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Pediatric brain tumors

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https://www.readbyqxmd.com/read/29776650/signaling-pathway-deregulation-and-molecular-alterations-across-pediatric-medulloblastomas
#1
REVIEW
B Lhermitte, A F Blandin, A Coca, E Guerin, A Durand, N Entz-Werlé
Medulloblastomas (MBs) account for 15% of brain tumors in children under the age of 15. To date, the overall 5-year survival rate for all children is only around 60%. Recent advances in cancer genomics have led to a fundamental change in medulloblastoma classification and is evolving along with the genomic discoveries, allowing to regularly reclassify this disease. The previous molecular classification defined 4 groups (WNT-activated MB, SHH-activated MB and the groups 3 and 4 characterized partially by NMYC and MYC driven MBs)...
May 15, 2018: Neuro-Chirurgie
https://www.readbyqxmd.com/read/29774098/establishment-of-primary-cell-culture-and-an-intracranial-xenograft-model-of-pediatric-ependymoma-a-prospect-for-therapy-development-and-understanding-of-tumor-biology
#2
Lorena Favaro Pavon, Tatiana Tais Sibov, Silvia Regina Caminada de Toledo, Daniela Mara de Oliveira, Francisco Romero Cabral, Jean Gabriel de Souza, Pamela Boufleur, Luciana C Marti, Jackeline Moraes Malheiros, Edgar Ferreira da Cruz, Fernando F Paiva, Suzana M F Malheiros, Manoel A de Paiva Neto, Alberto Tannús, Sérgio Mascarenhas de Oliveira, Nasjla Saba Silva, Andrea Maria Cappellano, Antonio Sérgio Petrilli, Ana Marisa Chudzinski-Tavassi, Sérgio Cavalheiro
Background: Ependymoma (EPN), the third most common pediatric brain tumor, is a central nervous system (CNS) malignancy originating from the walls of the ventricular system. Surgical resection followed by radiation therapy has been the primary treatment for most pediatric intracranial EPNs. Despite numerous studies into the prognostic value of histological classification, the extent of surgical resection and adjuvant radiotherapy, there have been relatively few studies into the molecular and cellular biology of EPNs...
April 24, 2018: Oncotarget
https://www.readbyqxmd.com/read/29773567/balanced-steady-state-free-precession-sequence-ciss-fiesta-3d-driven-equilibrium-radiofrequency-reset-pulse-increases-the-diagnostic-yield-for-spinal-drop-metastases-in-children-with-brain-tumors
#3
K Buch, P Caruso, D Ebb, S Rincon
BACKGROUND AND PURPOSE: Identification of spinal drop metastases is important in the staging and management of pediatric patients with primary brain tumors. Our aim was to assess the diagnostic utility of the balanced steady-state free precession (bSSFP) sequence (CISS/FIESTA/3D driven equilibrium radiofrequency reset pulse) for the detection of spinal drop metastases in pediatric patients with primary intracranial tumors. MATERIALS AND METHODS: This was a retrospective study of 44 pediatric patients with primary intracranial tumors undergoing MR imaging spine evaluation for drop metastases before radiation treatment...
May 17, 2018: AJNR. American Journal of Neuroradiology
https://www.readbyqxmd.com/read/29768295/requirement-for-head-magnetic-resonance-imaging-in-children-who-present-to-the-emergency-department-with-acute-nontraumatic-visual-disturbance
#4
Jeong-Yong Lee, Jung-Heon Kim, Hyung-Rae Cho, Jong-Seung Lee, Jeong-Min Ryu, Mi-Sun Yum, Tae-Sung Ko
OBJECTIVES: This study aimed to investigate the clinical features and head magnetic resonance imaging (MRI) findings in children who presented to the emergency department with acute nontraumatic visual disturbance and to study related clinical factors for discovering positive lesions on head MRI. METHODS: We performed a retrospective study of 1-month to 15-year-old children who underwent head MRI as an evaluation for acute nontraumatic visual disturbance as a chief complaint in our pediatric emergency department between March 2010 and March 2015...
