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Childhood low grade glioma

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https://www.readbyqxmd.com/read/29063957/significance-of-h3k27m-mutation-with-specific-histomorphological-features-and-associated-molecular-alterations-in-pediatric-high-grade-glial-tumors
#1
Süheyla Uyar Bozkurt, A Dagcinar, B Tanrikulu, N Comunoglu, B C Meydan, M Ozek, B Oz
PURPOSE: Pediatric high-grade gliomas (pHGGs) constitute almost 15% of all childhood brain tumors. Recurrent mutations such as H3K27M mutation in H3F3A and HIST1H3B genes encoding histone H3 and its variants were identified in approximately 30% of pediatric glioblastomas. This study aimed to ascertain the morphological and molecular characteristics of pHGGs with H3K27M mutation. METHODS: In total, 61 cases of pHGGs (anaplastic astrocytoma, 12; glioblastomas, 49) from four university hospitals were studied...
October 24, 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/29045978/-clinicopathologic-features-of-infant-dysembryoplastic-neuroepithelial-tumor-a-case-report-and-literature-review
#2
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/29016845/pediatric-low-grade-gliomas-next-biologically-driven-steps
#3
David T W Jones, Mark W Kieran, Eric Bouffet, Sanda Alexandrescu, Pratiti Bandopadhayay, Miriam Bornhorst, David Ellison, Jason Fangusaro, Michael I Fisher, Nicholas Foreman, Maryam Fouladi, Darren Hargrave, Cynthia Hawkins, Nada Jabado, Maura Massimino, Sabine Mueller, Giorgio Perilongo, Antoinette Y N Schouten-van Meeteren, Uri Tabori, Katherine Warren, Angela J Waanders, David Walker, William Weiss, Olaf Witt, Karen Wright, Yuan Zhu, Daniel C Bowers, Stefan M Pfister, Roger J Packer
Despite the fact that they are not typically life-threatening, low-grade gliomas (LGGs) remain a significant clinical challenge in pediatric neurooncology due to co-morbidities associated with these tumors and/or their treatments, and their propensity to multiply recur. LGGs, in total the most common brain tumors arising in childhood, can often become a chronic problem requiring decades of management. The 2nd international consensus conference on pediatric low-grade gliomas held in Padua, Italy in 2016, was convened in an attempt to advance the pace of translating biological discoveries on LGGs into meaningful clinical benefit...
August 2, 2017: Neuro-oncology
https://www.readbyqxmd.com/read/28989289/epidemiology-diagnosis-and-optimal-management-of-glioma-in-adolescents-and-young-adults
#4
REVIEW
Tejan P Diwanji, Alexander Engelman, James W Snider, Pranshu Mohindra
Neoplasms of the central nervous system (CNS) are the most frequently encountered solid tumors of childhood, but are less common in adolescents and young adults (AYA), aged 15-39 years. Gliomas account for 29%-35% of the CNS tumors in AYA, with approximately two-thirds being low-grade glioma (LGG) and the remaining being high-grade glioma (HGG). We review the epidemiology, work-up, and management of LGG and HGG, focusing on the particular issues faced by the AYA population relative to pediatric and adult populations...
2017: Adolescent Health, Medicine and Therapeutics
https://www.readbyqxmd.com/read/28828715/treatment-of-childhood-astrocytomas-with-irinotecan-and-cisplatin
#5
J Mora, S Perez-Jaume, O Cruz
BACKGROUND: Previously we described the outcome of children with spinal cord astrocytoma treated with irinotecan and cisplatin (I/C). We here report the review of the initial institutional experience using this combination for children with low-grade glioma (LGG). PROCEDURE: I/C chemotherapy consisted of weekly cisplatin (30 mg/m(2)) and irinotecan (50-65 mg/m(2)) for a total maximum of 16 doses, administered in an outpatient basis. RESULTS: Between November 2002 and December 2009, 46 children (median age 6...
