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Brain tumors in children

Istvan Bodi, Anastasios Giamouriadis, Naomi Sibtain, Ross Laxton, Andrew King, Francesco Vergani
Background: Primary CNS malignant rhabdoid tumors are very rare in adults and much less is known about their biological behavior than in children. Recently, two adult cases of SMARCB1 (also known as INI1)-deficient tumor with rhabdoid cells have been described, suggesting an emerging group of primary meningeal SMARCB1-deficient tumors. We have recently encountered a case of INI1-deficient tumor with similar histology and immunophenotype to the above cases, but with a superficial cerebral, yet apparent intra-axial origin...
2018: Surgical Neurology International
Balagangadhar R Totapally, Ashish H Shah, Toba Niazi
OBJECTIVE: Posterior fossa tumor surgery in children poses a significant morbidity and mortality. Large multi-institutional datasets characterizing the epidemiology and morbidity of children undergoing posterior fossa tumor surgery are lacking. The objective of this study is to describe the epidemiology and short term surgical outcomes of children presenting with cerebellar tumors. PATIENTS AND METHODS: A retrospective review of the Kids Inpatient Database (KID) for all hospital discharges in 2012 with a diagnosis of cerebellar tumor (ICD-9 diagnosis code 191...
February 26, 2018: Clinical Neurology and Neurosurgery
Akhgar Ghassabian, Paul S Albert, Mady Hornig, Edwina Yeung, Sara Cherkerzian, Risë B Goldstein, Stephen L Buka, Jill M Goldstein, Stephen E Gilman
Gestational inflammation may contribute to brain abnormalities associated with childhood neuropsychiatric disorders. Limited knowledge exists regarding the associations of maternal cytokine levels during pregnancy with offspring neurocognitive development. We assayed the concentrations of five cytokines (interleukin (IL)-6, IL-1β, IL-8, tumor necrosis factor alpha (TNF-α), and IL-10) up to four times in the 2nd and 3rd trimesters of pregnancy using stored prenatal sera from 1366 participants in the New England Family Study (enrollment 1959-1966)...
March 13, 2018: Translational Psychiatry
Fumihiko Nishimura, Young-Su Park, Yasushi Motoyama, Ichiro Nakagawa, Shuichi Yamada, Hiroyuki Nakase
BACKGROUND: Xanthomatous pituitary diseases rarely occur in childhood. We report a rare pediatric case of a xanthogranuloma that developed in the sellar region resulting in visual disturbance that was successfully treated by endoscopic endonasal surgery. CASE DESCRIPTIONS: A 13-year-old boy came to us with a headache and visual disturbance that had occurred 1 month prior. Clinical examination findings showed that he was alert with signs of bitemporal hemianopsia, an endocrinological examination showed partial hypopituitarism, and brain magnetic resonance imaging (MRI) revealed a cystic mass in the sellar turcica compressing the optic apparatus...
March 7, 2018: World Neurosurgery
Federico Bianchi, Gianpiero Tamburrini, Marco Gessi, Paolo Frassanito, Luca Massimi, Massimo Caldarelli
BACKGROUND: Primary central nervous system (CNS) neuroblastoma is a rare intracranial tumor affecting children mainly in the first years of life. It is usually a supratentorial tumor with a wide spectrum of clinical presentation, seizures, and focal neurological deficits being the most common presenting signs. CASE DESCRIPTION: A 2-year-old child was admitted to our ward after a generalized seizure. Neurological examination was normal. Radiological studies showed a small DWI hyperintense lesion of the right rectus gyrus...
March 8, 2018: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
L Holmes, P Chavan, T Blake, K Dabney
BACKGROUND: While survival in overall pediatric malignancy has improved during recent decades, brain/central nervous system (CNS) tumors has not demonstrated comparable survival advantage. Incidence and mortality data in this malignancy continue to illustrate race and sex differences; however, there are few data in the pediatric setting. This study sought to characterize brain/CNS tumors by socio-demographic and assess racial and sex variances in both cumulative incidence and mortality...