May 15, 2018: Pediatric Emergency Care
https://www.readbyqxmd.com/read/29758594/phox2b-is-a-reliable-immunomarker-in-distinguishing-peripheral-neuroblastic-tumors-from-cns-embryonal-tumors
#5
Sanda Alexandrescu, Vera Paulson, Adrian Dubuc, Azra Ligon, Hart G Lidov
INTRODUCTION: The PHOX2B gene regulates neuronal maturation in the brain stem nuclei associated with cardiorespiratory function, and in the autonomic sympathetic and enteric nervous system. PHOX2B expression is a reliable immunomarker for peripheral neuroblastic tumors, however no systematic evaluation of CNS embryonal tumors was included in the studies. We encountered two cases in which the differential diagnosis included neuroblastoma and CNS embryonal tumor, and we hypothesized that PHOX2B immunostain would be helpful establishing the diagnosis...
May 14, 2018: Histopathology
https://www.readbyqxmd.com/read/29753700/spectrum-and-prevalence-of-genetic-predisposition-in-medulloblastoma-a-retrospective-genetic-study-and-prospective-validation-in-a-clinical-trial-cohort
#6
Sebastian M Waszak, Paul A Northcott, Ivo Buchhalter, Giles W Robinson, Christian Sutter, Susanne Groebner, Kerstin B Grund, Laurence Brugières, David T W Jones, Kristian W Pajtler, A Sorana Morrissy, Marcel Kool, Dominik Sturm, Lukas Chavez, Aurelie Ernst, Sebastian Brabetz, Michael Hain, Thomas Zichner, Maia Segura-Wang, Joachim Weischenfeldt, Tobias Rausch, Balca R Mardin, Xin Zhou, Cristina Baciu, Christian Lawerenz, Jennifer A Chan, Pascale Varlet, Lea Guerrini-Rousseau, Daniel W Fults, Wiesława Grajkowska, Peter Hauser, Nada Jabado, Young-Shin Ra, Karel Zitterbart, Suyash S Shringarpure, Francisco M De La Vega, Carlos D Bustamante, Ho-Keung Ng, Arie Perry, Tobey J MacDonald, Pablo Hernáiz Driever, Anne E Bendel, Daniel C Bowers, Geoffrey McCowage, Murali M Chintagumpala, Richard Cohn, Timothy Hassall, Gudrun Fleischhack, Tone Eggen, Finn Wesenberg, Maria Feychting, Birgitta Lannering, Joachim Schüz, Christoffer Johansen, Tina V Andersen, Martin Röösli, Claudia E Kuehni, Michael Grotzer, Kristina Kjaerheim, Camelia M Monoranu, Tenley C Archer, Elizabeth Duke, Scott L Pomeroy, Redmond Shelagh, Stephan Frank, David Sumerauer, Wolfram Scheurlen, Marina V Ryzhova, Till Milde, Christian P Kratz, David Samuel, Jinghui Zhang, David A Solomon, Marco Marra, Roland Eils, Claus R Bartram, Katja von Hoff, Stefan Rutkowski, Vijay Ramaswamy, Richard J Gilbertson, Andrey Korshunov, Michael D Taylor, Peter Lichter, David Malkin, Amar Gajjar, Jan O Korbel, Stefan M Pfister
BACKGROUND: Medulloblastoma is associated with rare hereditary cancer predisposition syndromes; however, consensus medulloblastoma predisposition genes have not been defined and screening guidelines for genetic counselling and testing for paediatric patients are not available. We aimed to assess and define these genes to provide evidence for future screening guidelines. METHODS: In this international, multicentre study, we analysed patients with medulloblastoma from retrospective cohorts (International Cancer Genome Consortium [ICGC] PedBrain, Medulloblastoma Advanced Genomics International Consortium [MAGIC], and the CEFALO series) and from prospective cohorts from four clinical studies (SJMB03, SJMB12, SJYC07, and I-HIT-MED)...
May 9, 2018: Lancet Oncology
https://www.readbyqxmd.com/read/29742889/intraoperative-neurophysiological-monitoring-a-review-of-techniques-used-for-brain-tumor-surgery-in-children
#7
REVIEW
Keewon Kim, Charles Cho, Moon-Suk Bang, Hyung-Ik Shin, Ji-Hoon Phi, Seung-Ki Kim
Intraoperative monitoring (IOM) utilizes electrophysiological techniques as a surrogate test and evaluation of nervous function while a patient is under general anesthesia. They are increasingly used for procedures, both surgical and endovascular, to avoid injury during an operation, examine neurological tissue to guide the surgery, or to test electrophysiological function to allow for more complete resection or corrections. The application of IOM during pediatric brain tumor resections encompasses a unique set of technical issues...
