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Brain tumors in children

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https://www.readbyqxmd.com/read/28215462/lymph-node-metastasis-of-presacral-ependymoblastoma-in-a-young-child
#1
Malak Abedalthagafi, Sadeq W Aldandan, Mohamed Alrayis, Atif A Ahmed
Ependymoblastoma presenting outside the brain is rare in children. The overwhelming majority of presacral ependymal tumors are of the benign myxpapillary type. We present a case of ependymoblastoma in the presacral region of a four-year old child. The patient presented with a presacral mass and ipsilateral inguinal lymph node metastasis. Both masses revealed histologic and immunophenotypic features of ependymoblastoma associated with myxoid areas and high Ki-67 proliferation index. The masses were excised and the patient remained well for a year after diagnosis with no other therapy...
February 16, 2017: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
https://www.readbyqxmd.com/read/28214639/congenital-glioblastoma-with-distinct-clinical-and-molecular-characteristics-case-reports-and-a-literature-review
#2
Masahiro Kameda, Yoshihiro Otani, Tomotsugu Ichikawa, Akira Shimada, Koichi Ichimura, Isao Date
BACKGROUND: The molecular diagnosis of brain tumors is important in classifying tumors and determining appropriate treatment. Congenital glioblastoma multiforme (GBM) is a rare tumor that occurs in infants, and the prognosis is poor. Approximately 60 patients diagnosed as congenital GBM have been reported. However, few reports have conducted molecular analyses of congenital GBM. CASE DESCRIPTION: We describe two congenital GBM patients treated in our hospital, and report results of immunohistochemistry, fluorescent in situ hybridization (FISH), direct sequencing, and methylation analyses...
February 15, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28205381/the-clinical-significance-of-equivocal-findings-on-spinal-mri-in-children-with-medulloblastoma
#3
Julie Bennett, Ramy Ashmawy, Vijay Ramaswamy, Derek Stephens, Eric Bouffet, Normand Laperriere, Michael Taylor, Manohar Shroff, Ute Bartels
BACKGROUND: Medulloblastoma (MB) is the most common malignant brain tumor of childhood, with cerebrospinal fluid spread the most common site of metastasis. Currently, children diagnosed with MB and evidence of spinal metastasis are treated with an increased dose of craniospinal radiation (CSI). This report reviewed equivocal abnormalities including nerve root clumping, linear vascular enhancement, nerve root enhancement and/or other vague findings on spinal magnetic resonance imaging (MRI) to elucidate their prognostic significance and aid in risk stratification...
February 16, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28205254/manganese-and-selenium-concentrations-in-cerebrospinal-fluid-of-seriously-ill-children
#4
Tomáš Franěk, Karel Kotaška, Richard Průša
BACKGROUND: The homeostasis of essential trace elements such as selenium and manganese may be altered in patients with severe diseases of various etiologies (trauma brain injuries, tumors, leukemias, lymphomas, neurological diseases). METHODS: Concentration of manganese and selenium were determined in cerebrospinal fluid by electrothermal atomic absorption spectrometry in 50 hospitalized children with various clinical ethiologies including oncological, neurological, and brain related diseases...
February 15, 2017: Journal of Clinical Laboratory Analysis
https://www.readbyqxmd.com/read/28202703/investigations-in-gabaa-receptor-antibody-associated-encephalitis
#5
Marianna Spatola, Mar Petit-Pedrol, Mateus Mistieri Simabukuro, Thaís Armangue, Fernanda J Castro, Maria I Barcelo Artigues, Maria R Julià Benique, Leslie Benson, Mark Gorman, Ana Felipe, Ruben L Caparó Oblitas, Myrna R Rosenfeld, Francesc Graus, Josep Dalmau
OBJECTIVE: To report the clinical features, comorbidities, receptor subunit targets, and outcome in patients with anti-GABAA receptor (GABAAR) encephalitis. METHODS: Clinical study of 26 patients, including 17 new (April 2013-January 2016) and 9 previously reported patients. Antibodies to α1, β3, and γ2 subunits of the GABAAR were determined using reported techniques. RESULTS: Patients' median age was 40.5 years (interquartile range 48...
