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Ewings sarcoma

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https://www.readbyqxmd.com/read/28222219/longitudinal-follow-up-of-adult-survivors-of-ewing-sarcoma-a-report-from-the-childhood-cancer-survivor-study
#1
Neyssa M Marina, Qi Liu, Sarah S Donaldson, Charles A Sklar, Gregory T Armstrong, Kevin C Oeffinger, Wendy M Leisenring, Jill P Ginsberg, Tara O Henderson, Joseph P Neglia, Marilyn A Stovall, Yutaka Yasui, R Lor Randall, David S Geller, Leslie L Robison, Kirsten K Ness
BACKGROUND: Ewing sarcoma survivors (ESSs) are at increased risk for treatment-related complications. The incidence of treatment-related morbidity and late mortality with aging is unknown. METHODS: This study reports survival probabilities, estimated with the Kaplan-Meier method, and the cumulative incidence of cause-specific mortality and chronic conditions among ESSs in the Childhood Cancer Survivor Study who were treated between 1970 and 1986. Piecewise exponential models were used to estimate relative rates (RRs) and 95% confidence intervals (CIs) for these outcomes...
February 21, 2017: Cancer
https://www.readbyqxmd.com/read/28221727/docetaxel-bevacizumab-and-gemcitabine-for-very-high-risk-sarcomas-in-adolescents-and-young-adults-a-single-center-experience
#2
Christopher Kuo, Paul M Kent, Antonio D Logan, Karen B Tamulonis, Kristen L Dalton, Marta Batus, Karen Fernandez, Rebecca E Mcfall
BACKGROUND: Adolescent and young adult (AYA) patients with very high risk sarcomas have poor outcomes and are in need of novel therapies. PROCEDURE: From January 2005 to February 2016, we retrospectively identified all AYA patients with relapsed or metastatic high-grade sarcomas, who were treated with at least one cycle of docetaxel (T), bevacizumab (A), and gemcitabine (G) (TAG ; T = 100 mg/m(2) Day 8, A = 15 mg/kg Day 1, G = 1,000 mg/m(2) Days 1 and 8). RESULTS: Fourteen patients, median age of 20 (15-30), received a total of 80 cycles of TAG, and were followed for a median of 83 months...
April 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28216818/limb-salvage-with-microvascular-free-fibula-following-primary-bone-sarcoma-resection
#3
Sahasrabudhe Parag, Panchwagh Yogesh, Jesal Rathod, Panse Nikhil, Jadhav Amit
BACKGROUND: Extremity sarcomas are challenging to manage. Total eradication of tumour has to be balanced with restoration of limb function to prevent mortality and morbidity. Disease-free survival with maximum limb function is the ultimate goal in these patients. MATERIALS AND METHODS: We present a series of ten cases of extremity malignancies, where limb salvage was attempted with microvascular free fibula for limb reconstruction from the period of 2008 to 2015...
September 2016: Indian Journal of Plastic Surgery: Official Publication of the Association of Plastic Surgeons of India
https://www.readbyqxmd.com/read/28213847/ultra-short-stem-anchorage-in-the-proximal-tibial-epiphysis-after-intercalary-tumor-resections-analysis-of-reconstruction-survival-in-four-patients-at-a-mean-follow-up-of-56-months
#4
W K Guder, J Hardes, G Gosheger, M Nottrott, A Streitbürger
INTRODUCTION: Tumors localized in the proximal tibial meta-diaphysis often lead to osteoarticular resections. MATERIALS AND METHODS: In this study, we retrospectively reviewed four patients who underwent intercalary tumor resection and reconstruction using an ultra-short stem in the proximal tibial epiphysis, a procedure that to our knowledge has not been reported in literature so far. RESULTS: At the time of operation, the mean patient age was 26...
February 17, 2017: Archives of Orthopaedic and Trauma Surgery
https://www.readbyqxmd.com/read/28210452/ewing-sarcoma-of-the-oral-cavity-a-review
#5
REVIEW
Maria Margaix-Muñoz, José Bagán, Rafael Poveda-Roda
OBJECTIVES: A review is made of the clinical, diagnostic, therapeutic and survival characteristics of Ewing sarcoma (ES) of the oral cavity. MATERIAL AND METHODS: A systematic literature search was carried out, with restrictions referred to time (1960-2014), language (English and Spanish) and type of study (case reports, letters, datasets, reviews). The following MeSH terms and boolean operators were used: Ewing AND Sarcoma AND [tongue, jaw, maxilla, cheek, condyle OR temporomandibular, floor AND mouth, gum OR gingiva, palate OR palatal, lip, uvula, head AND neck]...
