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Pediatric brain tumors

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https://www.readbyqxmd.com/read/29324471/sellar-region-atypical-teratoid-rhabdoid-tumors-atrt-in-adults-display-dna-methylation-profiles-of-the-atrt-myc-subgroup
#1
Pascal D Johann, Susanne Bens, Florian Oyen, Rabea Wagener, Caterina Giannini, Arie Perry, Jack M Raisanen, Gerald F Reis, Sumihito Nobusawa, Kazunori Arita, Jörg Felsberg, Guido Reifenberger, Abbas Agaimy, Rolf Buslei, David Capper, Stefan M Pfister, Reinhard Schneppenheim, Reiner Siebert, Michael C Frühwald, Werner Paulus, Marcel Kool, Martin Hasselblatt
Atypical teratoid/rhabdoid tumor (ATRT) is a highly malignant brain tumor predominantly encountered in infants. Mutations of the SMARCB1 gene are the characteristic genetic lesion. A small group of ATRT stands out clinically, because these tumors are located in the sellar region of adults. To investigate if sellar region ATRT in adults represents a molecular distinct entity, we characterized molecular alterations in 7 sellar region ATRTs in adults as compared with 150 pediatric ATRTs and 47 pituitary adenomas using SMARCB1 sequencing, multiplex ligation-dependent probe amplification and fluorescence in situ hybridization as well as DNA methylation profiling...
January 10, 2018: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/29322843/immunotherapy-for-brain-tumors-understanding-early-successes-and-limitations
#2
Nicole A P Lieberman, Nicholas A Vitanza, Courtney A Crane
Adverse effects and toxicities related to standard treatments for brain tumors significantly reduce patients' quality of life. Although most immunotherapy approaches for solid tumors have not been successful, several early-phase clinical trials are beginning to reveal a potential role for immunotherapy in the treatment of brain tumors. In particular, methods that activate the innate immune system and induce a polyclonal anti-cancer response have demonstrated that brain tumors are susceptible to immune-mediated tumor destruction...
January 11, 2018: Expert Review of Neurotherapeutics
https://www.readbyqxmd.com/read/29322427/immunophenotyping-of-pediatric-brain-tumors-correlating-immune-infiltrate-with-histology-mutational-load-and-survival-and-assessing-clonal-t-cell-response
#3
Ashley S Plant, Shohei Koyama, Claire Sinai, Isaac H Solomon, Gabriel K Griffin, Keith L Ligon, Pratiti Bandopadhayay, Rebecca Betensky, Ryan Emerson, Glenn Dranoff, Mark W Kieran, Jerome Ritz
There is little known regarding the immune infiltrate present in pediatric brain tumors and how this compares to what is known about histologically similar adult tumors and its correlation with survival. Here, we provide a descriptive analysis of the immune infiltrate of 22 fresh pediatric brain tumor tissue samples of mixed diagnoses and 40 peripheral blood samples. Samples were analyzed using a flow cytometry panel containing markers for immune cell subtypes, costimulatory markers, inhibitory signals, and markers of activation...
January 10, 2018: Journal of Neuro-oncology
https://www.readbyqxmd.com/read/29315108/review-of-molecular-classification-and-treatment-implications-of-pediatric-brain-tumors
#4
Ana S Guerreiro Stucklin, Vijay Ramaswamy, Craig Daniels, Michael D Taylor
PURPOSE OF REVIEW: Brain tumors are the most common solid tumors and leading cause of cancer-related death in children. The advent of large-scale genomics has resulted in a plethora of profiling studies that have mapped the genetic and epigenetic landscapes of pediatric brain tumors, ringing in a new era of precision diagnostics and targeted therapies. In this review, we highlight the most recent findings, focusing on studies published after 2015, and discuss how new evidence is changing the care of children with brain tumors...
February 2018: Current Opinion in Pediatrics
https://www.readbyqxmd.com/read/29312599/histopathological-molecular-clinical-and-radiological-characterization-of-rosette-forming-glioneuronal-tumor-in-the-central-nervous-system
#5
Chenlong Yang, Jingyi Fang, Guang Li, Shaowu Li, Tingting Ha, Jiangfei Wang, Bao Yang, Jun Yang, Yulun Xu
Objective: A rosette-forming glioneuronal tumor (RGNT) is a rare entity originally described in the fourth ventricle. Recently, RGNTs occurring in extraventricular sites and those with malignant behaviors have been reported. The purpose of this study was to analyze the clinicoradiological and histopathological features, therapeutic strategies, and outcomes of RGNTs. Methods: We enrolled 38 patients diagnosed with RGNTs pathologically between August 2009 and June 2016...
