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Pediatric tumors

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https://www.readbyqxmd.com/read/28650886/elevated-fdg-activity-in-the-nonpneumatized-sphenoid-bone-in-an-infant
#1
Yin Jie Chen, Nelleke Tolboom, Lisa J States, Hongming Zhuang
A 7-month-old boy with malignant rhabdoid tumor of the right lateral neck, status post resection and chemotherapy, underwent FDG PET/CT for restaging. The images showed diffuse increased activity in the spleen and in the bone marrow of the appendicular bones and the spine, which is related to hematopoietin administered after chemotherapy. The images also revealed intense activity in the region of sphenoid bone, which is not a common region to have elevated FDG activity. The subsequent MRI scan showed that this activity was due to not-yet-converted red marrow in the sphenoid bone in this pediatric patient...
June 24, 2017: Clinical Nuclear Medicine
https://www.readbyqxmd.com/read/28650783/conservative-treatment-of-multiple-keratocystic-odontogenic-tumors-in-a-young-patient-with-nevoid-basal-cell-carcinoma-syndrome-by-decompression-a-7-year-follow-up-study
#2
Su-Hyun Kim, Min-Seok Oh, Yo-Seob Seo, Jin-Young Kim, Soon-Hyeun Nam, Su-Min Lim
Multiple keratocystic odontogenic tumors (KCOT) occurred in a young child is challenging problem in the field of pediatric dentistry, and might have been related to nevoid basal cell carcinoma syndrome (NBCCS). Because of high recurrence rate of KCOTs, complete surgical resection is generally accepted as definitive treatment. However, complete surgical resection could induce negative effect on the development of permanent teeth and growth of jaw. Herein, we reported successful treatment case of young KCOT patient with NBCCS...
2017: Journal of Clinical Pediatric Dentistry
https://www.readbyqxmd.com/read/28650485/neuroblastoma-is-composed-of-two-super-enhancer-associated-differentiation-states
#3
Tim van Groningen, Jan Koster, Linda J Valentijn, Danny A Zwijnenburg, Nurdan Akogul, Nancy E Hasselt, Marloes Broekmans, Franciska Haneveld, Natalia E Nowakowska, Johannes Bras, Carel J M van Noesel, Aldo Jongejan, Antoine H van Kampen, Linda Koster, Frank Baas, Lianne van Dijk-Kerkhoven, Margriet Huizer-Smit, Maria C Lecca, Alvin Chan, Arjan Lakeman, Piet Molenaar, Richard Volckmann, Ellen M Westerhout, Mohamed Hamdi, Peter G van Sluis, Marli E Ebus, Jan J Molenaar, Godelieve A Tytgat, Bart A Westerman, Johan van Nes, Rogier Versteeg
Neuroblastoma and other pediatric tumors show a paucity of gene mutations, which has sparked an interest in their epigenetic regulation. Several tumor types include phenotypically divergent cells, resembling cells from different lineage development stages. It has been proposed that super-enhancer-associated transcription factor (TF) networks underlie lineage identity, but the role of these enhancers in intratumoral heterogeneity is unknown. Here we show that most neuroblastomas include two types of tumor cells with divergent gene expression profiles...
June 26, 2017: Nature Genetics
https://www.readbyqxmd.com/read/28649600/oncolytic-herpes-virus-rrp450-shows-efficacy-in-orthotopic-xenograft-group-3-4-medulloblastomas-and-atypical-teratoid-rhabdoid-tumors
#4
Adam W Studebaker, Brian J Hutzen, Christopher R Pierson, Kellie B Haworth, Timothy P Cripe, Eric M Jackson, Jeffrey R Leonard
Pediatric brain tumors including medulloblastoma and atypical teratoid/rhabdoid tumor are associated with significant mortality and treatment-associated morbidity. While medulloblastoma tumors within molecular subgroups 3 and 4 have a propensity to metastasize, atypical teratoid/rhabdoid tumors frequently afflict a very young patient population. Adjuvant treatment options for children suffering with these tumors are not only sub-optimal but also associated with many neurocognitive obstacles. A potentially novel treatment approach is oncolytic virotherapy, a developing therapeutic platform currently in early-phase clinical trials for pediatric brain tumors and recently US Food and Drug Administration (FDA)-approved to treat melanoma in adults...