August 21, 2017: Clinical & Translational Oncology
https://www.readbyqxmd.com/read/28808787/malignant-transformation-of-a-conservatively-managed-incidental-childhood-cerebral-mass-lesion-controversy-regarding-management-paradigm
#6
Jehuda Soleman, Jonathan Roth, Zvi Ram, Michal Yalon, Shlomi Constantini
BACKGROUND: Incidental findings on neuroimaging in the pediatric population are an emerging treatment challenge. Treatment options for these incidental childhood brain mass lesions, which radiologically may be assumed to be low-grade gliomas (LGG), vary, ranging from careful conservative "wait and scan" treatment to surgical biopsy, gross total resection, and upfront radiation and/or chemotherapy. As malignant transformation of LGG in children is extremely rare, some series advocate careful conservative management of these lesions; however, universal treatment protocols are not totally agreed upon...
December 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/28649001/a-european-randomised-controlled-trial-of-the-addition-of-etoposide-to-standard-vincristine-and-carboplatin-induction-as-part-of-an-18-month-treatment-programme-for-childhood-%C3%A2-16%C3%A2-years-low-grade-glioma%C3%A2-a-final-report
#7
RANDOMIZED CONTROLLED TRIAL
Astrid K Gnekow, David A Walker, Daniela Kandels, Susan Picton, Giorgio Perilongo, Jacques Grill, Tore Stokland, Per Eric Sandstrom, Monika Warmuth-Metz, Torsten Pietsch, Felice Giangaspero, René Schmidt, Andreas Faldum, Denise Kilmartin, Angela De Paoli, Gian Luca De Salvo
BACKGROUND: The use of chemotherapy to manage newly diagnosed low grade glioma (LGG) was first introduced in the 1980s. One randomised trial has studied two- versus four-drug regimens with a duration of 12 months of treatment after resection. METHODS: Within the European comprehensive treatment strategy for childhood LGG, the International Society of Paediatric Oncology-Low Grade Glioma (SIOP LGG) Committee launched a randomised trial involving 118 institutions and 11 countries to investigate the addition of etoposide (100 mg/m(2), days 1, 2 & 3) to a four-course induction of vincristine (1...
August 2017: European Journal of Cancer
https://www.readbyqxmd.com/read/28009226/report-of-effective-trametinib-therapy-in-2-children-with-progressive-hypothalamic-optic-pathway-pilocytic-astrocytoma-documentation-of-volumetric-response
#8
Catherine Miller, Daniel Guillaume, Kathryn Dusenbery, H Brent Clark, Christopher Moertel
Brain tumors are the most common solid tumor in childhood, and astrocytomas account for the largest proportion of these tumors. Increasing sophistication in genetic testing has allowed for the detection of specific mutations within tumor subtypes that may represent targets for individualized tumor treatment. The mitogen-activating protein kinase (MAPK) pathway and, more specifically, BRAF mutations have been shown to be prevalent in pediatric pilocytic astrocytomas and may represent one such area to target...
March 2017: Journal of Neurosurgery. Pediatrics
https://www.readbyqxmd.com/read/27998231/hypothalamic-pituitary-axis-dysfunction-in-survivors-of-childhood-cns-tumors-importance-of-systematic-follow-up-and-early-endocrine-consultation
#9
REVIEW
Wassim Chemaitilly, Gregory T Armstrong, Amar Gajjar, Melissa M Hudson
The Oncology Grand Rounds series is designed to place original reports published in the Journal into clinical context. A case presentation is followed by a description of diagnostic and management challenges, a review of the relevant literature, and a summary of the authors' suggested management approaches. The goal of this series is to help readers better understand how to apply the results of key studies, including those published in Journal of Clinical Oncology, to patients seen in their own clinical practice...
December 20, 2016: Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology
https://www.readbyqxmd.com/read/27888085/high-definition-fiber-tractography-in-evaluation-and-surgical-planning-of-thalamopeduncular-pilocytic-astrocytomas-in-pediatric-population-case-series-and-review-of-literature
#10
REVIEW
Emrah Celtikci, Pinar Celtikci, David Tiago Fernandes-Cabral, Murat Ucar, Juan Carlos Fernandez-Miranda, Alp Ozgun Borcek
OBJECTIVE: Thalamopeduncular tumors (TPTs) of childhood present a challenge for neurosurgeons due to their eloquent location. Preoperative fiber tracking provides total or near-total resection, without additional neurologic deficit. High-definition fiber tractography (HDFT) is an advanced white matter imaging technique derived from magnetic resonance imaging diffusion data, shown to overcome the limitations of diffusion tensor imaging. We aimed to investigate alterations of corticospinal tract (CST) and medial lemniscus (ML) caused by TPTs and to demonstrate the application of HDFT in preoperative planning...