May 2018: Journal of Korean Neurosurgical Society
https://www.readbyqxmd.com/read/29742887/recent-advancement-of-the-molecular-diagnosis-in-pediatric-brain-tumor
#8
REVIEW
Jeong-Mo Bae, Jae-Kyung Won, Sung-Hye Park
Recent discoveries of brain tumor-related genes and fast advances in genomic testing technologies have led to the era of molecular diagnosis of brain tumor. Molecular profiling of brain tumor became the significant step in the diagnosis, the prediction of prognosis and the treatment of brain tumor. Because traditional molecular testing methods have limitations in time and cost for multiple gene tests, next-generation sequencing technologies are rapidly introduced into clinical practice. Targeted sequencing panels using these technologies have been developed for brain tumors...
May 2018: Journal of Korean Neurosurgical Society
https://www.readbyqxmd.com/read/29742885/malignant-brain-tumours-in-children-present-and-future-perspectives
#9
REVIEW
James T Rutka
In contrast to many of the malignant tumors that occur in the central nervous system in adults, the management, responses to therapy, and future perspectives of children with malignant lesions of the brain hold considerable promise. Within the past 5 years, remarkable progress has been made with our understanding of the basic biology of the molecular genetics of several pediatric malignant brain tumors including medulloblastoma, ependymoma, atypical teratoid rhabdoid tumour, and high grade glioma/diffuse intrinsic pontine glioma...
May 2018: Journal of Korean Neurosurgical Society
https://www.readbyqxmd.com/read/29742882/pediatric-high-grade-gliomas-in-the-context-of-cancer-predisposition-syndromes
#10
REVIEW
Orli Michaeli, Uri Tabori
Germline mutations in cancer causing genes result in high risk of developing cancer throughout life. These cancer predisposition syndromes (CPS) are especially prevalent in childhood brain tumors and impact both the patient's and other family members' survival. Knowledge of specific CPS may alter the management of the cancer, offer novel targeted therapies which may improve survival for these patients, and enables early detection of other malignancies. This review focuses on the role of CPS in pediatric high grade gliomas (PHGG), the deadliest group of childhood brain tumors...
May 2018: Journal of Korean Neurosurgical Society
https://www.readbyqxmd.com/read/29730815/pd-1-pd-l1-and-immune-related-gene-expression-pattern-in-pediatric-malignant-brain-tumors-clinical-correlation-with-survival-data-in-korean-population
#11
Kihwan Hwang, Eun Jung Koh, Eun Jin Choi, Tae Hee Kang, Jung Ho Han, Gheeyoung Choe, Sung-Hye Park, Jennifer Holmes Yearley, Lakshmanan Annamalai, Wendy Blumenschein, Manjiri Sathe, Terri McClanahan, Hun Jung, Kyu-Chang Wang, Seung-Ki Kim, Chae-Yong Kim
BACKGROUND: PD-L1 expression has been evaluated as a predictive biomarker for immunotherapy in numerous tumor types. However, very limited data are available in pediatric brain tumors. The aim of this study was to characterize PD-1 and PD-L1 expressions of four pediatric malignant brain tumors and gene expression profile. METHODS: This study included 89 pediatric patients receiving standard treatment at Seoul National University Children's Hospital and Seoul National University Bundang Hospital between 1990 and 2014: atypical teratoid/rhabdoid tumor (AT/RT) 20; ependymoma (EPN) 20; high grade glioma (HGG) 21; and medulloblastoma (MBL) 28...
May 5, 2018: Journal of Neuro-oncology
https://www.readbyqxmd.com/read/29730064/risk-of-radiation-vasculopathy-and-stroke-in-pediatric-patients-treated-with-proton-therapy-for-brain-and-skull-base-tumors
#12
Matthew D Hall, Julie A Bradley, Ronny L Rotondo, Ricardo Hanel, Chetan Shah, Christopher G Morris, Philipp R Aldana, Daniel J Indelicato
PURPOSE: To estimate the rate of and identify risk factors for vasculopathy after proton therapy in pediatric patients with central nervous system and skull base tumors. METHODS AND MATERIALS: Between 2006 and 2015, 644 pediatric patients with central nervous system and skull base tumors were treated with proton therapy at a single institution. The 3 most common histologies were craniopharyngioma (n = 135), ependymoma (n = 135), and low-grade glioma (n = 131)...