September 15, 2017: Molecular Therapy Oncolytics
https://www.readbyqxmd.com/read/28647700/molecular-proteomic-characterization-of-a-pediatric-medulloblastoma-xenograft
#5
George T Tsangaris, Konstantinos Dimas, Angeliki Malamou, Angeliki Katsafadou, Chrissa Papathanasiou, Dimitrios J Stravopodis, Constantinos E Vorgias, Maria Gazouli, Athanasios K Anagnostopoulos
BACKGROUND/AIM: Medulloblastoma (MBL), an archetypal primitive neuroectodermal tumor of the cerebellum, is the most common pediatric central nervous system malignancy representing approximately 20% of all childhood brain tumors. Herein, we report on a new xenotransplantable tumor cell line, derived from a 6-year-old female patient with cerebellar medulloblastoma, and the completele proteome molecular characterization of subsequent tumors from MBL xenotrasplanted mice. MATERIALS AND METHODS: Tumors were grown in nude mice as subcutaneous xenografts (MBLX) composed of small round cells with hyperchromatic nuclei and scant cytoplasm...
July 2017: Cancer Genomics & Proteomics
https://www.readbyqxmd.com/read/28646474/correlation-between-braf-v600e-mutation-and-clinicopathological-features-in-pediatric-papillary-thyroid-carcinoma
#6
Jiangqiao Geng, Huanmin Wang, Yuanhu Liu, Jun Tai, Yaqiong Jin, Jie Zhang, Lejian He, Libing Fu, Hong Qin, Yingluan Song, Jinzhu Su, Aiying Zhang, Xin Wen, Yongli Guo, Xin Ni
In adults, the presence of the BRAF (V600E) mutation in papillary thyroid cancer (PTC) has been demonstrated to be strongly associated with aggressive cancer-cell characteristics and poor patient prognosis. In contrast, the frequency of this mutation in pediatric PTC has undergone limited study, and the few available estimates range from 0 to 63%. Furthermore, the role of the BRAF (V600E) mutation in pediatric PTC is controversial; thus, the present study aimed to investigate the prevalence and role of the BRAF (V600E) mutation in 48 pediatric patients with PTC, aged 3-13 years...
June 15, 2017: Science China. Life Sciences
https://www.readbyqxmd.com/read/28644354/development-and-function-of-immune-cells-in-an-adolescent-patient-with-a-deficiency-in-the-interleukin-10-receptor
#7
Sharon Veenbergen, Marieke A van Leeuwen, Gertjan J Driessen, Rogier Kersseboom, Lilian F de Ruiter, Rolien H C Raatgeep, Dicky J Lindenbergh-Kortleve, Ytje Simons-Oosterhuis, Katharina Biermann, Dicky J J Halley, Lissy de Ridder, Johanna C Escher, Janneke N Samsom
OBJECTIVE: Monogenic defects in the interleukin-10 (IL-10) pathway are extremely rare and cause infantile-onset inflammatory bowel disease (IBD)-like pathology. Understanding how immune responses are dysregulated in monogenic IBD-like diseases can provide valuable insight in "classical" IBD pathogenesis. Here, we studied long-term immune cell development and function in an adolescent IL-10 receptor (IL10RA)-deficient patient who presented in infancy with severe colitis and fistulizing perianal disease and is currently treated with immune suppressants...
July 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28644246/effects-of-exercise-on-the-immune-function-of-pediatric-patients-with-solid-tumors-insights-from-the-papec-randomized-trial
#8
Carmen Fiuza-Luces, Julio R Padilla, Jaime Valentín, Elena Santana-Sosa, Alejandro Santos-Lozano, Fabián Sanchis-Gomar, Helios Pareja-Galeano, Javier S Morales, Steven J Fleck, Margarita Pérez, Alvaro Lassaletta, Luisa Soares-Miranda, Antonio Pérez-Martínez, Alejandro Lucia
The purpose of this study was to assess the effects of an in-hospital exercise intervention during neoadjuvant chemotherapy on the inflammatory profile and immune cell subpopulation in 20 children with solid tumors (control [n = 11] and exercise group [n = 9]). Although no significant interaction (group × time) effect was found with an analysis of variance test, we found a trend toward an interaction effect for natural killer cells expressing the immunoglobulin-like receptor KIR2DS4, with their numbers remaining stable in the exercise group but increasing in controls...
June 21, 2017: American Journal of Physical Medicine & Rehabilitation
https://www.readbyqxmd.com/read/28644072/clinicopathological-and-imaging-features-of-lipoastrocytoma-case-report
#9
Laxminadh Sivaraju, Saritha Aryan, Nandita Ghosal, Alangar S Hegde
Lipidized tumors of the central nervous system are very uncommon, with only a few cases described. We report a case of a 25-year-old woman with a tumor involving the left premotor area. She underwent gross total excision. Histologically, the tumor was composed of glial fibrillary acidic protein-positive glial cells with areas of lipidization. A diagnosis of lipoastrocytoma was rendered. At three-year follow-up she was doing well, supporting the presumed favorable prognosis of these uncommon tumors. Absence of xanthochromic appearance, mitotic activity, necrosis and poor reticulin activity are the differentiating features from the pleomorphic xanthoastrocytoma...
