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Neuroblastoma

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https://www.readbyqxmd.com/read/28818704/orphan-drug-regulation-a-missed-opportunity-for-children-and-adolescents-with-cancer
#1
Gilles Vassal, Pam Kearns, Patricia Blanc, Nicole Scobie, Delphine Heenen, Andy Pearson
BACKGROUND: Oncology represents a major sector in the field of orphan drug development in Europe. The objective was to evaluate whether children and adolescents with cancer benefited from the Orphan Drug Regulation. METHODS: Data on orphan drug designations (ODDs) and registered orphan drugs from 8th August 2000 to 10th September 2016 were collected from the Community Register of medicinal products for human use. Assessment history, product information and existence of paediatric investigation plans were searched and retrieved from the European Medicine Agency website...
August 14, 2017: European Journal of Cancer
https://www.readbyqxmd.com/read/28818170/post-chemotherapy-neuroblastoma-like-differentiation-in-ewing-sarcoma-of-humerus-report-of-a-rare-case-with-review-of-literature
#2
Nasir Ud Din, Amna Qasim, Zubair Ahmad
A21-year patient initially presented with a fracture of the humerus following minor trauma. Abone scan and biopsy were done due to the suspicion of pathological fracture and the biopsy confirmed the diagnosis of Ewing Sarcoma (EWS). Two months after initial presentation, chemotherapy was started and 5 cycles were given over a span of 6 months. Surgical resection of the tumor was then performed. The post-chemotherapy resection specimen, on histological examination, showed the presence of areas of neuroblastoma-like differentiation in otherwise morphologically classic EWS...
July 2017: Journal of the College of Physicians and Surgeons—Pakistan: JCPSP
https://www.readbyqxmd.com/read/28818093/case-report-value-of-gene-expression-profiling-in-the-diagnosis-of-atypical-neuroblastoma
#3
Anne C Harttrampf, Qingrong Chen, Eva Jüttner, Julia Geiger, Gordon Vansant, Javed Khan, Udo Kontny
BACKGROUND: Nephroblastoma and neuroblastoma belong to the most common abdominal malignancies in childhood. Similarities in the initial presentation may provide difficulties in distinguishing between these two entities, especially if unusual variations to prevalent patterns of disease manifestation occur. Because of the risk of tumor rupture, European protocols do not require biopsy for diagnosis, which leads to misdiagnosis in some cases. CASE PRESENTATION: We report on a 4½-year-old girl with a renal tumor displaying radiological and laboratory characteristics supporting the diagnosis of nephroblastoma...
August 17, 2017: BMC Research Notes
https://www.readbyqxmd.com/read/28816801/bevacizumab-associated-bowel-microperforation-in-a-patient-with-neuroblastoma
#4
Rachel Glincher, Anita P Price, Michael P LaQuaglia, Brian H Kushner, Shakeel Modak
The antivascular endothelial growth factor antibody, bevacizumab, is effective against several malignancies in adults but unproven in pediatric oncology. In early phase pediatric studies toxicities were similar to those in adults. Bowel perforation in adults is a rare but serious toxicity, but has not been hitherto reported in children. A 5-year-old boy with chemoresistant neuroblastoma treated with bevacizumab plus radioimmunotherapy developed acute abdominal pain. Computed tomography scan showed free abdominal air and pneumatosis coli...
August 14, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28815971/topographical-and-electrical-stimulation-of-neuronal-cells-through-microwrinkled-conducting-polymer-biointerfaces
#5
Alberto Bonisoli, Attilio Marino, Gianni Ciofani, Francesco Greco
The development of smart biointerfaces combining multiple functions is crucial for triggering a variety of cellular responses. In this work, wrinkled organic interfaces based on the conducting polymer poly(3,4-ethylene dioxythiophene) doped with poly(styrene sulfonate) are developed with the aim to simultaneously convey electrical and topographical stimuli to cultured cells. The surface wrinkling of thin films on heat-shrink polymer sheets allows for rapid patterning of self-assembled anisotropic topographies characterized by micro/sub-microscale aligned wrinkles...