February 2017: Journal of Clinical and Experimental Dentistry
https://www.readbyqxmd.com/read/28203170/primary-ewing-s-sarcoma-of-the-sinonasal-tract-a-case-report
#6
Tomoharu Suzuki, Ryuji Yasumatsu, Torahiko Nakashima, Shuji Arita, Hidetaka Yamamoto, Takashi Nakagawa
A 23-year-old male presented with a 3-month history of left purulent rhinorrhea, progressive nasal obstruction, and intermittent epistaxis. A fiberoptic examination revealed a large vascular polypoid mass completely filling the left nasal cavity. CT and MRI scans showed a large hypervascular mass involving the left nasal airway, maxillary antrum, and the anterior ethmoid cells. There was no bony erosion or contiguous spread, and the remaining sinuses, orbit, and cranial fossa were uninvolved. The patient underwent complete removal of the mass via an external lateral rhinotomy approach...
January 2017: Case Reports in Oncology
https://www.readbyqxmd.com/read/28199377/oncological-and-surgical-outcome-after-treatment-of-pelvic-sarcomas
#7
Stephan E Puchner, Philipp T Funovics, Christoph Böhler, Alexandra Kaider, Christoph Stihsen, Gerhard M Hobusch, Joannis Panotopoulos, Reinhard Windhager
BACKGROUND AND OBJECTIVES: Treatment of pelvic tumors remains challenging due to complex anatomy, poor oncological outcome and high complication rates. We sought to investigate the long-term oncological and surgical outcome of these patients. METHODS: Between 1980 and 2012, 147 patients underwent surgical treatment for pelvic sarcoma. Histological diagnosis was Chondrosarcoma in 54, Ewing's Sarcoma/PNET in 37, Osterosarcoma in 32 and others in 24 patients. Statistical analysis for the evaluation of oncological and surgical outcome was performed by applying Cox proportional hazards regression and Fine-Gray regression models for competing risk (CR) endpoints...
2017: PloS One
https://www.readbyqxmd.com/read/28197367/targeting-ewing-sarcoma-with-activated-and-gd2-specific-chimeric-antigen-receptor-engineered-human-nk-cells-induces-upregulation-of-immune-inhibitory-hla-g
#8
Sareetha Kailayangiri, Bianca Altvater, Christian Spurny, Silke Jamitzky, Sonja Schelhaas, Andreas H Jacobs, Constanze Wiek, Katharina Roellecke, Helmut Hanenberg, Wolfgang Hartmann, Heinz Wiendl, Susann Pankratz, Jutta Meltzer, Nicole Farwick, Lea Greune, Maike Fluegge, Claudia Rossig
Activated and in vitro expanded natural killer (NK) cells have substantial cytotoxicity against many tumor cells, but their in vivo efficacy to eliminate solid cancers is limited. Here, we used chimeric antigen receptors (CARs) to enhance the activity of NK cells against Ewing sarcomas (EwS) in a tumor antigen-specific manner. Expression of CARs directed against the ganglioside antigen GD2 in activated NK cells increased their responses to GD2+ allogeneic EwS cells in vitro and overcame resistance of individual cell lines to NK cell lysis...
2017: Oncoimmunology
https://www.readbyqxmd.com/read/28193773/dna-methylation-analysis-shows-epigenetic-heterogeneity-in-ewing-sarcoma
#9
(no author information available yet)
Ewing sarcoma is epigenetically heterogeneous despite the lack of genomic heterogeneity.
February 13, 2017: Cancer Discovery
https://www.readbyqxmd.com/read/28191614/undifferentiated-round-cell-ewing-like-sarcoma-not-always-so-round-nor-ewing-like
#10
EDITORIAL
Wei-Lien Wang, Alexander J Lazar
No abstract text is available yet for this article.
February 13, 2017: Virchows Archiv: An International Journal of Pathology
https://www.readbyqxmd.com/read/28188619/generation-of-conditional-oncogenic-chromosomal-translocations-using-crispr-cas9-genomic-editing-and-homology-directed-repair
#11
Lee Spraggon, Luciano G Martelotto, Julija Hmeljak, Tyler D Hitchman, Jiang Wang, Lu Wang, Emily K Slotkin, Pang-Dian Fan, Jorge S Reis-Filho, Marc Ladanyi
Chromosomal rearrangements encoding oncogenic fusion proteins are found in a wide variety of malignancies. The use of programmable nucleases to generate specific double-strand breaks in endogenous loci, followed by non-homologous end joining DNA repair, has allowed several of these translocations to be generated as constitutively expressed fusion genes within a cell population. Here, we describe a novel approach that combines CRISPR-Cas9 technology with homology-directed repair to engineer, capture and modulate the expression of chromosomal translocation products in a human cell line...