January 1, 2017: Neuroradiology Journal
https://www.readbyqxmd.com/read/28643220/denosumab-an-emerging-therapy-in-pediatric-bone-disorders
#10
REVIEW
Alison M Boyce
PURPOSE OF REVIEW: Denosumab is an inhibitor of receptor activator of nuclear factor kappa-B ligand (RANKL), and has emerged as an important novel therapy for skeletal disorders. This article examines the use of denosumab in children. RECENT FINDINGS: Considerable safety and efficacy data exists for denosumab treatment of adults with osteoporosis, bone metastases, and giant cell tumors. Pediatric data is limited; however, evidence suggests denosumab may be beneficial in decreasing bone turnover, increasing bone density, and preventing growth of certain skeletal neoplasms in children...
June 22, 2017: Current Osteoporosis Reports
https://www.readbyqxmd.com/read/28643124/-solid-pediatric-tumors-a-brief-survey-of-the-rarity-cabinet
#11
B Gürtl-Lackner, D Gisselsson-Nord, G Vujanic
Solid tumors in childhood are extremely rare entities, which are usually treated in specialized centers. Diagnosis and therapy are carried out according to a joint European protocol, whereby the pathological evaluation and therapy are carried out according to international guidelines. For the correct diagnosis and/or therapy of most tumors, analysis of specific genetic changes is mandatory; therefore, tumors have to be adequately sampled for parallel genetic analysis during the pathological work-up. A second opinion reference of the histopathological assessment is part of the international guidelines...
June 22, 2017: Der Pathologe
https://www.readbyqxmd.com/read/28643039/primary-intraventricular-meningiomas-in-children-experience-of-two-cases-with-review-of-literature
#12
Kapil D Muley, Salman T Shaikh, Chandrashekhar E Deopujari, Uday B Andar
Meningiomas are rare in the pediatric age group, more so in the intraventricular location. They arise in the lateral ventricles from the arachnoid cells contained within the choroid plexus, in the third ventricle from the velum interpositum and in the fourth ventricle from the choroids. These tumors are usually large and have an aggressive behaviour. Surgical management of intra-ventricular meningiomas is challenging because of their deep location, large size at presentation and increased vascularity. The authors report two such cases who presented with symptoms of raised intra cranial pressure and on evaluation were found to have associated hydrocephalus...
June 22, 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/28641811/tension-pneumocephalus-following-suboccipital-sitting-craniotomy-in-the-pediatric-population
#13
P Daszkiewicz, D Dziedzic
BACKGROUND: Sitting craniotomy often results in entrapment of air in fluid-filled intracranial cavities. Gas under pressure exerts a deleterious effect on adjacent nervous tissue, resulting in clinical deterioration. AIM OF STUDY: To assess the incidence of tension pneumocephalus (TP) and to define risk factors associated therewith. MATERIAL AND METHOD: Analysis included 100 consecutive patients (57 boys, 43 girls, mean age 9.7 y) undergoing suboccipital sitting craniotomy since 2012 to 2014...
May 11, 2017: Neurologia i Neurochirurgia Polska
https://www.readbyqxmd.com/read/28640941/phox2b-reliably-distinguishes-neuroblastoma-among-small-round-blue-cell-tumors
#14
Yin P Hung, John P Lee, Andrew M Bellizzi, Jason L Hornick
AIMS: Neuroblastoma shows considerable histologic overlap with other small round blue cell tumors. PHOX2B, a transcription factor essential for autonomic nervous system development, has been reported as an immunohistochemical marker for neuroblastoma. The purpose of this study was to validate the specificity and diagnostic utility of PHOX2B for peripheral neuroblastic tumors. METHODS AND RESULTS: We evaluated 240 cases (133 in whole-tissue sections; 107 in tissue microarrays), including 76 peripheral neuroblastic tumors [median age 2 years; including 4 adults] and 164 other tumors: 44 Wilms tumors; 20 Ewing sarcomas; 10 each CIC-rearranged round cell sarcomas, poorly differentiated synovial sarcomas, lymphoblastic lymphomas, alveolar rhabdomyosarcomas, embryonal rhabdomyosarcomas, mesenchymal chondrosarcomas, Merkel cell carcinomas, olfactory neuroblastomas, and melanomas; 5 each NUT midline carcinomas and desmoplastic small round cell tumors...
