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Pilocytic astrocytoma

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https://www.readbyqxmd.com/read/29143922/pre-treatment-lymphopenia-and-indication-of-tumor-induced-systemic-immunosuppression-in-medulloblastoma
#1
Seema Patel, Shiyang Wang, Matija Snuderl, Matthias A Karajannis
The presence of tumor-induced systemic immune suppression, including lymphopenia, has been recognized in adult patients with glioblastoma for several decades, and pre-treatment neutrophil-to-lymphocyte count ratio (NLCR) is associated with inferior clinical outcome in patients with glioblastoma. Whether tumor-induced systemic immune suppression is also present in children with malignant brain tumors is not known. We performed a retrospective analysis of pretreatment neutrophil and lymphocyte counts in pediatric patients with medulloblastoma (MB) compared to a control group of children with posterior fossa pilocytic astrocytoma (PA)...
November 16, 2017: Journal of Neuro-oncology
https://www.readbyqxmd.com/read/29141672/a-novel-git2-braf-fusion-in-pilocytic-astrocytoma
#2
Jeffrey Helgager, Hart G Lidov, Navin R Mahadevan, Mark W Kieran, Keith L Ligon, Sanda Alexandrescu
BACKGROUND: KIAA1549-BRAF fusion is the most common genetic event in pilocytic astrocytoma (PA), and leads to activation of the mitogen activated protein kinase (MAPK) signaling pathway. Fusions of BRAF with other partner genes, as well as other genetic alterations not involving BRAF but also leading to MAPK pathway activation have been described rarely. CASE PRESENTATION: We present a new fusion partner in the low-grade glioma of a 10-year-old male, who presented with headaches and recent episodes of seizures...
November 15, 2017: Diagnostic Pathology
https://www.readbyqxmd.com/read/29138945/glioblastoma-in-neurofibromatosis-1-patients-without-idh1-braf-v600e-and-tert-promoter-mutations
#3
Ichiyo Shibahara, Yukihiko Sonoda, Hiroyoshi Suzuki, Akifumi Mayama, Masayuki Kanamori, Ryuta Saito, Yasuhiro Suzuki, Shoji Mashiyama, Hiroshi Uenohara, Mika Watanabe, Toshihiro Kumabe, Teiji Tominaga
Pilocytic astrocytomas and low-grade gliomas are more common compared with glioblastomas in patients with neurofibromatosis 1 (NF1). A recent genome-wide analysis has shown frequent NF1 gene alterations in the mesenchymal subtype of a glioblastoma; however, little is known about clinicopathological features of glioblastomas in NF1 patients (NF1 glioblastomas). We analyzed four NF1 glioblastomas. Radiographical and intraoperative findings showed well-circumscribed tumors from surrounding brain. Pathological analysis presented a paucity of processes with an eosinophilic cytoplasm, bizarre nuclei, xanthomatous-like appearance, multinucleated giant cells, and histiocytoid appearance...
November 14, 2017: Brain Tumor Pathology
https://www.readbyqxmd.com/read/29125440/long-term-outcomes-of-primarily-metastatic-juvenile-pilocytic-astrocytoma-in-children
#4
Derek Yecies, Paul Graham Fisher, Samuel Cheshier, Michael Edwards, Gerald Grant
OBJECTIVE Primarily metastatic juvenile pilocytic astrocytoma (JPA) is rare, likely representing 2%-3% of all cases of JPA. Due to the rarity of primarily metastatic JPA, there is currently no standard treatment paradigm and the long-term outcomes are not fully known. The goal of this case series was to add to the current understanding of this disease process. METHODS The authors searched a comprehensive database of pediatric patients with brain and spinal cord tumors treated at Lucile Packard Children's Hospital from 1997 to 2016 and identified 5 patients with primarily metastatic JPA...
November 10, 2017: Journal of Neurosurgery. Pediatrics
https://www.readbyqxmd.com/read/29118304/pediatric-optic-pathway-hypothalamic-glioma
#5
Yasuo Aihara, Kentaro Chiba, Seiichiro Eguchi, Kosaku Amano, Takakazu Kawamata
Optic pathway/hypothalamic gliomas (OP/HGs) are rare astrocytic tumors that appear more commonly among young children and often are unresectable. They comprise approximately 2% of all central nervous system tumors and account for 3-5% of pediatric intracranial tumors. Initial manifestations are often visual disturbances, endocrinopathies and hypothalamic dysfunction such as the diencephalic syndrome, and sometimes hydrocephalus due to cerebrospinal fluid (CSF) outflow obstruction. In many cases, the tumors are diagnosed late in the clinical course because they silently enlarge...