February 11, 2017: Journal of Pathology
https://www.readbyqxmd.com/read/28188186/nccn-guidelines-insights-bone-cancer-version-2-2017
#12
J Sybil Biermann, Warren Chow, Damon R Reed, David Lucas, Douglas R Adkins, Mark Agulnik, Robert S Benjamin, Brian Brigman, G Thomas Budd, William T Curry, Aarati Didwania, Nicola Fabbri, Francis J Hornicek, Joseph B Kuechle, Dieter Lindskog, Joel Mayerson, Sean V McGarry, Lynn Million, Carol D Morris, Sujana Movva, Richard J O'Donnell, R Lor Randall, Peter Rose, Victor M Santana, Robert L Satcher, Herbert Schwartz, Herrick J Siegel, Katherine Thornton, Victor Villalobos, Mary Anne Bergman, Jillian L Scavone
The NCCN Guidelines for Bone Cancer provide interdisciplinary recommendations for treating chordoma, chondrosarcoma, giant cell tumor of bone, Ewing sarcoma, and osteosarcoma. These NCCN Guidelines Insights summarize the NCCN Bone Cancer Panel's guideline recommendations for treating Ewing sarcoma. The data underlying these treatment recommendations are also discussed.
February 2017: Journal of the National Comprehensive Cancer Network: JNCCN
https://www.readbyqxmd.com/read/28186860/traumatic-rib-injury-patterns-imaging-pitfalls-complications-and-treatment
#13
Brett S Talbot, Christopher P Gange, Apeksha Chaturvedi, Nina Klionsky, Susan K Hobbs, Abhishek Chaturvedi
The ribs are frequently affected by blunt or penetrating injury to the thorax. In the emergency department setting, it is vital for the interpreting radiologist to not only identify the presence of rib injuries but also alert the clinician about organ-specific injury, specific traumatic patterns, and acute rib trauma complications that require emergent attention. Rib injuries can be separated into specific morphologic fracture patterns that include stress, buckle, nondisplaced, displaced, segmental, and pathologic fractures...
February 10, 2017: Radiographics: a Review Publication of the Radiological Society of North America, Inc
https://www.readbyqxmd.com/read/28182081/cytopathologic-features-of-an-unusual-case-of-multiple-eccrine-spiradenomas-misdiagnosed-as-a-malignant-round-cell-tumor
#14
Bharat Rekhi, Archi Agarwal
A 28-year-old lady presented with multiple swellings in her left shoulder, associated with intermittent pain since last one and a half years. Radiologic imaging revealed multiple, well-defined, subcutaneous lesions in her left supraclavicular region. Fine needle aspiration cytology (FNAC) smears were initially reported as Ewing sarcoma, elsewhere. On review, the smears showed cohesive clusters of round-to-oval cells with scant cytoplasm, which were focally arranged in an acinar-rosetting pattern around hyaline "droplets/bodies," along with few scattered lymphocytes against a background of red blood cells...
January 2017: Journal of Cytology
https://www.readbyqxmd.com/read/28176976/the-importance-of-surgery-as-part-of-multimodal-therapy-in-rapid-progressive-primary-extraosseous-ewing-sarcoma-of-the-cervical-intra-and-epidural-space
#15
Richard Bostelmann, Mario Leimert, Hans Jakob Steiger, Kristin Gierga, Athanasios K Petridis
Primary extraosseous Ewing sarcomas (EESs) are an extremely rare pathological entity. Less than 32 cases have been reported in the literature. Here we report an uncommon case with very rapid progression in the cervical region with extra- and intradural involvement. We present a thorough review of the literature and discuss possible treatment modalities. The Medline database was searched using the search terms: Ewing sarcoma, extraosseus tumour, treatment, management, cervical spine. A previously healthy 29-year-old man complained of right-sided radiculopathy (C7)...
October 24, 2016: Clinics and Practice
https://www.readbyqxmd.com/read/28173669/-clinicopathologic-analysis-of-ewing-like-bcor-ccnb3-undifferentiated-sarcoma
#16
X J Wang, D H Zhao, Y M Wang, Y X Liu, X Li, M Y Li, W C Li, Z Wang
Objective: To investigate the morphologic, immunohistochemical, genetic, clinical features and prognosis of Ewing-like BCOR-CCNB3 gene fusion undifferentiated sarcoma (BCOR-CCNB3 fusion sarcoma). Method: Seventeen Ewing-like sarcoma cases were screened for CCNB3 expression and BCOR-CCNB3 fusion transcripts by immunohistochemistry and RT-PCR among 260 cases of Ewing-like sarcomas collected during Jan, 2006 to Dec, 2015. Three cases of BCOR-CCNB3 fusion sarcoma were found among 17 atypical Ewing sarcomas, and follow-up were conducted...
