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Ewings sarcoma

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https://www.readbyqxmd.com/read/28938120/prion-like-domains-program-ewing-s-sarcoma
#1
James Shorter
Prion-like domains have emerged as important drivers of neurodegenerative disease. Now, Boulay et al. establish that the translocated prion-like domain of the oncogenic EWS-FLI1 fusion protein enables phase-separation events, which inappropriately recruit chromatin-remodeling factors to elicit the aberrant transcriptional programs underlying Ewing's sarcoma.
September 21, 2017: Cell
https://www.readbyqxmd.com/read/28936339/utility-of-vs38c-in-the-diagnostic-and-prognostic-assessment-of-osteosarcoma-and-other-bone-tumours-tumour-like-lesions
#2
E S Hookway, Z Orosz, Y Uchihara, A Grigoriadis, A B Hassan, U Oppermann, N A Athanasou
BACKGROUND: VS38c is a monoclonal antibody that recognises a rough endoplasmic reticulum (rER) intracellular antigen termed cytoskeleton-linking membrane protein 63. rER is typically found in viable tumour cells and is abundant in osteosarcoma cells. The aim of this study was to determine the diagnostic and prognostic utility of VS38c in the histological assessment of osteosarcoma and other bone tumours/tumour-like leisons. METHODS: Immunohistochemical staining with VS38c was carried out on formalin-fixed specimens of osteosarcoma (pre/post-chemotherapy) and a wide range of benign and malignant bone lesions...
2017: Clinical Sarcoma Research
https://www.readbyqxmd.com/read/28929078/primary-ewing-s-sarcoma-of-the-temporal-bone-a-rare-case-report-and-literature-review
#3
Divya Gupta, Achal Gulati, Purnima
Ewing's sarcoma is a malignant, round cell tumor arising from the bones and primarily affecting children and adolescent, accounting for 3 % of all childhood malignancies. Although the long bones and the trunk are typically affected, rare cases of it involving isolated bones throughout the body have been reported. Involvement of the skull bones is rare, constituting 1-6 % of the total Ewing's sarcoma cases but those affecting the cranial bones are rarer still, constituting only 1 %. We describe an 8 months old infant having Ewing sarcoma, of the petrous and mastoid parts of temporal bone along with the occipital bone, whose clinical presentation mimicked mastoiditis with facial nerve palsy...
September 2017: Indian Journal of Otolaryngology and Head and Neck Surgery
https://www.readbyqxmd.com/read/28928879/-18-f-fdg-pet-ct-as-an-indicator-of-survival-in-ewing-sarcoma-of-bone
#4
Usama Salem, Behrang Amini, Hubert H Chuang, Najat C Daw, Wei Wei, Tamara Miner Haygood, John E Madewell, Colleen M Costelloe
Objective: The existing literature of 18 F-FDG PET/CT in Ewing sarcoma investigates mixed populations of patients with both soft tissue and bone primary tumors. The aim of our study was to evaluate whether the maximum standardized uptake value (SUVmax) obtained with 18F-FDG PET/CT before and after induction chemotherapy can be used as an indicator of survival in patients with Ewing sarcoma originating exclusively in the skeleton. Materials and Methods: A retrospective database search from 2004-2011 identified 28 patients who underwent 18 F-FDG PET/CT before (SUV1, n= 28) and after (SUV2, n=23) induction chemotherapy...
2017: Journal of Cancer
https://www.readbyqxmd.com/read/28903844/bone-marrow-involvement-in-metastatic-pediatric-ewing-sarcoma
#5
Saadiya Javed Khan, Irfana Ishaq, Haleema Saeed, Muhammad Bilal Fayyaz, Syed Ali Shazif Baqari, Rabia Mohammad Wali
OBJECTIVE: To evaluate the frequency of bone marrow involvement with metastatic lung and bone sites in newly-diagnosed pediatric patients with Ewing sarcoma (ES). STUDY DESIGN: An observational study. PLACE AND DURATION OF STUDY: Shaukat Khanum Memorial Cancer Hospital, Lahore, Pakistan, from January 2010 to October 2015. METHODOLOGY: Newly-diagnosed pediatric-age patients with ES were inducted. Ten patients were excluded because bone marrow aspiration/biopsy (BMAB) was not done...