November 9, 2017: Neurologia Medico-chirurgica
https://www.readbyqxmd.com/read/29065962/disordered-consciousness-or-disordered-wakefulness-the-importance-of-prolonged-polysomnography-for-the-diagnosis-drug-therapy-and-rehabilitation-of-an-unresponsive-patient-with-brain-injury
#6
Francesca Formica, Marco Pozzi, Paolo Avantaggiato, Erika Molteni, Filippo Arrigoni, Flavio Giordano, Emilio Clementi, Sandra Strazzer
Disorders of consciousness may follow brain injury, due to impairments of wakefulness and/or awareness. Polysomnography can identify elements that may be ascribed to impairments of specific neuroanatomical areas. Recognizing which impairments affect each patient is crucial for diagnosis, prognosis, and to select an appropriate therapy. We present a pediatric case of insufficient wakefulness in a patient with severe disability following a pilocytic astrocytoma. Polysomnography was crucial for diagnosis, as it detected a well-structured pattern with daytime sleep initiations in the REM sleep phase...
October 18, 2017: Journal of Clinical Sleep Medicine: JCSM: Official Publication of the American Academy of Sleep Medicine
https://www.readbyqxmd.com/read/29061673/pilocytic-astrocytoma-with-anaplasia-arising-from-the-optic-chiasm-in-a-very-elderly-patient
#7
Shintaro Hayashi, Norihiko Akao, Yoichi Nakazato, Koichi Okamoto
No abstract text is available yet for this article.
October 24, 2017: Neurology
https://www.readbyqxmd.com/read/29045978/-clinicopathologic-features-of-infant-dysembryoplastic-neuroepithelial-tumor-a-case-report-and-literature-review
#8
H Wang, J T Ye, H X Yao, D Li, Y Dong
Dysembryoplastic neuroepithelial tumor (DNT) has traditionally been viewed as rare benign tumors that present with seizure widely considered curable with surgery alone. Most DNTs occur in childhood and young adults. However, rare reported cases occur in infants. This paper reported an infant case of DNT and its diagnosis, differential diagnosis, treatment, molecular features and prognosis based on the review of current literatures. The age onset of this patient was only 11 months old. The clinical manifestations were partial seizures and the imaging data untypical; CT and MRI were all supportive of astrocytoma...
October 18, 2017: Beijing da Xue Xue Bao. Yi Xue Ban, Journal of Peking University. Health Sciences
https://www.readbyqxmd.com/read/29039591/dabrafenib-in-patients-with-recurrent-braf-v600e-mutated-malignant-glioma-and-leptomeningeal-disease
#9
Michael C Burger, Michael W Ronellenfitsch, Nadja I Lorenz, Marlies Wagner, Martin Voss, David Capper, Theophilos Tzaridis, Ulrich Herrlinger, Joachim P Steinbach, Gabriele Stoffels, Karl-Josef Langen, Christian Brandts, Christian Senft, Patrick N Harter, Oliver Bähr
BRAF V600E mutations occur frequently in malignant melanoma, but are rare in most malignant glioma subtypes. Besides, more benign brain tumors such as ganglioglioma, dysembryoblastic neuroepithelial tumours and supratentorial pilocytic astrocytomas, only pleomorphic xanthoastrocytomas (50-78%) and epitheloid glioblastoma (50%) regularly exhibit BRAF mutations. In the present study, we report on three patients with recurrent malignant gliomas harbouring a BRAF V600E mutation. All patients presented with markedly disseminated leptomeningeal disease at recurrence and had progressed after radiotherapy and alkylating chemotherapy...
October 2, 2017: Oncology Reports
https://www.readbyqxmd.com/read/28984141/dabrafenib-and-trametinib-in-brafv600e-mutated-glioma
#10
Nicholas F Brown, Thomas Carter, Neil Kitchen, Paul Mulholland
BRAFV600E mutations have been identified in a number of glioma subtypes, most frequently in pleomorphic xanthoastrocytoma, ganglioglioma, pilocytic astrocytoma, and epithelioid glioblastoma. Although the development of BRAF inhibitors has dramatically improved the clinical outcome for patients with BRAFV600E mutant tumors, resistance develops in a majority of patients due to reactivation of the MAPK pathway. Addition of MEK inhibition to BRAF inhibition improves survival. Here we report successful treatment of two patients with BRAFV600E mutant pleomorphic xanthoastrocytoma using the BRAF inhibitor dabrafenib in combination with the MEK inhibitor trametinib...