December 12, 2017: Oncotarget
https://www.readbyqxmd.com/read/29305683/the-use-of-5-aminolevulinic-acid-to-assist-gross-total-resection-of-pediatric-astroblastoma
#6
Yuji Agawa, Takafumi Wataya
PURPOSE: Astroblastoma is an uncommon pediatric neuroepithelial tumor. The prognosis and appropriate treatment of astroblastoma were not well understood. Previous reports suggested the best treatment for astroblastoma is surgical total resection. The authors report a case of pediatric astroblastoma that underwent gross total resection with the use of fluorescent guidance by 5-aminolevulinic acid (5-ALA). CASE REPORT: A 13-year-old girl presented with the tumor that was well-circumscribed cystic and solid mass with marked gadolinium enhancement in the right occipital lobe...
January 5, 2018: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/29304919/cranial-tumor-surgical-outcomes-at-a-high-volume-academic-referral-center
#7
Desmond A Brown, Benjamin T Himes, Brittny T Major, Benjamin F Mundell, Ravi Kumar, Bruce Kall, Fredric B Meyer, Michael J Link, Bruce E Pollock, John D Atkinson, Jamie J Van Gompel, W Richard Marsh, Giuseppe Lanzino, Mohamad Bydon, Ian F Parney
OBJECTIVE: To determine adverse event rates for adult cranial neuro-oncologic surgeries performed at a high-volume quaternary academic center and assess the impact of resident participation on perioperative complication rates. PATIENTS AND METHODS: All adult patients undergoing neurosurgical intervention for an intracranial neoplastic lesion between January 1, 2009, and December 31, 2013, were included. Cases were categorized as biopsy, extra-axial/skull base, intra-axial, or transsphenoidal...
January 2018: Mayo Clinic Proceedings
https://www.readbyqxmd.com/read/29301780/multiparametric-analysis-of-permeability-and-adc-histogram-metrics-for-classification-of-pediatric-brain-tumors-by-tumor-grade
#8
S Vajapeyam, D Brown, P R Johnston, K I Ricci, M W Kieran, H G W Lidov, T Y Poussaint
BACKGROUND AND PURPOSE: Accurate tumor grading is essential for treatment planning of pediatric brain tumors. We hypothesized that multiparametric analyses of a combination of permeability metrics and ADC histogram metrics would differentiate high- and low-grade tumors with high accuracy. MATERIALS AND METHODS: DTI and dynamic contrast-enhanced MR imaging using T1-mapping with flip angles of 2°, 5°, 10°, and 15°, followed by a 0.1-mmol/kg body weight gadolinium-based bolus was performed on all patients in addition to standard MR imaging...
January 4, 2018: AJNR. American Journal of Neuroradiology
https://www.readbyqxmd.com/read/29297180/giant-cardiac-tumors-in-the-newborn-an-unusual-image
#9
Joanna Kwiatkowska, Sebastian Ciemny, Dariusz Kozłowski
Primary heart tumors in the pediatric population are very rare and they range from 0.01% to 0.04%. Most are benign lesions of which about half are rhabdomyomas. Rhabdomyoma tumor diagnosis is associated with a 75-80% risk of TSC. TSC are characterised with numerous changes of hamartoma type located in the brain, kidneys, skin and other organs including in the heart. More than two thirds of newborns with TSC present rhabdomyomas in the heart. These changes may be asymptomatic, but in some cases they may cause heart failure, arrhythmias and death...
January 3, 2018: Folia Morphologica (Warsz)
https://www.readbyqxmd.com/read/29295876/rapid-clinical-and-radiographic-response-with-combined-dabrafenib-and-trametinib-in-adults-with-braf-mutated-high-grade-glioma
#10
Tanner M Johanns, Cole J Ferguson, Patrick M Grierson, Sonika Dahiya, George Ansstas
BRAF V600E mutations have been successfully treated with targeted therapy in melanoma, non-small cell lung cancer, and thyroid cancer. Interestingly, these mutations have also been identified in a subset of pediatric and adult brain tumors, with several cases reportedly responding to targeted therapy. However, these reports have been limited to single-agent BRAF inhibitor therapy and recurrent disease. Herein, we report dramatic clinical and radiographic responses to combination dabrafenib (BRAF inhibitor) and trametinib (MEK inhibitor) in 2 adults with high-grade gliomas (HGGs), with 1 patient treated in the first-line setting...