March 7, 2018: Journal of Racial and Ethnic Health Disparities
Sara Bolin, Anna Borgenvik, Camilla U Persson, Anders Sundström, Jun Qi, James E Bradner, William A Weiss, Yoon-Jae Cho, Holger Weishaupt, Fredrik J Swartling
Medulloblastoma (MB) is the most common malignant brain tumor in children. MYC genes are frequently amplified and correlate with poor prognosis in MB. BET bromodomains recognize acetylated lysine residues and often promote and maintain MYC transcription. Certain cyclin-dependent kinases (CDKs) are further known to support MYC stabilization in tumor cells. In this report, MB cells were suppressed by combined targeting of MYC expression and MYC stabilization using BET bromodomain inhibition and CDK2 inhibition, respectively...
March 7, 2018: Oncogene
Takashi Ishihara, Keiji Nogami, Yasufumi Takeshita, Satoshi Ochi, Midori Shima
Thrombosis and hemorrhage are serious complications in pediatric patients with solid tumors, and enhanced fibrinolysis associated with disseminated intravascular coagulation (DIC) is often observed. Fibrinolytic enzymes also play an important role in metastasis. Limited information is available, however, on the assessment of overall hemostatic function in children with malignant solid tumors. Methods We have investigated comprehensive hemostatic potential in these circumstances using simultaneous thrombin/plasmin generation assay (T/P-GA)...
March 5, 2018: Pediatrics International: Official Journal of the Japan Pediatric Society
Fatima Tensaouti, Anne Ducassou, Léonor Chaltiel, Annick Sevely, Stéphanie Bolle, Laetitia Padovani, Anais Jouin, Claire Alapetite, Stéphane Supiot, Aymeri Huchet, Valérie Bernier, Line Claude, Christine Kerr, Elisabeth Le Prisé, Anne-Isabelle Bertozzi-Salamon, Samuel Liceaga, Jean Albert Lotterie, Patrice Péran, Anne Laprie
BACKGROUND AND PURPOSE: Ependymoma is the third most common brain tumor in children. Radiation therapy (RT) is systematically administered after maximum surgical resection, utilizing recent advances in radiation delivery. Imaging can make a significant contribution to improving treatment outcome. This prompted us to look for significant preoperative and postoperative imaging markers for survival. MATERIAL AND METHODS: We undertook a national retrospective review of 121 patients who had undergone resection followed by RT...
February 27, 2018: Radiotherapy and Oncology: Journal of the European Society for Therapeutic Radiology and Oncology
Anna Papadopoulou, Argyrios Dinopoulos, George Koutsodontis, Roser Pons, Pelagia Vorgia, Vasiliki Koute, Athanassios Vratimos, Dimitrios Zafeiriou
Tuberous Sclerosis Complex (TSC) is a rare neurocutaneous syndrome inherited by an autosomal dominant manner. The disorder is commonly manifested by the presence of multiple benign tumors located in numerous tissues, including the brain, heart, skin and kidneys. Seizures, autism, developmental and behavioral delay, as well as non-neurological phenotypic findings, are suggestive of TSC. The identification of one pathogenic mutation in either the TSC1 or TSC2 genes is considered to be an independent diagnostic criterion...
February 9, 2018: European Journal of Paediatric Neurology: EJPN
Andrew H Zureick, Casey L Evans, Andrzej Niemierko, Julie A Grieco, Alexandra J Nichols, Barbara C Fullerton, Clayton B Hess, Claire P Goebel, Sara L Gallotto, Elizabeth A Weyman, Dillon E Gaudet, Jessica A Nartowicz, David H Ebb, Robin M Jones, Shannon M MacDonald, Nancy J Tarbell, Torunn I Yock, Margaret B Pulsifer
BACKGROUND: Radiotherapy (RT) in the pediatric brain tumor population causes late neurocognitive effects. In the current study, the authors investigated associations between clinical and dosimetric risk factors and memory outcomes in a cohort of patients treated with proton radiotherapy (PRT). METHODS: A total of 70 patients (median age at PRT, 12.1 years [range, 5.0-22.5 years]) who were treated with PRT were identified with baseline and follow-up evaluations of visual and verbal memory (Children's Memory Scale and the third edition of the Wechsler Memory Scale)...