October 6, 2017: CNS Oncology
https://www.readbyqxmd.com/read/28980149/hemorrhagic-presentation-of-intracranial-pilocytic-astrocytomas-literature-review
#11
REVIEW
G Lakshmi Prasad, B N Nandeesh, Girish R Menon
Pilocytic astrocytomas (PAs) are seemingly innocuous and benign tumors. However, in recent times, many case series have documented high rates of hemorrhage in these neoplasms. We hereby provide a detailed analysis on hemorrhagic pilocytic astrocytomas (HPA) in adults and report one such case managed at our institute. In addition, salient differences between adult and pediatric hemorrhagic PA have been briefed. Hospital records were retrieved for our case. Literature review was conducted by searching online databases for the following keywords-pilocytic astrocytoma, hemorrhage, cranial, pediatric, and adults...
October 4, 2017: Neurosurgical Review
https://www.readbyqxmd.com/read/28968690/regnet-an-r-package-for-network-based-propagation-of-gene-expression-alterations
#12
Michael Seifert, Andreas Beyer
Summary: Gene expression alterations and potentially underlying gene copy number mutations can be measured routinely in the wet lab, but it is still extremely challenging to quantify impacts of altered genes on clinically relevant characteristics to predict putative driver genes. We developed the R package regNet that utilizes gene expression and copy number data to learn regulatory networks for the quantification of potential impacts of individual gene expression alterations on user-defined target genes via network propagation...
August 31, 2017: Bioinformatics
https://www.readbyqxmd.com/read/28968159/pediatric-thalamic-gliomas-an-updated-review
#13
REVIEW
Avneesh Gupta, Nathan Shaller, Kathryn A McFadden
CONTEXT: - Neoplasms originating in the thalamus are rare overall (1% of all brain tumors); however, they comprise approximately 5% of pediatric intracranial tumors and approach 15% of all malignant pediatric intracranial tumors in some series. OBJECTIVE: - To update readers about the current understanding of the diverse histology, biology, and behavior of pediatric thalamic tumors. Histologic verification is now thought to be critical for planning treatment, and, as a result, biopsy and total/subtotal resections are much more common today than in the past...
October 2017: Archives of Pathology & Laboratory Medicine
https://www.readbyqxmd.com/read/28968151/cerebellar-pilocytic-astrocytoma-in-childhood-investigating-the-long-term-impact-of-surgery-on-cognitive-performance-and-functional-outcome
#14
Thomas Pletschko, Anna Felnhofer, Doris Lamplmair, Christian Dorfer, Thomas Czech, Monika Chocholous, Irene Slavc, Ulrike Leiss
OBJECTIVE: Previous studies differ regarding the long-term effects of surgically removed pediatric cerebellar pilocytic astrocytomas (CPA). Thus, the aim of this study was to investigate the long-term impact on neurocognitive and functional outcome and to analyze age as an influencing factor. METHODS: Fourteen CPA patients were compared to the age norm and to a group of 14 high-achieving peers regarding cognitive functioning, health-related quality of life (HRQoL), and stress regulation...
October 2, 2017: Developmental Neurorehabilitation
https://www.readbyqxmd.com/read/28960151/brainstem-pilocytic-astrocytoma-with-h3-k27m-mutation-case-report
#15
Shuhei Morita, Masayuki Nitta, Yoshihiro Muragaki, Takashi Komori, Kenta Masui, Takashi Maruyama, Koichi Ichimura, Yoshiko Nakano, Tatsuo Sawada, Shunichi Koriyama, Shunsuke Tsuzuki, Takayuki Yasuda, Kazutoshi Hashimoto, Akihiro Niwa, Takakazu Kawamata
In this report, the authors present the first case of adult brainstem pilocytic astrocytoma (PA) with the H3 K27M mutation. A 53-year-old man was incidentally found to have a 2.5-cm partially enhanced tumor in the tectum on MRI. The enhancement in the lesion increased over 3 years, and gross-total removal was performed via the occipital transtentorial approach. The resected tissue indicated PA, WHO Grade I, and genetic analysis revealed the H3 K27M mutation. However, although the radiological, surgical, and pathological findings all corresponded to PA, this entity can easily be misdiagnosed as diffuse midline glioma with the H3 K27M mutation, which is classified as a WHO Grade IV tumor according to the updated classification...