February 2017: World Neurosurgery
https://www.readbyqxmd.com/read/27881822/lowered-h3k27me3-and-dna-hypomethylation-define-poorly-prognostic-pediatric-posterior-fossa-ependymomas
#11
Jill Bayliss, Piali Mukherjee, Chao Lu, Siddhant U Jain, Chan Chung, Daniel Martinez, Benjamin Sabari, Ashley S Margol, Pooja Panwalkar, Abhijit Parolia, Melike Pekmezci, Richard C McEachin, Marcin Cieslik, Benita Tamrazi, Benjamin A Garcia, Gaspare La Rocca, Mariarita Santi, Peter W Lewis, Cynthia Hawkins, Ari Melnick, C David Allis, Craig B Thompson, Arul M Chinnaiyan, Alexander R Judkins, Sriram Venneti
Childhood posterior fossa (PF) ependymomas cause substantial morbidity and mortality. These tumors lack recurrent genetic mutations, but a subset of these ependymomas exhibits CpG island (CpGi) hypermethylation [PF group A (PFA)], implicating epigenetic alterations in their pathogenesis. Further, histological grade does not reliably predict prognosis, highlighting the importance of developing more robust prognostic markers. We discovered global H3K27me3 reduction in a subset of these tumors (PF-ve ependymomas) analogous to H3K27M mutant gliomas...
November 23, 2016: Science Translational Medicine
https://www.readbyqxmd.com/read/27742374/angiocentric-glioma-transformed-into-anaplastic-ependymoma-review-of-the-evidence-for-malignant-potential
#12
REVIEW
James A McCracken, Michael F Gonzales, Pramit M Phal, Katharine J Drummond
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...
December 2016: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
https://www.readbyqxmd.com/read/27659834/epilepsy-surgery-for-pediatric-low-grade-gliomas-of-the-cerebral-hemispheres-neurosurgical-considerations-and-outcomes
#13
REVIEW
Matthew T Brown, Frederick A Boop
INTRODUCTION: Pediatric low-grade tumors are found in roughly 1-3 % of patients with childhood epilepsy; seizures associated with these tumors are often medically refractory and often present a significant morbidity, greater than the presence of the tumor itself. DISCUSSION: The unique morbidity of the seizures often requires an epilepsy surgical approach over a standard oncologic resection to achieve a reduction in morbidity for the child. Multiple quality-of-life studies have shown that unless a patient is seizure-free, they remain disabled throughout their life; the best way to achieve this in our patient population is with a multidisciplinary team approach with treatment goals focusing primarily on the epilepsy...
October 2016: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/27659832/neurosurgical-tools-to-extend-tumor-resection-in-hemispheric-low-grade-gliomas-conventional-and-contrast-enhanced-ultrasonography
#14
REVIEW
Luca Mattei, Francesco Prada, Federico Giuseppe Legnani, Alessandro Perin, Alessandro Olivi, Francesco DiMeco
PURPOSE: Pediatric low-grade gliomas (LGGs) are the most frequent solid tumor in childhood. Based on an increasing number of literature reports, maximal safe resection is recommended as the first line of treatment whenever possible. However, distinguishing tumor tissue from the surrounding normal brain is often challenging with infiltrating neoplasms, even with the assistance of intraoperative, microscopic and conventional neuronavigation systems. Therefore, any technique that enhances the detection and visualization of LGGs intraoperatively is certainly desirable...
October 2016: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/27616346/clinical-trials-in-pediatric-neuro-oncology-what-is-missing-and-how-we-can-improve
#15
REVIEW
Lennox Byer, Cassie Kline, Sabine Mueller
Brain tumors are the most common solid tumor in childhood, yet outcomes vary dramatically. High-grade gliomas have dismal outcomes with poor survival. By contrast, low-grade gliomas, have high survival rates, but children suffer from morbidity of tumor burden and therapy-associated side effects. In this article, we discuss how current trial designs often miss the opportunity to include end points beyond tumor response and thus fail to offer complete assessments of therapeutic approaches. Quality of life, neurocognitive function and neurofunctional deficits need to be considered when assessing overall success of a therapy...