August 16, 2017: Macromolecular Bioscience
https://www.readbyqxmd.com/read/28815211/phenotypic-differences-between-asian-and-african-lineage-zika-viruses-in-human-neural-progenitor-cells
#6
Fatih Anfasa, Jurre Y Siegers, Mark van der Kroeg, Noreen Mumtaz, V Stalin Raj, Femke M S de Vrij, W Widagdo, Gülsah Gabriel, Sara Salinas, Yannick Simonin, Chantal Reusken, Steven A Kushner, Marion P G Koopmans, Bart Haagmans, Byron E E Martina, Debby van Riel
Recent Zika virus (ZIKV) infections have been associated with a range of neurological complications, in particular congenital microcephaly. Human neural progenitor cells (hNPCs) are thought to play an important role in the pathogenesis of microcephaly, and experimental ZIKV infection of hNPCs has been shown to induce cell death. However, the infection efficiency and rate of cell death have varied between studies, which might be related to intrinsic differences between African and Asian lineage ZIKV strains...
July 2017: MSphere
https://www.readbyqxmd.com/read/28814318/annexin-a2-could-enhance-multidrug-resistance-by-regulating-nf-%C3%AE%C2%BAb-signaling-pathway-in-pediatric-neuroblastoma
#7
Yi Wang, Kai Chen, Yihong Cai, Yuanxia Cai, Xiaojun Yuan, Lifeng Wang, Zhixiang Wu, Yeming Wu
BACKGROUND: Chemotherapy is one of major therapeutic regimens for neuroblastoma (NB) in children. However, recurrence and metastasis associated with poor prognosis caused by acquired multidrug resistance remains a challenge. There is a great need to achieve new insight into the molecular mechanism of drug resistance in NB. The aim of this study is to identify novel drug sensitivity-related biomarkers as well as new therapeutic targets to overcome chemoresistance. METHODS: We proteome-wide quantitatively compared protein expression of two NB cell lines with different drug sensitivities, isolated from the same patient prior to and following chemotherapy...
August 16, 2017: Journal of Experimental & Clinical Cancer Research: CR
https://www.readbyqxmd.com/read/28811280/dan-nbl1-promotes-collective-neural-crest-migration-by-restraining-uncontrolled-invasion
#8
Rebecca McLennan, Caleb M Bailey, Linus J Schumacher, Jessica M Teddy, Jason A Morrison, Jennifer C Kasemeier-Kulesa, Lauren A Wolfe, Madeline M Gogol, Ruth E Baker, Philip K Maini, Paul M Kulesa
Neural crest cells are both highly migratory and significant to vertebrate organogenesis. However, the signals that regulate neural crest cell migration remain unclear. In this study, we test the function of differential screening-selected gene aberrant in neuroblastoma (DAN), a bone morphogenetic protein (BMP) antagonist we detected by analysis of the chick cranial mesoderm. Our analysis shows that, before neural crest cell exit from the hindbrain, DAN is expressed in the mesoderm, and then it becomes absent along cell migratory pathways...
August 15, 2017: Journal of Cell Biology
https://www.readbyqxmd.com/read/28808732/individualized-risk-assessment-in-neuroblastoma-does-the-tumoral-metabolic-activity-on-123-i-mibg-spect-predict-the-outcome
#9
Julian M M Rogasch, Patrick Hundsdoerfer, Christian Furth, Florian Wedel, Frank Hofheinz, Paul-Christian Krüger, Holger Lode, Winfried Brenner, Angelika Eggert, Holger Amthauer, Imke Schatka
PURPOSE: Risk-adapted treatment in children with neuroblastoma (NB) is based on clinical and genetic factors. This study evaluated the metabolic tumour volume (MTV) and its asphericity (ASP) in pretherapeutic (123)I-MIBG SPECT for individualized image-based prediction of outcome. METHODS: This retrospective study included 23 children (11 girls, 12 boys; median age 1.8 years, range 0.3-6.8 years) with newly diagnosed NB consecutively examined with pretherapeutic (123)I-MIBG SPECT...
August 14, 2017: European Journal of Nuclear Medicine and Molecular Imaging
https://www.readbyqxmd.com/read/28807939/prc2-mediated-transcriptomic-alterations-at-the-embryonic-stage-govern-tumorigenesis-and-clinical-outcome-in-mycn-driven-neuroblastoma
#10
Shoma Tsubota, Satoshi Kishida, Teppei Shimamura, Miki Ohira, Satoshi Yamashita, Dongliang Cao, Shinichi Kiyonari, Toshikazu Ushijima, Kenji Kadomatsu
Pediatric cancers such as neuroblastoma are thought to involve a dysregulation of embryonic development. However, it has been difficult to identify the critical events that trigger tumorigenesis and differentiate them from normal development. In this study, we report the establishment of a spheroid culture method that enriches early-stage tumor cells from TH-MYCN mice, a preclinical model of neuroblastoma. Using this method, we found that tumorigenic cells were evident as early as day E13.5 during embryo development, when the MYC and PRC2 transcriptomes were significantly altered...