February 15, 2017: Neurology
https://www.readbyqxmd.com/read/28187964/brain-perfusion-and-diffusion-abnormalities-in-children-treated-for-posterior-fossa-brain-tumors
#6
Matthew D Li, Nils D Forkert, Palak Kundu, Cheryl Ambler, Robert M Lober, Terry C Burns, Patrick D Barnes, Iris C Gibbs, Gerald A Grant, Paul G Fisher, Samuel H Cheshier, Cynthia J Campen, Michelle Monje, Kristen W Yeom
OBJECTIVE: To compare cerebral perfusion and diffusion in survivors of childhood posterior fossa brain tumor with neurologically normal controls and correlate differences with cognitive dysfunction. STUDY DESIGN: We analyzed retrospectively arterial spin-labeled cerebral blood flow (CBF) and apparent diffusion coefficient (ADC) in 21 patients with medulloblastoma (MB), 18 patients with pilocytic astrocytoma (PA), and 64 neurologically normal children. We generated ANCOVA models to evaluate treatment effects on the cerebral cortex, thalamus, caudate, putamen, globus pallidus, hippocampus, amygdala, nucleus accumbens, and cerebral white matter at time points an average of 5...
February 7, 2017: Journal of Pediatrics
https://www.readbyqxmd.com/read/28185119/biallelic-brca2-mutations-in-two-black-south-african-children-with-fanconi-anaemia
#7
Candice Feben, Careni Spencer, Anneline Lochan, Nakita Laing, Karen Fieggen, Engela Honey, Tasha Wainstein, Amanda Krause
Fanconi anaemia (FA) is a genotypically and phenotypically heterogeneous genetic condition, characterized cytogenetically by chromosomal instability and breakage secondary to impaired DNA repair mechanisms. Affected individuals typically manifest growth restriction and congenital physical abnormalities and most progress to hematological disease including bone marrow aplasia. A rare genetic subtype of FA (FA-D1) is caused by biallelic mutations in the BRCA2 gene. Affected individuals manifest severe congenital anomalies and significant pigmentary changes and are additionally at risk for early onset leukemia and certain solid organ malignancies, including Wilms tumors and brain tumors...
February 9, 2017: Familial Cancer
https://www.readbyqxmd.com/read/28178670/new-in-vivo-avatars-of-diffuse-intrinsic-pontine-gliomas-dipg-from-stereotactic-biopsies-performed-at-diagnosis
#8
Alexandre Plessier, Ludivine Le Dret, Pascale Varlet, Kévin Beccaria, Joëlle Lacombe, Sébastien Mériaux, Françoise Geffroy, Laurence Fiette, Patricia Flamant, Fabrice Chrétien, Thomas Blauwblomme, Stéphanie Puget, Jacques Grill, Marie-Anne Debily, David Castel
Diffuse Instrinsic Pontine Glioma is the most aggressive form of High Grade Gliomas in children. The lack of biological material and the absence of relevant models have hampered the development of new therapeutics. Their extensive infiltration of the brainstem renders any surgical resection impossible and until recently biopsies were considered not informative enough and therefore not recommended. Thus, most models were derived from autopsy material. We aimed to develop relevant in vivo DIPG models that mimic this specific disease and its molecular diversity from tumor material obtained at diagnosis...
February 2, 2017: Oncotarget
https://www.readbyqxmd.com/read/28176655/epigenetics-in-clinical-management-of-children-and-adolescents-with-brain-tumors
#9
Andres Morales La Madrid, Mark W Kieran
Central nervous system (CNS) tumors represent the second most prevalent group of cancers in children and adolescents, yet account for the majority of childhood cancer-related deaths and considerable morbidity among survivors, due to high-intensity non-selective standard therapies delivered to immature nervous system structures undergoing development. These tumors arise at different ages -not infrequently very early in life-, in different locations and cellular contexts, have varied cell types of origin, and have heterogeneous responses to the "classic" current therapeutic approaches...
February 3, 2017: Current Cancer Drug Targets
https://www.readbyqxmd.com/read/28174375/-characteristics-of-adolescent-and-young-adult-cancer
#10
Keizo Horibe
In adolescent and young adult(AYA)population, cancer is the leading cause of death due to illness with the lowest mortality rate as well as children, and national measures for cancer was left behind. Leukemia, lymphomas, brain tumors, thyroid cancer, ovarian cancer, cervical cancer, breast cancer, testicular cancer and bone and soft tissue sarcomas account for majority of the cancers in this age group. There has been no significant improvement in AYA patients in contrast to other age groups. AYA patients with cancer are under growing independency, starting in life to the community, and reproductive age...
