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Pediatric brain tumors

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https://www.readbyqxmd.com/read/29163818/pediatric-brain-tumor-cells-release-exosomes-with-a-mirna-repertoire-that-differs-from-exosomes-secreted-by-normal-cells
#1
Ágota Tűzesi, Teresia Kling, Anna Wenger, Taral R Lunavat, Su Chul Jang, Bertil Rydenhag, Jan Lötvall, Steven M Pollard, Anna Danielsson, Helena Carén
High-grade gliomas (HGGs) are very aggressive brain tumors with a cancer stem cell component. Cells, including cancer stem cells, release vesicles called exosomes which contain small non-coding RNAs such as microRNAs (miRNAs). These are thought to play an important role in cell-cell communication. However, we have limited knowledge of the types of exosomal miRNAs released by pediatric HGG stem cells; a prerequisite for exploring their potential roles in HGG biology. Here we isolated exosomes released by pediatric glioma stem cells (GSCs) and compared their repertoire of miRNAs to genetically normal neural stem cells (NSCs) exosomes, as well as their respective cellular miRNA content...
October 27, 2017: Oncotarget
https://www.readbyqxmd.com/read/29163208/circadian-alterations-in-a-murine-model-of-hypothalamic-glioma
#2
José M Duhart, Lucila Brocardo, Carlos S Caldart, Luciano Marpegan, Diego A Golombek
The mammalian circadian system is controlled by a central oscillator located in the suprachiasmatic nuclei (SCN) of the hypothalamus, in which glia appears to play a prominent role. Gliomas originate from glial cells and are the primary brain tumors with the highest incidence and mortality. Optic pathway/hypothalamic gliomas account for 4-7% of all pediatric intracranial tumors. Given the anatomical location, which compromises both the circadian pacemaker and its photic input pathway, we decided to study whether the presence of gliomas in the hypothalamic region could alter circadian behavioral outputs...
2017: Frontiers in Physiology
https://www.readbyqxmd.com/read/29161788/aggressive-supratentorial-ependymoma-rela-fusion-positive-with-extracranial-metastasis-a-case-report
#3
Seong-Ik Kim, Yoojin Lee, Seung Ki Kim, Hyoung Jin Kang, Sung-Hye Park
Ependymoma is the third most common pediatric primary brain tumor. Ependymomas are categorized according to their locations and genetic abnormalities, and these two parameters are important prognostic factors for patient outcome. For supratentorial (ST) ependymomas, RELA fusion-positive ependymomas show a more aggressive behavior than YAP1 fusion-positive ependymomas. Extracranial metastases of intra-axial neuroepithelial tumors are extremely rare. In this paper, we report a case of aggressive anaplastic ependymoma arising in the right frontoparietal lobe, which had genetically 1q25 gain, CDKN2A homozygous deletion, and L1CAM overexpression...
November 2017: Journal of Pathology and Translational Medicine
https://www.readbyqxmd.com/read/29161113/disseminability-of-computerized-cognitive-training-performance-across-coaches
#4
Ashley S Fournier-Goodnight, Jason M Ashford, Kellie N Clark, Karen Martin-Elbahesh, Kristina K Hardy, Thomas E Merchant, Sima Jeha, Robert J Ogg, Hui Zhang, Lei Wang, Heather M Conklin
Cogmed is a computerized cognitive intervention utilizing coaches who receive standardized instruction in analyzing training indices and tailoring feedback to remotely monitor participant's performance. The goal of this study was to examine adherence, satisfaction, and efficacy of Cogmed across coaches. Survivors of pediatric brain tumors and acute lymphoblastic leukemia (N = 68) were randomized to intervention (Cogmed) or waitlist control. The intervention group was matched with one of two coaches. Cognitive assessments were completed before and after intervention, and participants and caregivers in the intervention group completed satisfaction surveys...
November 21, 2017: Applied Neuropsychology. Child
https://www.readbyqxmd.com/read/29159601/activating-fgfr1-mutations-in-sporadic-pheochromocytomas
#5
Jenny Welander, Małgorzata Łysiak, Michael Brauckhoff, Laurent Brunaud, Peter Söderkvist, Oliver Gimm
INTRODUCTION: Pheochromocytomas are neuroendocrine tumors of the adrenal glands. Up to 40% of the cases are caused by germline mutations in one of at least 15 susceptibility genes, making them the human neoplasms with the highest degree of heritability. Recurrent somatic alterations are found in about 50% of the more common sporadic tumors with NF1 being the most common mutated gene (20-25%). In many sporadic tumors, however, a genetic explanation is still lacking. MATERIALS AND METHODS: We investigated the genomic landscape of sporadic pheochromocytomas with whole-exome sequencing of 16 paired tumor and normal DNA samples and extended confirmation analysis in 2 additional cohorts comprising a total of 80 sporadic pheochromocytomas...