September 29, 2017: Journal of Neurosurgery
https://www.readbyqxmd.com/read/28947983/deceptive-morphologic-and-epigenetic-heterogeneity-in-diffuse-intrinsic-pontine-glioma
#16
Marianna Bugiani, Sophie E M Veldhuijzen van Zanten, Viola Caretti, Pepijn Schellen, Eleonora Aronica, David P Noske, William P Vandertop, Gertjan J L Kaspers, Dannis G van Vuurden, Pieter Wesseling, Esther Hulleman
Historically, the diagnosis of diffuse intrinsic pontine glioma (DIPG) was based on typical imaging findings and clinical characteristics instead of pathology. However, the discovery of mutations in histone H3 variants, and the availability of tumor material for molecular analysis, has led to a paradigm shift in DIPG research and clinical practice. Using data from whole-brain autopsies in a series of nine DIPG patients with known histone mutational status, we here aim to review histopathological characteristics with special focus on intratumoral heterogeneity (ITH) and histone 3 K27 trimethylation (H3 K27me3)...
September 1, 2017: Oncotarget
https://www.readbyqxmd.com/read/28941523/a-review-of-visual-and-oculomotor-outcomes-in-children-with-posterior-fossa-tumors
#17
Crandall E Peeler
Tumors of the posterior fossa represent the most common solid malignancy of childhood and can affect the visual system in several ways. This article outlines the relevant visual anatomy affected by these tumors and reviews the visual and oculomotor outcomes associated with the following 3 most common tumor types-medulloblastoma, juvenile pilocytic astrocytoma, and ependymoma. The available data suggest that the rate of permanent vision loss is low (5.9%-8.3%), with patients having juvenile pilocytic astrocytoma demonstrating the best outcomes...
May 2017: Seminars in Pediatric Neurology
https://www.readbyqxmd.com/read/28935546/fluorescein-guided-resection-of-intramedullary-spinal-cord-tumors-results-from-a-preliminary-multicentric-retrospective-study
#18
Francesco Acerbi, Claudio Cavallo, Karl-Michael Schebesch, Mehmet Osman Akcakaya, Camilla de Laurentis, Mustafa Kemal Hamamcioglu, Morgan Broggi, Alexander Brawanski, Jacopo Falco, Roberto Cordella, Paolo Ferroli, Telat Kiris, Julius Höhne
INTRODUCTION: IMSCTs are rare heterogenous lesions usually enhancing at pre-operative MRI, due to a damaged BBB. SF is a dye that accumulates in areas of the CNS with a damaged BBB. Given the pattern of MRI contrast-enhancement of the majority of IMSCTs, the use of this fluorescent tracer could improve tumor visualization and quality of resection. In this paper, we present the first experience on the application of fluorescein-guided technique for surgical removal of IMSCTs. METHODS: 11 patients (6 males, 5 females, mean age 50...
September 18, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28928859/idh1-r132h-mutation-is-accompanied-with-malignant-progression-of-paired-primary-recurrent-astrocytic-tumours
#19
Luyan Mu, Wanzhen Xu, Qingla Li, Haitao Ge, Hongbo Bao, Songsong Xia, Jingjing Ji, Jie Jiang, Yuwen Song, Qiang Gao
IDH1 R132H mutation is an important marker of survival in patients with gliomas. Although there are many changes of genes in tumour malignant progression, IDH1 R132H mutation status in glioma progression remained unclear. Here, an in-depth characterization of IDH1 R132H mutations were assessed by immunohistochemistry in 55 paired primary-recurrent astrocytomas tissues, including 5 paired primary pilocytic astrocytoma (pPA, WHO grade I), 35 paired primary low grade astrocytoma (pLGA, WHO grade II and III) and 15 paired primary high grade astrocytoma (pHGA/ Glioblastoma, WHO grade IV)...
2017: Journal of Cancer
https://www.readbyqxmd.com/read/28919136/pilocytic-astrocytoma-mimicking-cavernous-angioma-imaging-features-and-histological-characteristics
#20
M Gaha, F Bouzayen, Y Limam, M Mokni, H Jemni-Gharbi, K Tlili-Graiess
Pilocytic astrocytoma (PA) commonly occurs during the first two decades of life. Typical locations include cerebellum, optic nerve, optic chiasm/hypothalamus and brainstem. PA should be considered in the differential diagnosis of patients with brain tumors manifesting with hemorrhagic onset. We report a case of a hemorrhagic onset of cerebellar PA in a young adult with imaging findings mimicking cavernous angioma. We also discuss imaging features and histological characteristics with a focus on the etiology of the hemorrhagic onset...
September 2017: Neuro-Chirurgie
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