August 14, 2017: Cancer Research
https://www.readbyqxmd.com/read/28807729/cpg-demethylation-in-the-neurotoxicity-of-1-methyl-4-phenylpyridinium-might-mediate-transcriptional-up-regulation-of-%C3%AE-synuclein-in-sh-sy5y-cells
#11
Jian Yang, Zhaofei Yang, Xuan Wang, Min Sun, Yong Wang, Xiaomin Wang
The accumulation of α-synuclein is the primary pathological hallmark of Parkinson's disease (PD). In PD patients, CpG demethylation of intron-1 has been reported to be associated with α-synuclein up-regulation. Environmental factor, for example neurotoxin, is a major etiological risk factor in PD pathogenesis. However, the role of CpG methylation in neurotoxin-induced PD has not been addressed completely yet. To explore CpG methylation associating with α-synuclein transcription and its underlying mechanisms in the neurotoxin-induced PD pathology, human neuroblastoma SH-SY5Y cells were treated with neurotoxins 6-hydroxydopamine (6-OHDA) and 1-methyl-4-phenylpyridinium (MPP(+))...
August 11, 2017: Neuroscience Letters
https://www.readbyqxmd.com/read/28807373/usefulness-of-cocaine-drops-in-investigating-infant-anisocoria
#12
Gilles C Martin, Pierre-Antoine Aymard, Charlotte Denier, Caroline Seghir, Marc Abitbol, Nathalie Boddaert, Dominique Bremond-Gignac, Matthieu P Robert
INTRODUCTION: Whereas apraclonidine has eclipsed cocaine test in the exploration of unilateral miosis in adults, its use in infants is avoided because of the risk of central nervous system depression. This chart review evaluates the usefulness of cocaine drops in infants. METHODS: Infants under the age of one referred for unilateral miosis between November 1, 2009 and November 1, 2015, were reviewed. Patients underwent the following protocol: (1) in case of isolated miosis, cocaine test was performed...
August 4, 2017: European Journal of Paediatric Neurology: EJPN
https://www.readbyqxmd.com/read/28807002/high-resolution-measurement-of-duf1220-domain-copy-number-from-whole-genome-sequence-data
#13
David P Astling, Ilea E Heft, Kenneth L Jones, James M Sikela
BACKGROUND: DUF1220 protein domains found primarily in Neuroblastoma BreakPoint Family (NBPF) genes show the greatest human lineage-specific increase in copy number of any coding region in the genome. There are 302 haploid copies of DUF1220 in hg38 (~160 of which are human-specific) and the majority of these can be divided into 6 different subtypes (referred to as clades). Copy number changes of specific DUF1220 clades have been associated in a dose-dependent manner with brain size variation (both evolutionarily and within the human population), cognitive aptitude, autism severity, and schizophrenia severity...
August 14, 2017: BMC Genomics
https://www.readbyqxmd.com/read/28801367/comparison-of-hematological-aspects-between-neuroblastoma-patients-with-and-without-bone-marrow-infiltration
#14
Weida Wang, Xiongwen Peng, Cuirong Liang, Hao Chen, Yingchun Zhang, Xiaofei Sun, Leyuan Shen, Yue Lu
Bone marrow involvement in neuroblastoma indicates an advanced stage of the disease. In this study, we retrospectively analyzed the bone marrow infiltration status and hematological parameters of 111 cases of neuroblastoma. Among the 111 cases, 62 (55.9%) exhibited marrow infiltration. Erythrocytopenia, anemia, and thrombocytopenia were significantly (p<0.05) associated with marrow infiltration of >10% malignant cells. The evaluation of blood cell count parameters with receiver operating characteristic curves indicated that erythrocytopenia can substantially improve the accurate prediction of the risk of bone marrow involvement with a Youden index of 0...