November 20, 2017: World Journal of Surgery
https://www.readbyqxmd.com/read/29152094/xenotransplantation-of-pediatric-low-grade-gliomas-confirms-the-enrichment-of-braf-v600e-mutation-and-preservation-of-cdkn2a-deletion-in-a-novel-orthotopic-xenograft-mouse-model-of-progressive-pleomorphic-xanthoastrocytoma
#6
Mari Kogiso, Lin Qi, Holly Lindsay, Yulun Huang, Xiumei Zhao, Zhigang Liu, Frank K Braun, Yuchen Du, Huiyuan Zhang, Goeun Bae, Sibo Zhao, Sarah G Injac, Mary Sobieski, David Brunell, Vidya Mehta, Diep Tran, Jeffrey Murray, Patricia A Baxter, Xiao-Jun Yuan, Jack M Su, Adekunle Adesina, Laszlo Perlaky, Murali Chintagumpala, D Williams Parsons, Ching C Lau, Clifford C Stephan, Xinyan Lu, Xiao-Nan Li
To identify cellular and molecular changes that driver pediatric low grade glioma (PLGG) progression, we analyzed putative cancer stem cells (CSCs) and evaluated key biological changes in a novel and progressive patient-derived orthotopic xenograft (PDOX) mouse model. Flow cytometric analysis of 22 PLGGs detected CD133(+) (<1.5%) and CD15(+) (20.7 ± 28.9%) cells, and direct intra-cranial implantation of 25 PLGGs led to the development of 1 PDOX model from a grade II pleomorphic xanthoastrocytoma (PXA). While CSC levels did not correlate with patient tumor progression, neurosphere formation and in vivo tumorigenicity, the PDOX model, IC-3635PXA, reproduced key histological features of the original tumor...
October 20, 2017: Oncotarget
https://www.readbyqxmd.com/read/29143958/intraspinal-mesenchymal-chondrosarcoma-report-of-a-pediatric-case-and-literature-review
#7
Angela Di Giannatale, Marta Colletti, Ida Russo, Valentina Ferruzzi, Vito Andrea Dell' Anna, Raffaele Cozza, Giovanna Stefania Colafati, Raffaella Messina, Angela Mastronuzzi, Rita De Vito, Giuseppe Maria Milano
PURPOSE: Mesenchymal chondrosarcoma (MCS) is an aggressive variant of chondrosarcoma and is a rare tumor, particularly within the pediatric population. Commonly, MCS originates in the bone, but it can also arise in extraskeletal sites, such as the brain and the intraspinal area. Due to the rarity of this tumor, there are no guidelines for its optimal treatment. METHODS: We report a case of intradural extramedullary MCS, located at the T11-T12 level, in a 14-year-old male...
November 15, 2017: Tumori
https://www.readbyqxmd.com/read/29143922/pre-treatment-lymphopenia-and-indication-of-tumor-induced-systemic-immunosuppression-in-medulloblastoma
#8
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/29135108/addressing-the-post-irradiation-hypothalamic-pituitary-endocrine-abnormalities-of-brain-tumors-in-pediatric-patients
#9
Louloudenia Velentza, Maria Tolia, Charikleia Christakou, Michail Nikolaou, Ioannis Zerdes, Nikolaos Tsoukalas, Jiannis Hajiioannou, Konstantinos Tsanadis, Georgios Rigas, Michail Mitsis, Kyriaki Theodorou, Kyriaki Pistevou-Gombaki, Periklis Tsekeris, George Kyrgias
PURPOSE: Hypothalamic-pituitary axis is susceptible to radiotherapy, causing endocrine disorders to childhood cancer survivors. We conducted a systematic review in order to assess the radiation-induced toxicity that leads to hormone secretion abnormalities and their severity in children with brain tumors. METHODS: The data were collected by relevant studies on PubMed and EMBASE. Articles up to December 2016 were included. We selected studies which focused on children patients (<18 yr old) with brain tumors treated with radiotherapy and the consequences for their endocrine system...