June 22, 2017: Histopathology
https://www.readbyqxmd.com/read/28640707/response-assessment-in-neuro-oncology-clinical-trials
#15
Patrick Y Wen, Susan M Chang, Martin J Van den Bent, Michael A Vogelbaum, David R Macdonald, Eudocia Q Lee
Development of novel therapies for CNS tumors requires reliable assessment of response and progression. This requirement has been particularly challenging in neuro-oncology for which contrast enhancement serves as an imperfect surrogate for tumor volume and is influenced by agents that affect vascular permeability, such as antiangiogenic therapies. In addition, most tumors have a nonenhancing component that can be difficult to accurately quantify. To improve the response assessment in neuro-oncology and to standardize the criteria that are used for different CNS tumors, the Response Assessment in Neuro-Oncology (RANO) working group was established...
June 22, 2017: Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology
https://www.readbyqxmd.com/read/28640705/genomic-analysis-of-childhood-brain-tumors-methods-for-genome-wide-discovery-and-precision-medicine-become-mainstream
#16
Stephen C Mack, Paul A Northcott
Recent breakthroughs in next-generation sequencing technology and complementary genomic platforms have transformed our capacity to interrogate the molecular landscapes of human cancers, including childhood brain tumors. Numerous high-throughput genomic studies have been reported for the major histologic brain tumor entities diagnosed in children, including interrogations at the level of the genome, epigenome, and transcriptome, many of which have yielded essential new insights into disease biology. The nature of these discoveries has been largely platform dependent, exemplifying the usefulness of applying different genomic and computational strategies, or integrative approaches, to address specific biologic and/or clinical questions...
June 22, 2017: Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology
https://www.readbyqxmd.com/read/28640698/pediatric-gliomas-current-concepts-on-diagnosis-biology-and-clinical-management
#17
Dominik Sturm, Stefan M Pfister, David T W Jones
Gliomas are the most common CNS tumors in children and adolescents, and they show an extremely broad range of clinical behavior. The majority of pediatric gliomas present as benign, slow-growing lesions classified as grade I or II by the WHO classification of CNS tumors. These pediatric low-grade gliomas (LGGs) are fundamentally different from IDH-mutant LGGs occurring in adults, because they rarely undergo malignant transformation and show excellent overall survival under current treatment strategies. However, a significant fraction of gliomas develop over a short period of time and progress rapidly and are therefore classified as WHO grade III or IV high-grade gliomas (HGGs)...
June 22, 2017: Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology
https://www.readbyqxmd.com/read/28640695/gadolinium-brain-deposition-after-macrocyclic-gadolinium-administration-a-pediatric-case-control-study
#18
Daniel Tibussek, Christin Rademacher, Julian Caspers, Bernd Turowski, Jörg Schaper, Gerald Antoch, Dirk Klee
Purpose To determine whether signal intensity (SI) in T1 sequences as a potential indicator of gadolinium deposition increases after repeated administration of the macrocyclic gadolinium-based contrast agents (GBCAs) gadoteridol and gadoterate meglumine in a pediatric cohort. Materials and Methods This retrospective case-control study of children with brain tumors who underwent nine or more contrast material-enhanced brain magnetic resonance (MR) imaging studies from 2008 to 2015 was approved by the local ethics board...
June 21, 2017: Radiology
https://www.readbyqxmd.com/read/28640379/comparative-and-correlative-assessments-of-cytokine-complement-and-antibody-patterns-in-pediatric-type-1-diabetes
#19
M Abdel-Latif, A A Abdel-Moneim, M H El-Hefnawy, R G Khalil
One of the most widespread and effective environmental factors is the infection with enteroviruses (EVs) which accelerate β - cell destruction in type 1 diabetes (T1D). This study represented a comparison between diabetic EV(+) and EV- children as well as correlation analysis between autoantibodies, T1D markers, cytokines, complement activation products and anti- coxsackievirus (CV) IgG. EV RNA was detected in Egyptian children with T1D (26.2%) and healthy controls (0%). Detection of anti - CV IgG in T1D - EV(+) resulted in 64% positivity...
June 22, 2017: Clinical and Experimental Immunology
https://www.readbyqxmd.com/read/28639954/rare-endobronchial-inflammatory-myofibroblastic-tumor-in-pediatric-patient-detected-on-pet-ct-imaging
#20
Pelin Ozcan Kara, Zehra Pinar Koc, Elvan Caglar Citak, Ali Nayci, Gokhan Berktug Bahadir, Taylan Kara
Inflammatory myofibroblastic tumor (IMT) can be seen in all age groups, although it is more common in children and adolescents. We report the FDG PET/CT findings in an 8-year-old boy with endobronchial IMT. Endobronchial IMT is more commonly seen in young adults.
June 20, 2017: Clinical Nuclear Medicine
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