October 2016: CNS Oncology
https://www.readbyqxmd.com/read/27577993/targeted-detection-of-genetic-alterations-reveal-the-prognostic-impact-of-h3k27m-and-mapk-pathway-aberrations-in-paediatric-thalamic-glioma
#16
Scott Ryall, Rahul Krishnatry, Anthony Arnoldo, Pawel Buczkowicz, Matthew Mistry, Robert Siddaway, Cino Ling, Sanja Pajovic, Man Yu, Joshua B Rubin, Juliette Hukin, Paul Steinbok, Ute Bartels, Eric Bouffet, Uri Tabori, Cynthia Hawkins
Paediatric brain tumours arising in the thalamus present significant diagnostic and therapeutic challenges to physicians due to their sensitive midline location. As such, genetic analysis for biomarkers to aid in the diagnosis, prognosis and treatment of these tumours is needed. Here, we identified 64 thalamic gliomas with clinical follow-up and characterized targeted genomic alterations using newly optimized droplet digital and NanoString-based assays. The median age at diagnosis was 9.25 years (range, 0...
August 31, 2016: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/27311729/cognitive-outcomes-among-survivors-of-focal-low-grade-brainstem-tumors-diagnosed-in-childhood
#17
Kellie N Clark, Jason M Ashford, Atmaram S Pai Panandiker, Paul Klimo, Thomas E Merchant, Catherine A Billups, Heather M Conklin
Pediatric focal low-grade brainstem tumors are associated with excellent prognosis. Surgical resection and conformal radiation therapy are front-line treatment options; radiation therapy (RT) serves as an excellent treatment for disease progression. Given high survival rates and limited research regarding functional outcomes, the current study examined neurocognitive outcomes in a group of low-grade brainstem glioma survivors. Forty-three survivors of focal low-grade brainstem gliomas underwent neurocognitive assessment (58 % male; median = 6...
September 2016: Journal of Neuro-oncology
https://www.readbyqxmd.com/read/27123665/pediatric-pleomorphic-xanthoastrocytoma-treated-with-surgical-resection-alone-clinicopathologic-features
#18
Andrew J Dodgshun, Alexandra Sexton-Oates, Richard Saffery, Duncan MacGregor, Michael J Sullivan
Pleomorphic xanthoastrocytoma (PXA) is a rare brain tumor that usually occurs in children and young adults. It has characteristic histologic features and is regarded as a WHO grade II lesion. Overall survival is reported to be >60%, but published series usually consist of a range of ages and treatment modalities. Gross total resection is associated with superior survival but recurrence rates after gross total resection are not well described, particularly in a pediatric population. We describe 16 cases over 20 years at our institution of pediatric PXA treated with surgical resection alone with a 5-year relapse-free survival of 40% (95% confidence interval, 20%-82%) and overall survival of 76% (95% confidence interval, 55%-100%)...
October 2016: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/27034984/mutations-in-chromatin-machinery-and-pediatric-high-grade-glioma
#19
REVIEW
Rishi R Lulla, Amanda Muhs Saratsis, Rintaro Hashizume
Pediatric central nervous system tumors are the most common solid tumor of childhood. Of these, approximately one-third are gliomas that exhibit diverse biological behaviors in the unique context of the developing nervous system. Although low-grade gliomas predominate and have favorable outcomes, up to 20% of pediatric gliomas are high-grade. These tumors are a major contributor to cancer-related morbidity and mortality in infants, children, and adolescents, with long-term survival rates of only 10 to 15%. The recent discovery of somatic oncogenic mutations affecting chromatin regulation in pediatric high-grade glioma has markedly improved our understanding of disease pathogenesis, and these findings have stimulated the development of novel therapeutic approaches targeting epigenetic regulators for disease treatment...
March 2016: Science Advances
https://www.readbyqxmd.com/read/27020562/neuro-oncology-long-term-outcomes-in-childhood-low-grade-glioma
#20
Hemi Malkki
No abstract text is available yet for this article.
April 2016: Nature Reviews. Neurology
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