March 2, 2018: Cancer
Hila Rosenfeld-Keidar, Rinat Eshel, Aviva Pinhasov, Menachem Bitan, Sabina Edelman, Marcela Broitman, Rina Dvir, Efraim Sadot, Dror Levin, Michal Manisterski, Sivan Berger-Achituv, Ronit Elhasid
Numerous adults' studies demonstrated that preaphaeresis CD34+ cells significantly correlate with the number of CD34+ cells collected by the aphaeresis procedure. Equivalent studies in children are scarce. We studied retrospectively 92 aphaeresis procedures performed following chemotherapy (44) or in steady state (48) in 60 pediatric patients (40 males, 20 females), median age of 7.5 years. Aphaeresis procedures were performed using a SPECTRA Optica (TERUMOBCT) continuous flow cell separator. CD34+ cell concentrations were assessed using flow cytometry...
March 2, 2018: Pediatric Transplantation
Sivan Gershanov, Helen Toledano, Shalom Michowiz, Orit Barinfeld, Albert Pinhasov, Nitza Goldenberg-Cohen, Mali Salmon-Divon
Purpose: Medulloblastoma (MB), the most common malignant brain tumor in children, is divided into four tumor subgroups: wingless-type (WNT), sonic hedgehog (SHH), Group 3, and Group 4. Ideally, clinical practice and treatment design should be subgroup specific. While WNT and SHH subgroups have well-defined biomarkers, distinguishing Group 3 from Group 4 is not straightforward. MicroRNAs (miRNAs), which regulate posttranscriptional gene expression, are involved in MB tumorigenesis. However, the miRNA-messenger RNA (mRNA) regulatory network in MB is far from being fully understood...
2018: Cancer Management and Research
Joshua Casaos, Sakibul Huq, Tarik Lott, Raphael Felder, John Choi, Noah Gorelick, Michael Peters, Yuanxuan Xia, Russell Maxwell, Tianna Zhao, Chenchen Ji, Thomas Simon, Julie Sesen, Sarah J Scotland, Richard E Kast, Jeffrey Rubens, Eric Raabe, Charles G Eberhart, Eric M Jackson, Henry Brem, Betty Tyler, Nicolas Skuli
Atypical teratoid/rhabdoid tumors (AT/RT) are highly aggressive, malignant tumors and are the most common malignant brain tumor in children under 6 months of age. Currently, there is no standard treatment for AT/RT. Recent studies have reported potential anti-tumoral properties of ribavirin, a guanosine analog and anti-viral molecule approved by the Food and Drug Administration for treatment of hepatitis C. We previously demonstrated that ribavirin inhibited glioma cell growth in vitro and in vivo . Based on these results and the fact that no pre-clinical model of ribavirin in AT/RT exists, we decided to investigate the effect of ribavirin on several human AT/RT cell lines (BT12, BT16, and BT37) both in vitro and in vivo ...
January 30, 2018: Oncotarget
Sreedharan Thankarajan Arunraj, Girish Kumar Parida, Nishikant Avinash Damle, Saurabh Arora, Sreenivasa Reddy, Dhritiman Chakraborty, Meghna Prabhu, Madhavi Tripathi, Chandrasekhar Bal
Medulloblastoma, also known as cerebellar primitive neuroectodermal tumor, is the most common brain tumor in children and arises in the posterior cranial fossa. We present the case of a patient with desmoplastic type of medulloblastoma, which showed recurrence more than once. When Ga-DOTANOC PET-CT was done, the lesions showed somatostatin receptor expression, opening another potential therapeutic option for this patient.