January 2017: Gan to Kagaku Ryoho. Cancer & Chemotherapy
https://www.readbyqxmd.com/read/28171696/predicting-changes-in-adaptive-functioning-and-behavioral-adjustment-following-treatment-for-a-pediatric-brain-tumor-a-report-from-the-brain-radiation-investigative-study-consortium
#11
Kristen R Hoskinson, Kelly R Wolfe, Keith Owen Yeates, E Mark Mahone, Kim M Cecil, M Douglas Ris
OBJECTIVE: Children are at risk for behavioral and adaptive difficulties following pediatric brain tumor. This study explored whether familial/demographic, developmental, diagnostic, or treatment-related variables best predict post-treatment behavioral and adaptive functioning. METHODS: Participants included 40 children (mean age = 12.76 years, SD = 4.01) post-treatment (mean time since diagnosis = 1.99 years, SD = 0.21) for pediatric brain tumor...
February 7, 2017: Psycho-oncology
https://www.readbyqxmd.com/read/28167297/loss-of-pin1-suppresses-hedgehog-driven-medulloblastoma-tumorigenesis
#12
Tao Xu, Honglai Zhang, Sung-Soo Park, Sriram Venneti, Rork Kuick, Kimberly Ha, Lowell Evan Michael, Mariarita Santi, Chiyoko Uchida, Takafumi Uchida, Ashok Srinivasan, James M Olson, Andrzej A Dlugosz, Sandra Camelo-Piragua, Jean-François Rual
Medulloblastoma is the most common malignant brain tumor in children. Therapeutic approaches to medulloblastoma (combination of surgery, radiotherapy, and chemotherapy) have led to significant improvements, but these are achieved at a high cost to quality of life. Alternative therapeutic approaches are needed. Genetic mutations leading to the activation of the Hedgehog pathway drive tumorigenesis in ~30% of medulloblastoma. In a yeast two-hybrid proteomic screen, we discovered a novel interaction between GLI1, a key transcription factor for the mediation of Hedgehog signals, and PIN1, a peptidylprolyl cis/trans isomerase that regulates the postphosphorylation fate of its targets...
February 3, 2017: Neoplasia: An International Journal for Oncology Research
https://www.readbyqxmd.com/read/28156540/family-camp-a-multi-disciplinary-intervention-for-brain-tumor-patients-and-families
#13
Mary Lovely, Abigail Levinson Marks, Amanda K LaMarre, Susan M Chang, Jennifer Clarke, Judy Patt, Emelia Barani
: 240 Background: The diagnosis of a brain tumor is a catastrophic life changing event that impacts the entire family. Patients and caregivers experience dramatic role changes, concern for their children, financial stress, and isolation. For many, the situation is overwhelming. A novel intervention to address these needs is "Family Camp". Our vision for camp was to provide respite to the entire family, decrease caregiver stress, improve family connections and promote a sense of understanding and connection with the community at large...
October 9, 2016: Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology
https://www.readbyqxmd.com/read/28156496/influence-of-early-phase-clinical-trial-enrollment-on-patterns-of-end-of-life-care-for-children-with-advanced-cancer
#14
Chalinee Monsereenusorn, Clement Ma, Hasan Al-Sayegh, Joanne Wolfe, Carlos Rodriguez-Galindo
: 151 Background: Early phase clinical trials are critical to enhancing therapies for children with advanced cancer. However, trial enrollment may intensify end-of-life (EOL) care. We evaluated patterns of EOL care for patients at a large cancer center. METHODS: Single-center, retrospective cohort study of pediatric oncology patients, ages 6 months-21 years, who died in 2010-2014. We queried electronic medical records to assess frequencies of medical procedures (e...
October 9, 2016: Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology
https://www.readbyqxmd.com/read/28148893/stem-cell-cultures-derived-from-pediatric-brain-tumors-accurately-model-the-originating-tumors
#15
Anna Wenger, Susanna Larsson, Anna Danielsson, Kirstine Juul Elbæk, Petronella Kettunen, Magnus Tisell, Magnus Sabel, Birgitta Lannering, Claes Nordborg, Elizabeth Schepke, Helena Carén
Brain tumors are the leading cause of cancer-related death in children but high-grade gliomas in children and adolescents have remained a relatively under-investigated disease despite this. A better understanding of the cellular and molecular pathogenesis of the diseases is required in order to improve the outcome for these children. In vitro-cultured primary tumor cells from patients are indispensable tools for this purpose by enabling functional analyses and development of new therapies. However, relevant well-characterized in vitro cultures from pediatric gliomas cultured under serum-free conditions have been lacking...
