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Ewing sarcoma

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https://www.readbyqxmd.com/read/29661713/review-with-novel-markers-facilitates-precise-categorization-of-41-cases-of-diagnostically-challenging-undifferentiated-small-round-cell-tumors-a-clinicopathologic-immunophenotypic-and-molecular-analysis
#1
Isidro Machado, Akihiko Yoshida, María Gema Nieto Morales, Lucas Faria Abrahão-Machado, Samuel Navarro, Julia Cruz, Javier Lavernia, Antonina Parafioriti, Piero Picci, Antonio Llombart-Bosch
BACKGROUND: Despite extensive immunohistochemical (IHC) and molecular studies combined with morphologic findings, a group of round/ovoid cell tumors histologically similar to Ewing sarcomas (ES) but lacking EWSR1-rearrangements may remain unclassifiable. DESIGN: We retrospectively analyzed 41 Ewing-like tumors (formalin-fixed, paraffin-embedded) previously determined as negative or non-informative for EWSR1-rearrangements by FISH and/or RT-PCR. A new histopathology revision and additional IHC and molecular analyses were carried out in order to investigate whether additional IHC and/or molecular testing in combination with the morphological findings may help in reaching a definitive diagnosis...
November 29, 2017: Annals of Diagnostic Pathology
https://www.readbyqxmd.com/read/29656296/impact-of-pathological-fractures-on-the-prognosis-of-primary-malignant-bone-sarcoma-in-children-and-adults-a-single-center-retrospective-study-of-205-patients
#2
Miriam Schlegel, Martina Zeumer, Peter Michael Prodinger, Klaus Woertler, Marc Steinborn, Rüdiger von Eisenhart-Rothe, Stefan Burdach, Hans Rechl, Irene von Luettichau
BACKGROUND: The purpose of this study was to investigate whether pathological fractures (PF) influence the prognosis of patients with osteosarcoma (OS) or Ewing tumor (ET) regarding 5-year survival, occurrence of metastases, and local recurrence. METHODS: We retrospectively analyzed 205 patients with metastatic and nonmetastatic OS or ET. Survival analysis was performed for all patients and differentiated for patients with OS (n = 127) and ET (n = 78) as well as for adults (n = 101) and children (n = 104)...
April 13, 2018: Oncology
https://www.readbyqxmd.com/read/29652875/evaluation-of-recurrence-of-musculoskeletal-tumors-with-thallium-201-scintigraphy-plus-spect-ct-in-pediatric-population
#3
Sevastián S Medina-Ornelas, Herlinda Vera-Hermosillo, Rafael Delgado-Espín, Francisco O García-Pérez
Background: Imaging studies, particularly simple and contrast-enhanced tomography, constitute the first diagnostic approach to detect recurrence of musculoskeletal tumors. The aim of the present retrospective study was to demonstrate the usefulness of scintigraphy plus SPECT/CT (single photon emission computed tomography) with thallium-201 (201 Tl) in the evaluation of malignant musculoskeletal tumors with suspicion of recurrence or metastatic disease. Methods: Eight weeks after the last therapy, 72 scintigraphy and SPECT/CT studies were performed to assess regional recurrence and metastatic disease in 42 patients with different types of malignant musculoskeletal tumors, such as osteosarcoma, Ewing's sarcoma, rhabdomyosarcoma, retinoblastoma, synovial sarcoma, and Wilms tumor at the Hospital Infantil de México Federico Gómez...
2018: Boletín Médico del Hospital Infantil de México
https://www.readbyqxmd.com/read/29649003/somatic-mutation-of-the-cohesin-complex-subunit-confers-therapeutic-vulnerabilities-in-cancer
#4
Yunhua Liu, Hanchen Xu, Kevin Van der Jeught, Yujing Li, Sheng Liu, Lu Zhang, Yuanzhang Fang, Xinna Zhang, Milan Rodovich, Bryan P Schneider, Xiaoming He, Cheng Huang, Chi Zhang, Jun Wan, Guang Ji, Xiongbin Lu
Synthetic lethality-based strategy has been developed to identify therapeutic targets in cancer harboring tumor suppressor gene mutations, as exemplified by the effectiveness of PARP inhibitors in BRCA1/2-mutated tumors. However, many synthetic lethal interactors are less reliable due to the fact that such genes usually do not perform fundamental or indispensable functions in the cell. Here we developed an approach to identify the "essential lethality" arose from these mutated/deleted essential genes, which are largely tolerated in cancer cells due to genetic redundancy...