January 2018: Journal of the National Comprehensive Cancer Network: JNCCN
https://www.readbyqxmd.com/read/29288854/lesional-temporal-lobe-epilepsy-beware-the-deceitful-panic-attack
#11
Kay O Kulason, Julia R Schneider, Ralph Rahme, Bidyut Pramanik, Derek Chong, John A Boockvar
BACKGROUND: Ganglioglioma is a rare, benign, intraaxial glioneuronal tumor, but a relatively common cause of pharmacoresistant temporal lobe epilepsy (TLE). Given its often nonspecific neuropsychiatric manifestations and frequently negative electroencephalographic workup, TLE can be easily misdiagnosed as a psychiatric disorder, particularly panic attacks. CASE DESCRIPTION: We present a case of a 17-year old boy who was found to have lesional TLE secondary to a left temporal ganglioglioma, 5 years after having been misdiagnosed with panic disorder and undergone ineffective and unnecessary psychotherapy...
December 27, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/29286561/intracranial-metastasis-in-fibrolamellar-hepatocellular-carcinoma
#12
William J Hammond, Gadi Lalazar, James A Saltsman, Benjamin A Farber, Enrico Danzer, Tshering C Sherpa, Charles D Banda, Jeffrey R Andolina, Sasan Karimi, Cameron W Brennan, Michael S Torbenson, Michael P La Quaglia, Sanford M Simon
Fibrolamellar hepatocellular carcinoma (FLHCC) is a rare liver malignancy in adolescents and young adults. Surgery is the mainstay of therapy for primary and metastatic disease. Most patients relapse, with development of both local and distant metastases. Brain metastases from solid tumors are rare in the pediatric and young adult population. Here, we document three patients with brain metastases from FLHCC, confirmed by histology and molecular characterization of the chimeric fusion DNAJB1-PRKACA, each necessitating neurosurgical intervention...
December 29, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/29285383/effect-of-o6-methylguanine-dna-methyltransferase-methylation-in-medulloblastoma
#13
Tomoko Kurimoto, Akihide Kondo, Ikuko Ogino, Junya Fujimura, Atsushi Arakawa, Hajime Arai, Toshiaki Shimizu
Medulloblastoma is a highly malignant brain tumor that predominately affects children and requires multimodal treatment, including chemotherapy with alkylating agents. O6-methylguanine-DNA methyltransferase (MGMT) is a DNA repair enzyme that plays an important role in tumor resistance to alkylating agents. Recent studies demonstrated that MGMT promoter methylation suppresses the expression of MGMT and is associated with favorable outcomes of malignant glioma patients. However, the MGMT methylation status and its prognostic impact on medulloblastoma have not been fully elucidated to date...
December 2017: Molecular and Clinical Oncology
https://www.readbyqxmd.com/read/29280680/smarcb1-deficient-tumors-of-childhood-a-practical-guide
#14
Bruce R Pawel
The SMARCB1 gene ( INI1, BAF47) is a member of the SWItch/Sucrose Non-Fermentable (SWI/SNF) chromatin remodeling complex, involved in the epigenetic regulation of gene transcription. SMARCB1 acts as a tumor suppressor gene, and loss of function of both alleles gives rise to SMARCB1-deficient tumors. The prototypical SMARCB1-deficient tumor is the malignant rhabdoid tumor (MRT) which was first described in the kidney but also occurs in soft tissue, viscera, and the brain (where it is referred to as atypical teratoid rhabdoid tumor or AT/RT)...
January 1, 2017: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/29280516/suppression-of-stat3-nh2-terminal-domain-chemosensitizes-medulloblastoma-cells-by-activation-of-protein-inhibitor-of-activated-stat3-via-de-repression-by-microrna-21
#15
Sutapa Ray, Don W Coulter, Shawn D Gray, Jason A Sughroue, Shrabasti Roychoudhury, Erin M McIntyre, Nagendra K Chaturvedi, Kishor K Bhakat, Shantaram S Joshi, Timothy R McGuire, John G Sharp
Medulloblastoma (MB) is a malignant pediatric brain tumor with poor prognosis. Signal transducers and activators of transcription-3 (STAT3) is constitutively activated in MB where it functions as an oncoprotein, mediating cancer progression and metastasis. Here, we have delineated the functional role of activated STAT3 in MB, by using a cell permeable STAT3-NH2 terminal domain inhibitor (S3-NTDi) that specifically perturbs the structure/function of STAT3. We have implemented several biochemical experiments using human MB tumor microarray (TMA) and pediatric MB cell lines, derived from high-risk SHH-TP53-mutated and MYC-amplified Non-WNT/SHH tumors...