January 26, 2017: Oncotarget
https://www.readbyqxmd.com/read/28137267/targeting-cd133-improves-chemotherapeutic-efficacy-of-recurrent-pediatric-pilocytic-astrocytoma-following-prolonged-chemotherapy
#16
Guifa Xi, Yuping Derek Li, Gordan Grahovac, Veena Rajaram, Nitin Wadhwani, Tatiana Pundy, Barbara Mania-Farnell, Charles David James, Tadanori Tomita
BACKGROUND: Pilocytic astrocytomas (PAs) are the most common pediatric central nervous system neoplasms. In the majority of cases these tumors are benign and receive favorable prognosis following gross total surgical resection. In patients with progressive or symptomatic tumors, aggressive surgical resection is generally not feasible, thus radiation or chemotherapy are accepted initial or adjuvant interventions. Due to serious long-lasting side-effects, radiation is limited in young children; therefore, chemotherapy is widely practiced as an adjuvant treatment for these patients...
January 31, 2017: Molecular Cancer
https://www.readbyqxmd.com/read/28125924/performance-based-and-parent-ratings-of-attention-in-children-treated-for-a-brain-tumor-the-significance-of-radiation-therapy-and-tumor-location-on-outcome
#17
Kimberly P Raghubar, E Mark Mahone, Keith Owen Yeates, M Douglas Ris
Children are at increased risk for cognitive difficulties following the diagnosis and treatment of a brain tumor. Radiation therapy (RT) and tumor location are commonly-cited predictors of neurocognitive functioning. Disruptions to foundational neurocognitive processes such as attention, working memory, and processing speed underlie declines on measures of general intellectual functioning. While several studies have examined visual sustained attention in pediatric brain tumor patients, auditory sustained attention has yet to be examined...
January 26, 2017: Child Neuropsychology: a Journal on Normal and Abnormal Development in Childhood and Adolescence
https://www.readbyqxmd.com/read/28120771/diffuse-intrinsic-pontine-gliomas-in-children-interest-of-robotic-frameless-assisted-biopsy-a-technical-note
#18
H A Coca, H Cebula, M Benmekhbi, M P Chenard, N Entz-Werle, F Proust
INTRODUCTION: Diffuse intrinsic pontine gliomas (DIPG) constitute 10-15% of all brain tumors in the pediatric population; currently prognosis remains poor, with an overall survival of 7-14 months. Recently the indication of DIPG biopsy has been enlarged due to the development of molecular biology and various ongoing clinical and therapeutic trials. Classically a biopsy is performed using a stereotactic frame assisted procedure but the workflow may sometimes be heavy and more complex especially in children...
December 2016: Neuro-Chirurgie
https://www.readbyqxmd.com/read/28114639/incidence-of-recurrence-in-posterior-circulation-childhood-arterial-ischemic-stroke
#19
Michael Y Uohara, Lauren A Beslow, Lori Billinghurst, Brianna M Jones, Sudha K Kessler, Daniel J Licht, Rebecca N Ichord
Importance: Childhood arterial ischemic stroke (CAIS) affects approximately 1.6 per 100 000 children per year, while stroke recurs in up to 20% of patients at 5 years. Factors determining the risk of recurrence are incompletely understood. Objective: To investigate the incidence of the recurrence of CAIS in the posterior and anterior circulations to determine if the risk differs between the 2 locations. Design, Setting, and Participants: A retrospective analysis of CAIS was conducted among children enrolled in a single-center prospective consecutive cohort at The Children's Hospital of Philadelphia between January 1, 2006, and January 1, 2015...
January 23, 2017: JAMA Neurology
https://www.readbyqxmd.com/read/28111040/-immunotherapy-in-brain-tumors
#20
Emilie De Carli, Matthieu Delion, Audrey Rousseau
Diffuse gliomas represent the most common primary central nervous system (CNS) tumors in adults and children alike. Glioblastoma is the most frequent and malignant form of diffuse glioma with a median overall survival of 15 months despite aggressive treatments. New therapeutic approaches are needed to prolong survival in this always fatal disease. The CNS has been considered for a long time as an immune privileged organ, in part because of the existence of the blood-brain barrier. Nonetheless, immunotherapy is a novel approach in the therapeutic management of glioma patients, which has shown promising results in several clinical trials, especially in the adult population...
February 2017: Annales de Pathologie
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