March 29, 2018: International Journal of Radiation Oncology, Biology, Physics
https://www.readbyqxmd.com/read/29721192/pd-l1-expression-in-medulloblastoma-an-evaluation-by-subgroup
#13
Allison M Martin, Christopher J Nirschl, Magda J Polanczyk, W Robert Bell, Thomas R Nirschl, Sarah Harris-Bookman, Jillian Phallen, Jessica Hicks, Daniel Martinez, Aleksandra Ogurtsova, Haiying Xu, Lisa M Sullivan, Alan K Meeker, Eric H Raabe, Kenneth J Cohen, Charles G Eberhart, Peter C Burger, Mariarita Santi, Janis M Taube, Drew M Pardoll, Charles G Drake, Michael Lim
Background: This study evaluated the expression of PD-L1 and markers of immune mediated resistance in human medulloblastoma (MB), the most common malignant pediatric brain tumor. Results: Overall levels of PD-L1 in human MB were low; however, some cases demonstrated robust focal expression associated with increased immune infiltrates. The case with highest PD-L1 expression was a sonic hedgehog (SHH) MB. In cell lines, SHH MB, which are low-MYC expressing, demonstrated both constitutive and inducible expression of PD-L1 while those in Group 3/4 that expressed high levels of MYC had only inducible expression...
April 10, 2018: Oncotarget
https://www.readbyqxmd.com/read/29717567/risk-of-brain-tumor-induction-from-pediatric-head-ct-procedures-a-systematic-literature-review
#14
REVIEW
John P Sheppard, Thien Nguyen, Yasmine Alkhalid, Joel S Beckett, Noriko Salamon, Isaac Yang
Head computed tomography (CT) is instrumental for managing patients of all ages. However, its low dose radiation may pose a low but non-zero risk of tumor induction in pediatric patients. Here, we present a systematic literature review on the estimated incidence of brain tumor induction from head CT exams performed on children and adolescents. MEDLINE was searched using an electronic protocol and bibliographic searches to identify articles related to CT, cancer, and epidemiology or risk assessment. Sixteen studies that predicted or measured head CT-related neoplasm incidence or mortality were identified and reviewed...
April 2018: Brain Tumor Research and Treatment
https://www.readbyqxmd.com/read/29713910/employment-status-and-termination-among-survivors-of-pediatric-brain-tumors-a-cross-sectional-survey
#15
Iori Sato, Akiko Higuchi, Takaaki Yanagisawa, Shiho Murayama, Toshihiro Kumabe, Kazuhiko Sugiyama, Akitake Mukasa, Nobuhito Saito, Yutaka Sawamura, Mizuhiko Terasaki, Soichiro Shibui, Jun Takahashi, Ryo Nishikawa, Yasushi Ishida, Kiyoko Kamibeppu
BACKGROUND: Some childhood cancer survivors experience employment difficulties. This study aimed to describe pediatric brain-tumor survivors' employment status. METHODS: A cross-sectional, observational study was conducted, with questionnaires distributed to 101 pediatric brain-tumor survivors (aged 15 years or older) and their attending physicians from nine institutions in Japan. We compared category and time-series histories for participants' first-time employment using national census information...
April 30, 2018: International Journal of Clinical Oncology
https://www.readbyqxmd.com/read/29709735/safeness-and-utility-of-concomitant-intraoperative-monitoring-with-intraoperative-magnetic-resonance-imaging-in-children-a-pilot-study
#16
Sandra Dias, Johannes Sarnthein, Elisabeth Jehli, Marian Christoph Neidert, Luca Regli, Oliver Bozinov
OBJECTIVE: High-field intraoperative MR (ioMRI) has become increasingly available in neurosurgery centers. There is little experience with the combination of ioMRI with intraoperative neurophysiological neuromonitoring (IONM). We provide a first series of pediatric patients undergoing brain tumor surgery with 3T ioMRI and IONM. METHODS: We conducted a pilot study were we included all consecutive children operated for brain tumors between October 2013 and April 2016 where concomitant ioMRI and somatosensory evoked potentials (SEP) and motor evoked potentials (MEP) were used...