February 27, 2018: Clinical Nuclear Medicine
Beat Bojaxhiu, Frank Ahlhelm, Marc Walser, Lorenzo Placidi, Ulrike Kliebsch, Lorentzos Mikroutsikos, Petra Morach, Alessandra Bolsi, Tony Lomax, Alessia Pica, Damien C Weber
PURPOSE: To assess the rate of radiation necrosis (RN) and white matter lesions (WMLs) in pediatric patients with primary brain tumors treated with pencil beam scanning (PBS) proton therapy (PT) with or without concomitant chemotherapy at the PSI. METHODS AND MATERIALS: Between 1999 and 2015, 171 pediatric patients (age <18 years) were treated with PT. Median age at diagnosis was 3.3 years (range, 0.3-17.0 years), and the median delivered dose was 54 Gy (relative biological effectiveness) (range, 40...
March 15, 2018: International Journal of Radiation Oncology, Biology, Physics
Laetitia Padovani, Frédérique Chapon, Nicolas André, Mohamed Boucekine, Anne Geoffray, Franck Bourdeau, Julien Masliah-Planchon, Line Claude, Aymeri Huchet, Anne Laprie, Stephane Supiot, Bernard Coche-Dequéant, Christine Kerr, Claire Alapetite, Julie Leseur, Tandat Nguyen, Sophie Chapet, Valerie Bernier, Pierre-Yves Bondiau, Georges Noel, Jean Louis Habrand, Stephanie Bolle, François Doz, Christelle Dufour, Xavier Muracciole, Christian Carrie
PURPOSE: To identify the incidence of patients with perihippocampal metastases to assess the risk of brain relapse when sparing the hippocampal area. Medulloblastoma (MB) represents 20% of pediatric brain tumors. For high-risk MB patients, the 3- to 5-year event-free survival rate has recently improved from 50% to >76%. Many survivors, however, experience neurocognitive side effects. Several retrospective studies of patients receiving whole brain irradiation (WBI) have suggested a relationship between the radiation dose to the hippocampus and neurocognitive decline...
March 15, 2018: International Journal of Radiation Oncology, Biology, Physics
Atosa Estekizadeh, Natalia Landázur, Jiri Bartek, Christian Beltoft Brøchner, Belghis Davoudi, Helle Broholm, Mohsen Karimi, Tomas J Ekström, Afsar Rahbar
Among all brain tumors diagnosed in children, medulloblastomas (MBs) are associated with a poor prognosis. The etiology of MB is not fully understood, yet the impact of epigenetic alterations of oncogenes has previously been established. During the past decade, the human cytomegalovirus (HCMV) has been detected in several types of cancer, including MB. Since DNA methylation occurs in the cell nucleus and this is considered a host defence response, we studied the impact of HCMV infection on DNA methyltransferase (DNMT‑1) in MB (D324) cells, human umbilical vein endothelial cells (HUVECs) as well as in MB tissue sections...
February 26, 2018: International Journal of Oncology
Maria Tsoli, Jie Liu, Laura Franshaw, Han Shen, Cecilia Cheng, MoonSun Jung, Swapna Joshi, Anahid Ehteda, Aaminah Khan, Angel Montero-Carcabosso, Pierre J Dilda, Philip Hogg, David S Ziegler
Diffuse Intrinsic Pontine Gliomas (DIPG) are the most devastating of all pediatric brain tumors. They mostly affect young children and, as there are no effective treatments, almost all patients with DIPG will die of their tumor within 12 months of diagnosis. A key feature of this devastating tumor is its intrinsic resistance to all clinically available therapies. It has been shown that glioma development is associated with metabolic reprogramming, redox state disruption and resistance to apoptotic pathways...
January 26, 2018: Oncotarget
Jacqueline B Stone, Joanne F Kelvin, Lisa M DeAngelis
Background: Fertility preservation (FP) is an infrequently addressed issue for young adults with primary brain tumors. Given the improved prognosis and enhanced technology in reproductive medicine, more primary brain tumor patients see procreation as feasible, making the discussion of FP increasingly important. The goals of this study were to describe patients who received FP counseling by a fertility nurse specialist (FNS) and determine which sociodemographic and disease-related factors predict acceptance of referral to a reproductive specialist...
March 2017: Neuro-oncology Practice
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