April 12, 2018: Journal of Clinical Investigation
https://www.readbyqxmd.com/read/29629581/vascularized-fibular-epiphyseal-transfer-for-proximal-humeral-reconstruction-in-children-with-a-primary-sarcoma-of-bone
#5
J D Stevenson, R Doxey, A Abudu, M Parry, S Evans, F Peart, L Jeys
Aims Preserving growth following limb-salvage surgery of the upper limb in children remains a challenge. Vascularized autografts may provide rapid biological incorporation with the potential for growth and longevity. In this study, we aimed to describe the outcomes following proximal humeral reconstruction with a vascularized fibular epiphyseal transfer in children with a primary sarcoma of bone. We also aimed to quantify the hypertrophy of the graft and the annual growth, and to determine the functional outcomes of the neoglenofibular joint...
April 1, 2018: Bone & Joint Journal
https://www.readbyqxmd.com/read/29626752/efficacy-of-onc201-in-desmoplastic-small-round-cell-tumor
#6
Andrea A Hayes-Jordan, Xiao Ma, Brian A Menegaz, Salah-Eddine Lamhamedi-Cherradi, Charles V Kingsley, Jalen A Benson, Pamela E Camacho, Joseph A Ludwig, Cynthia R Lockworth, Gloria E Garcia, Suzanne L Craig
Desmoplastic Small Round Cell Tumor (DSRCT) is a rare sarcoma tumor of adolescence and young adulthood, which harbors a recurrent chromosomal translocation between the Ewing's sarcoma gene (EWSR1) and the Wilms' tumor suppressor gene (WT1). Patients usually develop multiple abdominal tumors with liver and lymph node metastasis developing later. Survival is poor using a multimodal therapy that includes chemotherapy, radiation and surgical resection, new therapies are needed for better management of DSRCT. Triggering cell apoptosis is the scientific rationale of many cancer therapies...
April 4, 2018: Neoplasia: An International Journal for Oncology Research
https://www.readbyqxmd.com/read/29626598/ewsr1-nfatc2-gene-fusion-in-a-soft-tissue-tumor-with-epithelioid-round-cell-morphology-and-abundant-stroma-a-case-report-and-review-of-the-literature
#7
Jarish N Cohen, Amit J Sabnis, Gregor Krings, Soo-Jin Cho, Andrew E Horvai, Jessica L Davis
Mesenchymal round cell tumors are a diverse group of neoplasms defined by primitive, often high-grade cytomorphology. The most common molecular alterations detected in these tumors are gene rearrangements involving EWSR1 to one of many fusion partners. Rare EWSR1-NFATC2 gene rearrangements, corresponding to a t(20;22) gene translocation, have been described in mesenchymal tumors with clear round cell morphology and a predilection for the skeleton. We present a case of a tumor harboring the EWSR1-NFATC2 gene fusion arising in the subcutaneous tissue of a young woman...
April 4, 2018: Human Pathology
https://www.readbyqxmd.com/read/29625604/apatinib-for-advanced-sarcoma-results-from-multiple-institutions-off-label-use-in-china
#8
Lu Xie, Wei Guo, Ye Wang, Taiqiang Yan, Tao Ji, Jie Xu
BACKGROUND: Anti-angiogenesis Tyrosine kinase inhibitors (TKIs) have been proved to show promising effects on prolonging progression-free survival (PFS) for advanced sarcoma after failure of standard multimodal Therapy. Methylsulfonic apatinib is one of those TKIs which specifically inhibits VEGFR-2. This paper summarizes the experience of three Peking University affiliated hospitals in off-label use of apatinib in the treatment of extensively pre-treated sarcoma. METHODS: We retrospectively analysed files of patients with advanced sarcoma not amenable to curative treatment, who were receiving an apatinib-containing regimen between June 1, 2015 and December 1, 2016...