August 2017: Journal of the College of Physicians and Surgeons—Pakistan: JCPSP
https://www.readbyqxmd.com/read/28902354/ews-fli1-positively-regulates-autophagy-by-increasing-atg4b-expression-in-ewing-sarcoma-cells
#6
Qunshan Lu, Yuankai Zhang, Liang Ma, Deqiang Li, Ming Li, Jianmin Li, Peilai Liu
Ewing sarcoma (ES) is the most common malignant bone tumor in children and young adults. It is characterized by chromosomal translocations fusing the EWS gene with an ETS oncogene, most frequently FLI1. In the present study, the authors aimed to investigate the function of EWS-FLI1 in autophagy in ES cells, and identified that EWS-FLI1 positively regulates autophagy in ES cells. ATG4B expression was observed markedly upregulated by EWS-FLI1 overexpression, and silencing of ATG4B dramatically inhibits autophagy in ES cells...
October 2017: International Journal of Molecular Medicine
https://www.readbyqxmd.com/read/28900327/metastatic-ewing-s-sarcoma-revisiting-the-evidence-on-the-fence
#7
REVIEW
Nehal Khanna, Avinash Pandey, Jyoti Bajpai
Metastatic Ewing's sarcoma is a challenging disease for oncology care providers with wide spectrum of disease at presentation, widely varying approach to the treatment and varied outcomes. The paucity of randomized evidence is a barrier in developing a consensus. This perspective provides the evidence "for and against" the benefit of aggressive approach including local and systemic therapy in patients presenting with metastatic Ewing's sarcoma and provide general recommendations so as to help select patients who will benefit with definitive intent treatment and also, avoid aggressive approach in patients with dismal outcome...
April 2017: Indian Journal of Medical and Paediatric Oncology
https://www.readbyqxmd.com/read/28899971/matrix-screen-identifies-synergistic-combination-of-parp-inhibitors-and-nicotinamide-phosphoribosyltransferase-nampt-inhibitors-in-ewing-sarcoma
#8
Christine M Heske, Mindy I Davis, Joshua T Baumgart, Kelli M Wilson, Michael V Gormally, Lu Chen, Xiaohu Zhang, Michele Ceribelli, Damien Duveau, Rajarshi Guha, Marc Ferrer, Fernanda I Arnaldez, Jiuping Jay Ji, Huong-Lan Tran, Yiping Zhang, Arnulfo Mendoza, Lee J Helman, Craig J Thomas
PURPOSE: While many cancers are showing remarkable responses to targeted therapies, pediatric sarcomas, including Ewing sarcoma, remain recalcitrant. To broaden the therapeutic landscape, we explored the in vitro response of Ewing sarcoma cell lines against a large collection of investigational and approved drugs to identify candidate combinations. EXPERIMENTAL DESIGN: Drugs displaying activity as single agents were evaluated in combinatorial (matrix) format to identify highly active, synergistic drug combinations, and combinations were subsequently validated in multiple cell lines using various agents from each class...
September 12, 2017: Clinical Cancer Research: An Official Journal of the American Association for Cancer Research
https://www.readbyqxmd.com/read/28888998/tnf-related-apoptosis-inducing-ligand-trail-for-bone-sarcoma-treatment-pre-clinical-and-clinical-data
#9
Kenneth Samora Rankin, Eleftherios Tsiridis, Craig Gerrand, Anja Krippner-Heidenreich, Kenneth Dalgarno, Ricardo Da Conceicao Ribeiro, Emma Haagensen, Dimitra Papanikolaou, Kapriniotis Konstantinos, Zakareya Gamie
Bone sarcomas are rare, highly malignant mesenchymal tumours that affect teenagers and young adults, as well as older patients. Despite intensive, multimodal therapy, patients with bone sarcomas have poor 5-year survival, close to 50%, with lack of improvement over recent decades. TNF-related apoptosis-inducing ligand (TRAIL), a member of the tumour necrosis factor (TNF) ligand superfamily (TNFLSF), has been found to induce apoptosis in cancer cells while sparing nontransformed cells, and may therefore offer a promising new approach to treatment...
September 6, 2017: Cancer Letters
https://www.readbyqxmd.com/read/28884875/successful-collection-of-peripheral-blood-stem-cells-upon-vide-chemomobilization-in-sarcoma-patients
#10
Katharina Kriegsmann, Christoph Heilig, Martin Cremer, Philipp Novotny, Mark Kriegsmann, Thomas Bruckner, Carsten Müller-Tidow, Gerlinde Egerer, Patrick Wuchter
OBJECTIVES: In patients with Ewing sarcoma and some distinct subgroups of soft tissue sarcoma (STS), a quantitatively sufficient autologous peripheral blood stem cell (PBSC) collection for stem cell support might facilitate treatment continuation, dose-intensification and high-dose chemotherapy. Here we provide a detailed evaluation of PBSC collection upon vincristine, ifosfamide, doxorubicin, and etoposide (VIDE) chemomobilization. METHODS: Mobilization and collection parameters of 42 sarcoma patients (Ewing sarcoma n=35, other STS n=7) were analyzed retrospectively...
