, Rouzbeh Banan, Christian Hartmann, Helmut Bertalanffy, Concezio Di Rocco
BACKGROUND: Primary intracranial anaplastic gangliogliomas are rare tumors in the pediatric patient group. Most of them present with symptoms of elevated pressure or symptomatic epilepsy. Extraaxial location is far more common than axial location. On MRI examination, they mimic pilocytic astrocytomas. The outcome after surgery depends mainly on the possible amount of surgical resection, and oncological therapy is necessary to prevent recurrence of the disease. CASE REPORT: An 11-year-old boy presented with headache and double vision due to obstructive hydrocephalus...
February 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/27910945/thyroid-transcription-factor-1-distinguishes-subependymal-giant-cell-astrocytoma-from-its-mimics-and-supports-its-cell-origin-from-the-progenitor-cells-in-the-medial-ganglionic-eminence
#19
Jen-Fan Hang, Chih-Yi Hsu, Shih-Chieh Lin, Chih-Chun Wu, Han-Jui Lee, Donald Ming-Tak Ho
Subependymal giant cell astrocytoma is a benign brain tumor mostly associated with tuberous sclerosis complex. However, it may be misinterpreted as other high-grade brain tumors due to the presence of large tumor cells with conspicuous pleomorphism and occasional atypical features, such as tumor necrosis and endothelial proliferation. In this study, we first investigated thyroid transcription factor-1 (TTF-1) expression in a large series of subependymal giant cell astrocytomas and other histologic and locational mimics to validate the diagnostic utility of this marker...
December 2, 2016: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
https://www.readbyqxmd.com/read/27860162/desmoplastic-infantile-astrocytoma-ganglioglioma-with-rare-braf-v600d-mutation
#20
Ashley Greer, Nicholas K Foreman, Andrew Donson, Kurtis D Davies, B K Kleinschmidt-DeMasters
BACKGROUND: Desmoplastic infantile astrocytoma (DIA) and desmoplastic infantile gangliogliomas (DIGs) are rare, massive, cystic and solid tumors of infants usually found in superficial cerebral hemispheres. They manifest prominent desmoplastic stroma, admixed neoplastic astrocytes, primitive-appearing small cells, and additional neoplastic ganglion cells in the case of DIGs. While v-Raf murine sarcoma viral oncogene homolog B (BRAF) mutation is found in up to 50% of pediatric gangliogliomas, two recent studies found that it was rare in DIA/DIGs; we sought to assess BRAF status in DIA/DIGs from our institution...
November 10, 2016: Pediatric Blood & Cancer
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