September 2017: Journal of B.U.ON.: Official Journal of the Balkan Union of Oncology
https://www.readbyqxmd.com/read/29131101/outcomes-and-prognostic-factors-in-pediatric-oligodendroglioma-a-population-based-study
#10
Nicholas J Goel, Kalil G Abdullah, Shih-Shan Lang
BACKGROUND/AIMS: Pediatric oligodendroglioma (pODG) is a rare primary brain tumor that remains poorly understood. Demographics, outcomes, and prognostic factors were analyzed in 346 pODG cases from the Surveillance, Epidemiology, and End Results database. METHODS: Gender, race, age, tumor location, tumor size, tumor grade, extent of resection, and use of radiotherapy were evaluated with respect to overall survival (OS) by univariate and multivariate analysis. These factors were assessed in the pediatric cohort and 5,753 adult oligodendroglioma cases for comparison...
November 2, 2017: Pediatric Neurosurgery
https://www.readbyqxmd.com/read/29125440/long-term-outcomes-of-primarily-metastatic-juvenile-pilocytic-astrocytoma-in-children
#11
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/29124086/the-pediatric-ependymoma-protein-database-pepd
#12
George Th Tsangaris, Athanasios K Anagnostopoulos
Proteomics, through application of modern high-end mass spectrometric (MS) approaches, offers the advantage of in-depth analysis of cancer tissues regarding their protein composition. Pediatric brain tumor malignancies are scarcely approached by modern holistic technologies (e.g. genomics, proteomics) due to rarity of samples and most importantly difficulty in their collection. Ependymoma, is the third most common tumor in children and is thought to arise from ependymal cells in the wall of the cerebral ventricles or the spinal canal...
December 2017: Data in Brief
https://www.readbyqxmd.com/read/29119343/long-term-survivors-of-childhood-brain-tumors-impact-on-general-health-and-quality-of-life
#13
REVIEW
Priyamvada Gupta, Rakesh Jalali
PURPOSE OF REVIEW: We review and summarize the key issues affecting general health and quality of life (QOL) of pediatric long-term survivors of brain tumors. RECENT FINDINGS: Long-term survivors of brain tumors are at risk of considerable late morbidity and mortality. Lengthening survival in brain tumors has highlighted the deep impact of tumor and its treatment on the physical, psychological, functional, and social health and QOL of these survivors. Evolution in tumor therapy including surgery, radiotherapy, and systemic therapies, etc...
November 8, 2017: Current Neurology and Neuroscience Reports
https://www.readbyqxmd.com/read/29118478/intraoperative-squash-smear-cytology-in-cns-lesions-a-study-of-150-pediatric-cases
#14
Arpita Jindal, Kanwalpreet Kaur, Kusum Mathur, Vinod Kumari, Himanshi Diwan
Background: Tumors of the central nervous system in the pediatric age group occur relatively frequently during the early years of life. Brain tumors are the most common solid malignancies of childhood and only second to acute childhood leukemia. Squash cytology is an indispensable diagnostic aid to central nervous system (CNS) lesions. The definitive diagnosis of brain lesions is confirmed by histological examination. Aim: To study the cytology of CNS lesions in pediatric population and correlate it with histopathology...
October 2017: Journal of Cytology
https://www.readbyqxmd.com/read/29099739/4sc-202-as-a-potential-treatment-for-the-pediatric-brain-tumor-medulloblastoma
#15
Shanta M Messerli, Mariah M Hoffman, Etienne Z Gnimpieba, Hella Kohlhof, Ratan D Bhardwaj
This project involves an examination of the effect of the small molecule inhibitor 4SC-202 on the growth of the pediatric brain cancer medulloblastoma. The small molecule inhibitor 4SC-202 significantly inhibits the viability of the pediatric desmoplastic cerebellar human medulloblastoma cell line DAOY, with an IC50 = 58.1 nM, but does not affect the viability of noncancerous neural stem cells (NSC). 4SC-202 exposure inhibits hedgehog expression in the DAOY cell line. Furthermore, microarray analysis of human medulloblastoma patient tumors indicate significant upregulation of key targets in the Hedgehog signaling pathway and Protein Tyrosine Kinase (PTK7)...
November 3, 2017: Brain Sciences
https://www.readbyqxmd.com/read/29099727/supplemental-computational-phantoms-to-estimate-out-of-field-absorbed-dose-in-photon-radiotherapy
#16
Kyle Joseph Gallagher, Jaad Tannous, Racile Nabha, Joelle Ann Feghali, Zeina Ayoub, Wassim T Jalbout, Bassem Youssef, Phillip J Taddei
The purpose of this study was to develop a straightforward method of supplementing patient anatomy and estimating out-of-field absorbed dose for a cohort of pediatric radiotherapy patients with limited recorded anatomy. A cohort of nine children, aged 2 to 14 years, who received three-dimensional conformal radiotherapy for low grade localized brain tumors (LBTs), were randomly selected for this study. The extents of these patients' computed tomography simulation image sets were cranial only. To approximate their missing anatomy, we supplemented the LBT patients' image sets with computed tomography images of patients in a previous study with larger extents of matched sex, height, and mass and for whom contours of organs at risk for radiogenic cancer had already been delineated...