September 8, 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/28882943/tissue-directed-implantation-using-ultrasound-visualization-for-development-of-biologically-relevant-metastatic-tumor-xenografts
#11
Raelene A VAN Noord, Tina Thomas, Melanie Krook, Sahiti Chukkapalli, Mark J Hoenerhoff, Jonathan R Dillman, Elizabeth R Lawlor, Valerie P Opipari, Erika A Newman
BACKGROUND: Advances in cancer therapeutics depend on reliable in vivo model systems. To develop biologically relevant xenografts, ultrasound was utilized for tissue-directed implantation of neuroblastoma (NB) cell line and patient-derived tumors in the adrenal gland, and for renal subcapsular engraftment of Ewing's sarcoma (ES). MATERIALS AND METHODS: NB xenografts were established by direct adrenal injection of luciferase-transfected NB cell lines (IMR32, SH-SY5Y, SK-N-BE2) or NB patient-derived tumor cells (UMNBL001, UMNBL002)...
September 2017: In Vivo
https://www.readbyqxmd.com/read/28881742/eribulin-alone-or-in-combination-with-the-plk1-inhibitor-bi-6727-triggers-intrinsic-apoptosis-in-ewing-sarcoma-cell-lines
#12
Lilly Magdalena WeiΔ, Manuela Hugle, Simone Fulda
In this study, we investigated the molecular mechanisms of eribulin-induced cell death and its therapeutic potential in combination with the PLK1 inhibitor BI 6727 in Ewing sarcoma (ES). Here, we show that eribulin triggers cell death in a dose-dependent manner in a panel of ES cell lines. In addition, eribulin at subtoxic, low nanomolar concentrations acts in concert with BI 6727 to induce cell death and to suppress long-term clonogenic survival. Mechanistic studies reveal that eribulin monotherapy at cytotoxic concentrations and co-treatment with eribulin at subtoxic concentrations together with BI 6727 arrest cells in the M phase of the cell cycle prior to the onset of cell death...
August 8, 2017: Oncotarget
https://www.readbyqxmd.com/read/28878542/ewings-sarcoma-of-ilium-resection-and-reconstruction-with-femoral-head-allograft
#13
Yugal Karkhur, Lalit Maini, Anurag Tiwari, Tarun Verma
Ewing's sarcoma is a common malignant bone tumor seen in 5-15 years age group. It often arises from diaphysis of long bones. Ewing's sarcoma arising from the ilium is very rare, and it has an unfavourable prognosis. We present a rare case report of Ewings sarcoma of ilium with no metastasis in a two and a half year old boy, who was treated with neoadjuvant chemotherapy followed by surgical excision of the tumor and reconstruction using allograft from the femoral head fixed with multiple k-wires and screw. The patient is disease free at one year follow up and the allograft has taken the shape of growing ilium and excellent functionality and gait with minimal limp...
August 2017: Journal of Clinical Orthopaedics and Trauma
https://www.readbyqxmd.com/read/28873262/reactivation-of-twist1-contributes-to-ewing-sarcoma-metastasis
#14
Sun Choo, Ping Wang, Robert Newbury, William Roberts, Jing Yang
BACKGROUND: Ewing sarcoma is a cancer of bone and soft tissue. Despite aggressive treatment, survival remains poor, particularly in patients with metastatic disease. Failure to treat Ewing sarcoma is due to the lack of understanding of the molecular pathways that regulate metastasis. In addition, no molecular prognostic markers have been identified for Ewing sarcoma to risk stratify patients. PROCEDURE: Ewing sarcoma patients were divided into high or low Twist1 gene expression and survival curves were generated using the R2 microarray-based Genomic Analysis platform (http://r2...
September 5, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28869107/molecular-characterization-of-a-series-of-solitary-fibrous-tumors-including-immunohistochemical-expression-of-stat6-and-natb2-stat6-fusion-transcripts-using-reverse-transcriptase-rt-polymerase-chain-reaction-pcr-technique-an-indian-experience
#15
Bharat Rekhi, Omshree Shetty, Parul Tripathi, Prachi Bapat, Mukta Ramadwar, Jyoti Bajpai, Ajay Puri
A solitary fibrous tumor (SFT) is characterized by a diverse clinicopathologic spectrum. Recent studies have unraveled STAT6 as a useful diagnostic immunohistochemical (IHC) marker for a SFT and NAB2-STAT6 as its specific gene fusion transcript. Thirty-three SFTs were tested for STAT6 immunostaining by polymer detection technique. STAT6 immunoexpression was further graded, based on intensity (mild, moderate and strong) and percentage of immunopositive tumor cells, ranging from 1 to 25%(1+); 26-50%(2+); 51-75%(3+) and in more than 75%(4+) tumor nuclei...