April 6, 2018: BMC Cancer
https://www.readbyqxmd.com/read/29620679/diagnostic-accuracy-of-18f-fdg-pet-ct-in-the-staging-and-assessment-of-response-to-chemotherapy-in-children-with-ewing-sarcoma
#9
Antonio Ruggiero, Valerio Lanni, Alberto Librizzi, Palma Maurizi, Giorgio Attinà, Stefano Mastrangelo, Alessandro Giordano, Riccardo Riccardi
INTRODUCTION: The purpose of this study was to evaluate the potential role of fluorine-18 fluorodeoxyglucose (18F-FDG) positron-emission tomography/computed tomography (PET-CT) in the staging and assessment of chemotherapy response in Ewing sarcoma. MATERIALS AND METHODS: For 13 patients with Ewing sarcoma, whole-body FDG PET-CT was assessed for site of primary disease, disease extent, and response to therapy. Chest CT, localized magnetic resonance imaging or CT of primary site, and bone scintigrams were evaluated for imaging features of the primary lesion and presence or absence of metastatic disease...
April 3, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29616903/sinonasal-ewing-sarcoma-a-case-report-and-literature-review
#10
Jonathan K Lin, Jonathan Liang
INTRODUCTION: Ewing sarcoma (ES) of the sinonasal tract and associated primitive neuroectodermal tumors are rare. To our knowledge, only 10 case reports of sinonasal ES of the nose or paranasal sinuses have been reported. Furthermore, there has been only 1 case of sinonasal ES arising from the middle turbinate. Recommended management of sinonasal ES stems from the management of its osseous counterpart, ES, but treatment with surgery, radiotherapy, and chemotherapy is varied. Five-year survival rates vary from 21% to 70%, with the lower rates representing patients presenting with metastatic disease...
March 7, 2018: Permanente Journal
https://www.readbyqxmd.com/read/29611053/extra-osseous-ewing-sarcoma-of-the-pancreas-case-report-with-radiologic-pathologic-and-molecular-correlation-and-brief-review-of-the-literature
#11
Miglena K Komforti, Evgeniya Sokolovskaya, Catherine A D'Agostino, Ryma Benayed, Rebecca M Thomas
In 2002, due to extensive histomorphologic, immunohistochemical, and cytogenetic similarities, the World Health Organization unified undifferentiated small round blue cell neoplasms of soft tissue and bone (previously segregated as Ewing sarcoma or Primitive Neuroectodermal tumor) into one category: Ewing family of tumors (EFT). Osseous EFT are more common, and while extra-osseous EFT can occur anywhere in the body, those of the pancreas are rare and likely to be seen in the second decade of life in the head of the pancreas...
April 2, 2018: Virchows Archiv: An International Journal of Pathology
https://www.readbyqxmd.com/read/29610692/successful-complete-response-of-tumor-thrombus-after-combined-with-chemotherapy-and-irradiation-for-ewing-sarcoma
#12
Yusuke Minami, Seiichi Matsumoto, Keisuke Ae, Taisuke Tanizawa, Keiko Hayakawa, Yuki Funauchi, Sakae Okumura, Yutaka Takazawa
Pelvic Ewing sarcoma is associated with a worse prognosis. Thromboembolic events are relatively common in pediatric patients with cancers including sarcomas. We have presented a case of Ewing sarcoma arising from the left iliac bone with tumor thrombus of inferior vena cava (IVC) which was obtained complete response by both chemotherapy and irradiation. Magnetic resonance imaging (MRI) scan demonstrated that the tumor arising from the left iliac bone extended into the left side of sacral bone, suggesting the difficulty of surgical resection...