April 27, 2018: World Neurosurgery
https://www.readbyqxmd.com/read/29704232/prognostic-and-microrna-profile-analysis-for-cd44-positive-expression-pediatric-posterior-fossa-ependymoma
#17
C Shu, Q Wang, X Yan, J Wang
BACKGROUND: Ependymoma is the third most common pediatric brain tumor and occurs most frequently in the posterior fossa. However, the lack of immortalized cell lines, xenografts, or animal models has significantly hindered the study of pediatric posterior fossa ependymoma (P-PF-EPN) pathogenesis. This prompted us to use clinical big data to study this rare disease. METHODS: Application of the robust rank aggregation method revealed CD44 as a reliable biomarker in P-PF-EPN...
April 27, 2018: Clinical & Translational Oncology
https://www.readbyqxmd.com/read/29700887/cic-nutm1-fusion-a-case-which-expands-the-spectrum-of-nut-rearranged-epithelioid-malignancies
#18
Inga-Marie Schaefer, Paola Dal Cin, Christopher D M Fletcher, Glenn J Hanna, Christopher A French
NUT carcinoma (NC) shows very aggressive clinical behavior, occurs predominantly in the thorax and head and neck region of children and adults, and is defined by the presence of NUT (aka NUTM1) rearrangement, mostly BRD4-NUTM1 fusion resulting from t(15;19)(q13, p13.1). So-called "NUT variants" harbor alternate fusions between NUTM1 and BRD3, NSD3, ZNF532, or unknown partners. Rare cases of pediatric tumors with CIC-NUTM1 fusion were recently reported in somatic soft tissue, brain, and kidney. However, such cases have not been identified in adult patients and the presence of a fusion between CIC, characteristic of CIC-rearranged sarcoma, and NUTM1, a defining feature of NC - poses a diagnostic challenge...
April 26, 2018: Genes, Chromosomes & Cancer
https://www.readbyqxmd.com/read/29697883/variation-of-t-2-relaxation-times-in-pediatric-brain-tumors-and-their-effect-on-metabolite-quantification
#19
Dominic Carlin, Ben Babourina-Brooks, Nigel P Davies, Martin Wilson, Andrew C Peet
BACKGROUND: Metabolite concentrations are fundamental biomarkers of disease and prognosis. Magnetic resonance spectroscopy (MRS) is a noninvasive method for measuring metabolite concentrations; however, quantitation is affected by T2 relaxation. PURPOSE: To estimate T2 relaxation times in pediatric brain tumors and assess how variation in T2 relaxation affects metabolite quantification. STUDY TYPE: Retrospective. POPULATION: Twenty-seven pediatric brain tumor patients (n = 17 pilocytic astrocytoma and n = 10 medulloblastoma) and 24 age-matched normal controls...
April 26, 2018: Journal of Magnetic Resonance Imaging: JMRI
https://www.readbyqxmd.com/read/29692922/-18-f-fdopa-pet-mri-for-monitoring-early-response-to-bevacizumab-in-children-with-recurrent-brain-tumors
#20
Karen Gauvain, Maria Rosana Ponisio, Amy Barone, Michael Grimaldi, Ephraim Parent, Hayden Leeds, Manu Goyal, Joshua Rubin, Jonathan McConathy
Background: Noninvasively predicting early response to therapy in recurrent pediatric brain tumors provides a challenge. 3,4-dihydroxy-6-[18 F]fluoro-L-phenylalanine (18 F-FDOPA) PET/MRI has not been previously studied as a tool to evaluate early response to antiangiogenic therapy in children. The purpose of this study was to evaluate the safety and feasibility of using 18 F-FDOPA PET/MRI to assess response to bevacizumab in children with relapsed brain tumors. Materials and Methods: Six patients with recurrent gliomas (5 low-grade, 1 high-grade) planned to undergo treatment with bevacizumab were enrolled...
March 2018: Neuro-oncology Practice
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