November 3, 2017: Physics in Medicine and Biology
https://www.readbyqxmd.com/read/29093006/rapid-intraoperative-diagnosis-of-pediatric-brain-tumors-using-stimulated-raman-histology
#17
Todd C Hollon, Spencer Lewis, Balaji Pandian, Yashar S Niknafs, Mia R Garrard, Hugh Garton, Cormac O Maher, Kathryn McFadden, Matija Snuderl, Andrew P Lieberman, Karin Muraszko, Sandra Camelo-Piragua, Daniel A Orringer
Accurate histopathologic diagnosis is essential for providing optimal surgical management of pediatric brain tumors. Current methods for intraoperative histology are time- and labor-intensive and often introduce artifacts that limit interpretation. Stimulated Raman histology (SRH) is a novel label-free imaging technique that provides intraoperative histologic images of fresh, unprocessed surgical specimens. Here we evaluate the capacity of SRH for use in the intraoperative diagnosis of pediatric type brain tumors...
November 1, 2017: Cancer Research
https://www.readbyqxmd.com/read/29090526/a-pediatric-brain-tumor-consortium-phase-ii-trial-of-capecitabine-rapidly-disintegrating-tablets-with-concomitant-radiation-therapy-in-children-with-newly-diagnosed-diffuse-intrinsic-pontine-gliomas
#18
Lindsay B Kilburn, Mehmet Kocak, Patricia Baxter, Tina Young Poussaint, Arnold C Paulino, Christine McIntyre, Annabelle Lemenuel-Diot, Christine Lopez-Diaz, Larry Kun, Murali Chintagumpala, Jack M Su, Alberto Broniscer, Justin N Baker, Eugene I Hwang, Maryam Fouladi, James M Boyett, Susan M Blaney
BACKGROUND: We conducted a phase II study of oral capecitabine rapidly disintegrating tablets given concurrently with radiation therapy (RT) to assess progression-free survival (PFS) in children with newly diagnosed diffuse intrinsic pontine gliomas (DIPG). PATIENTS AND METHODS: Children 3-17 years with newly diagnosed DIPG were eligible. Capecitabine, 650 mg/m(2) /dose BID (maximum tolerated dose [MTD] in children with concurrent radiation), was administered for 9 weeks starting the first day of RT...
November 1, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/29079783/nanog-driven-cell-reprogramming-and-self-renewal-maintenance-in-ptch1-granule-cell-precursors-after-radiation-injury
#19
Barbara Tanno, Simona Leonardi, Gabriele Babini, Paola Giardullo, Ilaria De Stefano, Emanuela Pasquali, Anna Saran, Mariateresa Mancuso
Medulloblastoma (MB) is the most common pediatric brain tumor, comprising four distinct molecular variants, one of which characterized by activation of the Sonic Hedgehog (SHH) pathway, driving 25-30% of sporadic MB. SHH-dependent MBs arise from granule cell precursors (GCPs), are fatal in 40-70% of cases and radioresistance strongly contributes to poor prognosis and tumor recurrence. Patched1 heterozygous (Ptch1 (+/-)) mice, carrying a germ-line heterozygous inactivating mutation in the Ptch1 gene, the Shh receptor and negative regulator of the pathway, are uniquely susceptible to MB development after radiation damage in neonatal cerebellum...
October 27, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29071526/the-ubiquitin-proteasome-pathway-in-adult-and-pediatric-brain-tumors-biological-insights-and-therapeutic-opportunities
#20
Wafik Zaky, Christa Manton, Claudia P Miller, Soumen Khatua, Vidya Gopalakrishnan, Joya Chandra
Nearly 20 years ago, the concept of targeting the proteasome for cancer therapy began gaining momentum. This concept was driven by increased understanding of the biology/structure and function of the 26S proteasome, insight into the role of the proteasome in transformed cells, and the synthesis of pharmacological inhibitors with clinically favorable features. Subsequent in vitro, in vivo, and clinical testing culminated in the FDA approval of three proteasome inhibitors-bortezomib, carfilzomib, and ixazomib -for specific hematological malignancies...
October 25, 2017: Cancer Metastasis Reviews
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