2018: Case Reports in Orthopedics
https://www.readbyqxmd.com/read/29610659/survival-is-influenced-by-approaches-to-local-treatment-of-ewing-sarcoma-within-an-international-randomised-controlled-trial-analysis-of-eicess-92
#13
Jeremy Whelan, Allan Hackshaw, Anne McTiernan, Robert Grimer, David Spooner, Jessica Bate, Andreas Ranft, Michael Paulussen, Herbert Juergens, Alan Craft, Ian Lewis
Background: Two national clinical trial groups, United Kingdom Children's Cancer and Leukaemia Group (CCLG) and the German Paediatric Oncology and Haematology Group (GPOH) together undertook a randomised trial, EICESS-92, which addressed chemotherapy options for Ewing's sarcoma. We sought the causes of unexpected survival differences between the study groups. Methods: 647 patients were randomised. Cox regression analyses were used to compare event-free survival (EFS) and overall survival (OS) between the two study groups...
2018: Clinical Sarcoma Research
https://www.readbyqxmd.com/read/29582409/epha2-receptor-is-a-key-player-in-the-metastatic-onset-of-ewing-sarcoma
#14
Silvia Garcia-Monclús, Roser López-Alemany, Olga Almacellas-Rabaiget, David Herrero-Martín, Juan Huertas-Martinez, Laura Lagares-Te, Piedad Alba-Pavón, Lourdes Hontecillas-Prieto, Jaume Mora, Enrique de Álava, Santi Rello-Varona, Paloma H Giangrande, Oscar M Tirado
Ewing sarcoma (ES) is the second most common bone malignancy affecting children and young adults with poor prognosis due to high metastasis incidence. Our group previously described that EphA2, a tyrosine kinase receptor, promotes angiogenesis in Ewing sarcoma (ES) cells via ligand-dependent signaling. Now we wanted to explore EphA2 ligand-independent activity, controlled upon phosphorylation at S897 (p-EphA2S897 ), as it has been linked to metastasis in several malignancies. By reverse genetic engineering we explored the phenotypic changes after EphA2 removal or reintroduction...
March 26, 2018: International Journal of Cancer. Journal International du Cancer
https://www.readbyqxmd.com/read/29582320/soft-tissue-sarcomas-new-opportunity-of-treatment-with-parp-inhibitors
#15
Monica Mangoni, Mariangela Sottili, Giulia Salvatore, Domenico Campanacci, Guido Scoccianti, Giovanni Beltrami, Camilla Delli Paoli, Luca Dominici, Virginia Maragna, Emanuela Olmetto, Icro Meattini, Isacco Desideri, Pierluigi Bonomo, Daniela Greto, Lorenzo Livi
BACKGROUND: Poly(ADP-ribose) polymerases (PARP) are a large family of enzymes involved in several cellular processes, including DNA single-strand break repair via the base-excision repair pathway. PARP inhibitors exert antitumor activity by both catalytic PARP inhibition and PARP-DNA trapping, moreover PARP inhibition represents a potential synthetic lethal approach against cancers with specific DNA-repair defects. Soft tissue sarcoma (STSs) are a heterogeneous group of mesenchymal tumors with locally destructive growth, high risk of recurrence and distant metastasis...
March 26, 2018: La Radiologia Medica
https://www.readbyqxmd.com/read/29581854/ire1%C3%AE-xbp1-inhibitors-exerted-anti-tumor-activities-in-ewing-s-sarcoma
#16
Yu Tanabe, Yoshiyuki Suehara, Shinji Kohsaka, Takuo Hayashi, Keisuke Akaike, Kenta Mukaihara, Taisei Kurihara, Youngji Kim, Taketo Okubo, Midori Ishii, Saiko Kazuno, Kazuo Kaneko, Tsuyoshi Saito
Ewing's sarcoma (ES) is the second-most frequent pediatric bone tumor. Chromosomal translocation t(11;22)(q24:q12) results in the formation of EWS/FLI1 gene fusion, which is detected in approximately 90% of tumors of the Ewing family. Several transcriptome studies have provided lists of genes associated with EWS/FLI1 expression. However, the protein expression profiles associated with EWS/FLI1 have yet to be elucidated. In this study, to identify the regulated proteins associated with EWS/FLI1 and therapeutic targets in ES, we conducted proteomic studies using EWS/FLI1 knockdown in four Ewing's sarcoma cell lines and human mesenchymal stem cells (hMSCs) expressing EWS/FLI1...