August 2017: Annals of Clinical and Laboratory Science
https://www.readbyqxmd.com/read/28800395/using-chemistry-to-target-neuroblastoma
#15
Jeanne N Hansen, Xingguo Li, Y George Zheng, Louis T Lotta, Abhishek Dedhe, Nina F Schor
Neuroblastoma is a cancer of the neural crest almost exclusively seen in childhood. While children with single, small primary tumors are often cured with surgery alone, the 65% of children with neuroblastoma whose disease has metastasized have less than a 50% chance of surviving five years after diagnosis. Innovative pharmacological strategies are critically needed for these children. Efforts to identify novel targets that afford ablation of neuroblastoma with minimal toxicity to normal tissues are underway...
August 11, 2017: ACS Chemical Neuroscience
https://www.readbyqxmd.com/read/28800318/mir-513c-suppresses-neuroblastoma-cell-migration-invasion-and-proliferation-through-direct-targeting-glutaminase-gls
#16
Hong-Liang Xia, Yao Lv, Chun-Wei Xu, Ming-Cui Fu, Ting Zhang, Xiang-Ming Yan, Shu Dai, Qian-Wei Xiong, Yun Zhou, Jian Wang, Xu Cao
Neuroblastoma is a brain malignancy of childhood and accounts for 7-10% of childhood cancers, leading to approximately 15% of pediatric cancer deaths. MicroRNAs (miRNAs) are a family of short (about 18-25 nucleotides), noncoding and single stranded endogenous RNAs, which complementarily bind to the 3' untranslated regions of their target genes. Recently, glutamine metabolism has been recognized as an important nutrition source for tumor cells, and hence targeting glutamine metabolism could benefit to development of anti-cancer agents...
July 2, 2017: Cancer Biomarkers: Section A of Disease Markers
https://www.readbyqxmd.com/read/28800142/alcohol-mediated-missplicing-of-mcl-1-pre-mrna-is-involved-in-neurotoxicity
#17
Rahsan Sariyer, Francesca I De Simone, Martina Donadoni, Jan B Hoek, Sulie L Chang, Ilker Kudret Sariyer
BACKGROUND: Heavy and chronic ethanol (EtOH) exposure can cause significant structural and functional damage to the adult brain. The most devastating consequence of EtOH exposure is the neurotoxicity associated with the depletion of neurons. Regulation of splice variants in the brain can modulate protein functions, which may ultimately affect behaviors associated with alcohol dependence and EtOH-mediated neurotoxicity. Since alcohol consumption is associated with neurotoxicity, it is possible that altered splicing of survival and pro-survival factors during the development of alcoholism may contribute to the neurotoxicity...
August 11, 2017: Alcoholism, Clinical and Experimental Research
https://www.readbyqxmd.com/read/28799545/cancer-inhibiting-rock-in-neuroblastoma
#18
Crunkhorn Sarah
No abstract text is available yet for this article.
August 11, 2017: Nature Reviews. Drug Discovery
https://www.readbyqxmd.com/read/28798667/the-foxp2-driven-network-in-developmental-disorders-and-neurodegeneration
#19
Franz Oswald, Patricia Klöble, André Ruland, David Rosenkranz, Bastian Hinz, Falk Butter, Sanja Ramljak, Ulrich Zechner, Holger Herlyn
The transcription repressor FOXP2 is a crucial player in nervous system evolution and development of humans and songbirds. In order to provide an additional insight into its functional role we compared target gene expression levels between human neuroblastoma cells (SH-SY5Y) stably overexpressing FOXP2 cDNA of either humans or the common chimpanzee, Rhesus monkey, and marmoset, respectively. RNA-seq led to identification of 27 genes with differential regulation under the control of human FOXP2, which were previously reported to have FOXP2-driven and/or songbird song-related expression regulation...
2017: Frontiers in Cellular Neuroscience
https://www.readbyqxmd.com/read/28798342/nanoparticle-based-plasmonic-transduction-for-modulation-of-electrically-excitable-cells
#20
Parveen Bazard, Robert D Frisina, Joseph P Walton, Venkat R Bhethanabotla
There is a compelling need for the development of new sensory and neural prosthetic devices which are capable of more precise point stimulation. Current prosthetic devices suffer from the limitation of low spatial resolution due to the non-specific stimulation characteristics of electrical stimulation, i.e., the spread of electric fields generated. We present a visible light stimulation method for modulating the firing patterns of electrically-excitable cells using surface plasmon resonance phenomena. In in-vitro studies using gold (Au) nanoparticle-coated nanoelectrodes, we show that this method (substrate coated with nanoparticles) has the potential for incorporating this new technology into neural stimulation prosthetics, such as cochlear implants for the deaf, with very high spatial resolution...
August 10, 2017: Scientific Reports
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