August 26, 2017: Pathology, Research and Practice
https://www.readbyqxmd.com/read/28868758/programmed-cell-death-ligand-1-pd-l1-expression-is-not-a-predominant-feature-in-ewing-sarcomas
#16
Christian Spurny, Sareetha Kailayangiri, Silke Jamitzky, Bianca Altvater, Eva Wardelmann, Uta Dirksen, Jendrik Hardes, Wolfgang Hartmann, Claudia Rossig
BACKGROUND: Programmed cell death 1 (PD-1) receptor engagement on T cells by its ligand programmed cell death ligand 1 (PD-L1) is a key mechanism of immune escape, and antibody blockade of the interaction has emerged as an effective immunotherapeutic strategy in some cancers. The role and relevance of the PD-1 checkpoint in Ewing sarcoma (EwS) is not yet understood. PROCEDURE: Here, we investigated expression of PD-L1 and PD-1 in EwS by immunohistochemistry analysis of pretherapeutic tumor biopsies and in tumor xenografts following treatment with human T cells engineered to express a chimeric antigen receptor (CAR) against the tumor-associated antigen GD2 ...
September 4, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28864478/ews-fli1-retargets-baf-chromatin-remodeling-complexes-in-ewing-sarcoma
#17
(no author information available yet)
BAF recruitment to GGAA microsatellites activates the Ewing sarcoma transcriptional program.
September 1, 2017: Cancer Discovery
https://www.readbyqxmd.com/read/28864350/immunohistochemical-analysis-of-nkx2-2-etv4-and-bcor-in-a-large-series-of-genetically-confirmed-ewing-sarcoma-family-of-tumors
#18
Isidro Machado, Akihiko Yoshida, José Antonio López-Guerrero, María Gema Nieto, Samuel Navarro, Piero Picci, Antonio Llombart-Bosch
Ewing sarcoma is an aggressive neoplasm of pediatric and adolescent patients. Immunohistochemistry (IHC) can be used to support the morphologic diagnosis of Ewing sarcoma family of tumors (ESFT) in a convincing clinical/radiological context. Although neither NKX2.2 nor CD99 alone are entirely specific, when combined, the diagnostic specificity is high. The aim of the present study was to investigate the IHC expression of NKX2.2, ETV4 and BCOR in a large series of genetically confirmed ESFT. The results for CD99 and CAV-1 immunoreactivity, and the histological and fusion gene subtypes were retrieved from our previous study...
August 25, 2017: Pathology, Research and Practice
https://www.readbyqxmd.com/read/28863036/clinical-activity-of-eribulin-in-advanced-desmoplastic-small-round-cell-tumor
#19
Sheik Emambux, Michele Kind, Francois Le Loarer, Maud Toulmonde, Eberhard Stoeckle, Antoine Italiano
Desmoplastic small round-cell tumor is a rare but highly aggressive tumor occurring mainly in adolescents and young adults. Prolonged progression-free survival has been documented in patients who have undergone aggressive multimodality therapy - that is, multiagent intensive chemotherapy, debulking surgery, and radiation therapy. Eribulin is a microtubule-dynamics inhibitor, and it has recently been shown to be active in liposarcomas. In preclinical models, eribulin activities have also been shown to occur in Ewing's sarcoma cell lines, rhabdomyosarcomas and osteosarcomas...
August 31, 2017: Anti-cancer Drugs
https://www.readbyqxmd.com/read/28853064/receptor-activator-of-nuclear-transcription-factor-nf-%C3%AE%C2%BAb-rank-its-ligand-rankl-and-natural-inhibitor-of-rankl-osteoprotegerin-opg-in-the-blood-serum-of-patients-with-primary-bone-tumors
#20
N E Kushlinskii, E S Gershtein, Yu N Solov'ev, Yu S Timofeev, I V Babkina, A O Dolinkin, A A Zuev, O I Kostyleva
The content of components of the RANK/RANKL/OPG system, the key regulator of homeostasis in the bone tissue, in blood serum samples from 199 patients with primary bone neoplasms and 131 practically healthy volunteers was measured by ELISA. Borderline giantcell tumor of the bone with high osteoclastogenic and osteolytic activity is characterized by an increase in the level of all components of this system and highest ratio of sRANKL/OPG in the blood serum. Study indexes in patients with various benign neoplasms and tumor-like bone lesions were lower than in patients with giant-cell tumor...
August 2017: Bulletin of Experimental Biology and Medicine
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