February 8, 2017: Zhonghua Bing Li Xue za Zhi Chinese Journal of Pathology
https://www.readbyqxmd.com/read/28163522/ewing-s-sarcoma-of-the-cranial-vault
#17
Geetha Narayanan, K P Sreelesh, Thara Somanathan, Lali V Soman
Primary Ewing's sarcoma (EWS) arising from cranial bones is rare and accounts for only 1%-4% of all EWS. We report the case of a 15-year-old girl with EWS of the frontoparietal region of the skull. She underwent excision following which she received combination chemotherapy with vincristine, doxorubicin, cyclophosphamide alternating with ifosfamide, and VP16 and local radiation of 45 Gy. She is alive in complete remission at 40 months.
December 2016: Journal of Neurosciences in Rural Practice
https://www.readbyqxmd.com/read/28160567/ews-fli1-confers-exquisite-sensitivity-to-nampt-inhibition-in-ewing-sarcoma-cells
#18
Cornelia N Mutz, Raphaela Schwentner, Dave N T Aryee, Eric D J Bouchard, Edgard M Mejia, Grant M Hatch, Maximilian O Kauer, Anna M Katschnig, Jozef Ban, Antje Garten, Javier Alonso, Versha Banerji, Heinrich Kovar
Ewing sarcoma (EwS) is the second most common bone cancer in children and adolescents with a high metastatic potential. EwS development is driven by a specific chromosomal translocation resulting in the generation of a chimeric EWS-ETS transcription factor, most frequently EWS-FLI1.Nicotinamide adenine dinucleotide (NAD) is a key metabolite of energy metabolism involved in cellular redox reactions, DNA repair, and in the maintenance of genomic stability. This study describes targeting nicotinamide phosphoribosyltransferase (NAMPT), the rate-limiting enzyme of NAD synthesis, by FK866 in EwS cells...
February 1, 2017: Oncotarget
https://www.readbyqxmd.com/read/28148901/c-ebp%C3%AE-1-promotes-transformation-and-chemoresistance-in-ewing-sarcoma-cells
#19
Jamie D Gardiner, Lisa M Abegglen, Xiaomeng Huang, Bryce E Carter, Elizabeth A Schackmann, Marcus Stucki, Christian N Paxton, R Lor Randall, James F Amatruda, Angelica R Putnam, Heinrich Kovar, Stephen L Lessnick, Joshua D Schiffman
CEBPB copy number gain in Ewing sarcoma was previously shown to be associated with worse clinical outcome compared to tumors with normal CEBPB copy number, although the mechanism was not characterized. We employed gene knockdown and rescue assays to explore the consequences of altered CEBPB gene expression in Ewing sarcoma cell lines. Knockdown of EWS-FLI1 expression led to a decrease in expression of all three C/EBPβ isoforms while re-expression of EWS-FLI1 rescued C/EBPβ expression. Overexpression of C/EBPβ-1, the largest of the three C/EBPβ isoforms, led to a significant increase in colony formation when cells were grown in soft agar compared to empty vector transduced cells...
January 27, 2017: Oncotarget
https://www.readbyqxmd.com/read/28141799/the-spectrum-of-ewsr1-rearranged-neoplasms-at-a-tertiary-sarcoma-centre-assessing-772-tumour-specimens-and-the-value-of-current-ancillary-molecular-diagnostic-modalities
#20
Jonathan Noujaim, Robin L Jones, John Swansbury, David Gonzalez, Charlotte Benson, Ian Judson, Cyril Fisher, Khin Thway
BACKGROUND: EWSR1 rearrangements were first identified in Ewing sarcoma, but the spectrum of EWSR1-rearranged neoplasms now includes many soft tissue tumour subtypes including desmoplastic small round cell tumour (DSRCT), myxoid liposarcoma (MLPS), extraskeletal myxoid chondrosarcoma (EMC), angiomatoid fibrous histiocytoma (AFH), clear cell sarcoma (CCS) and myoepithelial neoplasms. We analysed the spectrum of EWSR1-rearranged soft tissue neoplasms at our tertiary sarcoma centre, by assessing ancillary molecular diagnostic modalities identifying EWSR1-rearranged tumours and reviewing the results in light of our current knowledge of these and other Ewing sarcoma-like neoplasms...
January 31, 2017: British Journal of Cancer
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