December 27, 2017: Molecular Carcinogenesis
https://www.readbyqxmd.com/read/29260390/parent-perspectives-and-preferences-for-strategies-regarding-nonsedated-mri-scans-in-a-pediatric-oncology-population
#16
Breya Walker, Heather M Conklin, Doralina L Anghelescu, Lacey P Hall, Wilburn E Reddick, Robert Ogg, Lisa M Jacola
PURPOSE: Children with cancer frequently require MRI scans for clinical purposes. Sedation with general anesthesia (GA) is often used to promote compliance, reduce motion, and alleviate anxiety. The use of GA for MRI scans is costly in terms of time, personnel, and medications. In addition, prominent risks are associated with anesthesia exposure in patients with complex medical conditions. Successful behavioral interventions have been implemented in clinical research settings to promote scan success and compliance...
December 19, 2017: Supportive Care in Cancer: Official Journal of the Multinational Association of Supportive Care in Cancer
https://www.readbyqxmd.com/read/29250697/two-girl-patients-with-medulloblastoma-case-reports
#17
Laura Daniela Marinău, Cristina Elena Singer, Cristian Meşină, Elena Carmen Niculescu, Ileana Puiu, Ileana Octavia Petrescu, Cristiana Geormăneanu, Augustina Cornelia Enculescu, Daniela Elise Tache, Ştefana Oana Purcaru, Simona Răciulă, Cosmina Lucia Damian
In childhood, the most common type of brain tumors is medulloblastoma, a highly malignant primary brain tumor that is found in the cerebellum or posterior fossa. The tumor mass increases and generates obstructive hydrocephalus. Risk factors (that might be involved in some cases) include the genetic syndrome such as type 1 neurofibromatosis, exposure to ionizing radiation and Epstein-Barr virus. Medulloblastoma is associated with recessively inherited Turcot disease and with conditions as ataxia-telangiectasia syndrome in several cases...
2017: Romanian Journal of Morphology and Embryology, Revue Roumaine de Morphologie et Embryologie
https://www.readbyqxmd.com/read/29249552/pretreatment-cognition-in-patients-diagnosed-with-pediatric-brain-tumors
#18
Elin Irestorm, Sean Perrin, Ingrid Tonning Olsson
BACKGROUND: There is a large body of literature identifying risk factors for the long-term cognitive alterations found in survivors of pediatric brain tumors. Less is known about baseline cognitive functioning in this population, but studies suggest that cognitive dysfunctions are often present at the time of diagnosis. This study aimed to identify potential risk factors for lower cognitive function at the time of pediatric brain tumor diagnosis. METHODS: Participants were children and adolescents (n = 101) diagnosed with a pediatric brain tumor between 2006 and 2015, who underwent a pretreatment neuropsychologic assessment...
November 22, 2017: Pediatric Neurology
https://www.readbyqxmd.com/read/29246098/optic-pathway-gliomas-in-neurofibromatosis-type-1
#19
Cynthia J Campen, David H Gutmann
Neurofibromatosis type 1 (NF1) is one of the most common brain tumor predisposition syndromes, in which affected children are prone to the development of low-grade gliomas. While NF1-associated gliomas can be found in several brain regions, the majority arise in the optic nerves, chiasm, tracts, and radiations (optic pathway gliomas; OPGs). Owing to their location, 35-50% of affected children present with reduced visual acuity. Unfortunately, despite tumor stabilization following chemotherapy, vision does not improve in most children...
January 2018: Journal of Child Neurology
https://www.readbyqxmd.com/read/29241176/surgery-of-intracranial-gliomas-in-children
#20
James A Balogun, James T Rutka
Gliomas are the most common type of brain cancer in the pediatric patients, constituting about 50% of all childhood intracranial tumors. This is a highly heterogeneous group, varying from the benign WHO histopathological grades I and II to malignant WHO grades III and IV. The histology and location are significant prognostic factors, which influence the decision for surgical intervention, as well as the extent of possible tumor removal. In low-grade gliomas, surgery remains the initial option and should be directed at gross total resection in favorable locations, such as the cerebral hemispheres and the cerebellum...
2018: Progress in Neurological Surgery
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