March 6, 2018: Oncotarget
https://www.readbyqxmd.com/read/29580035/fluorescence-in-situ-hybridization-fish-for-differential-diagnosis-of-soft-tissue-sarcomas
#17
Amna Asif, Sajid Mushtaq, Usman Hassan, Noreen Akhtar, Mudassar Hussain, Muhammad Azam, Romena Qazi
Introduction:Soft tissue sarcomas are rare tumors comprising 1 percent of solid malignancies. The latest edition of WHO soft tissue pathology lists 94 benign and malignant soft tissue tumors. Many of these show a large degree of morphological overlap. Immunohistochemistry has been shown to be reliable in many cases for differential diagnosis of lesions, although cytogenetic tests are considered the gold standard for many entities.Fluorescence in-situ hybridization (FISH) is a cytogenetic technique that uses fluorescent probes that bind to only those parts of the chromosome which have a high degree of sequence complementarity...
March 27, 2018: Asian Pacific Journal of Cancer Prevention: APJCP
https://www.readbyqxmd.com/read/29572501/array-based-dna-methylation-profiling-in-sarcomas-with-small-blue-round-cell-histology-provides-valuable-diagnostic-information
#18
Christian Koelsche, Wolfgang Hartmann, Daniel Schrimpf, Damian Stichel, Susanne Jabar, Andreas Ranft, David E Reuss, Felix Sahm, David T W Jones, Melanie Bewerunge-Hudler, Marcel Trautmann, Thomas Klingebiel, Christian Vokuhl, Manfred Gessler, Eva Wardelmann, Iver Petersen, Daniel Baumhoer, Uta Flucke, Cristina Antonescu, Manel Esteller, Stefan Fröhling, Marcel Kool, Stefan M Pfister, Gunhild Mechtersheimer, Uta Dirksen, Andreas von Deimling
Undifferentiated solid tumors with small blue round cell histology and expression of CD99 mostly resemble Ewing sarcoma. However, they also may include other tumors such as mesenchymal chondrosarcoma, synovial sarcoma, or small cell osteosarcoma. Definitive classification usually requires detection of entity-specific mutations. While this approach identifies the majority of Ewing sarcomas, a subset of lesions remains unclassified and, therefore, has been termed "Ewing-like sarcomas" or small blue round cell tumors not otherwise specified...
March 23, 2018: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
https://www.readbyqxmd.com/read/29570692/preclinical-evaluation-of-vemurafenib-as-therapy-for-braf-v600e-mutated-sarcomas
#19
Sarina Gouravan, Leonardo A Meza-Zepeda, Ola Myklebost, Eva W Stratford, Else Munthe
The BRAFV600E mutation, which in melanoma is targetable with vemurafenib, is also found in sarcomas and we here evaluate the therapeutic potential in sarcoma cell lines. METHODS: Four sarcoma cell lines harboring the BRAFV600E mutation, representing liposarcomas (SA-4 and SW872), Ewing sarcoma (A673) and atypical synovial sarcoma (SW982), were treated with vemurafenib and the effects on cell growth, apoptosis, cell cycle progression and cell signaling were determined. RESULTS: Vemurafenib induced a strong cytostatic effect in SA-4 cells, mainly due to cell cycle arrest, whereas only moderate levels of apoptosis were observed...
March 23, 2018: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/29563856/microrna-181c-prevents-apoptosis-by-targeting-of-fas-receptor-in-ewing-s-sarcoma-cells
#20
Masanori Kawano, Kazuhiro Tanaka, Ichiro Itonaga, Tatsuya Iwasaki, Hiroshi Tsumura
Background: MicroRNAs (miRNAs) are endogenous, small non-coding RNAs that play important roles in multiple biological processes. Here, we show that miRNAs play an important function in the down-regulation of FAS expression in Ewing's sarcoma (ES) cells. Methods: To identify and characterize possible oncogenic factors in ES, we employed a microarray-based approach to profile the changes in the expression of miRNAs and their target mRNAs in five ES cell lines and human mesenchymal stem cells (hMSCs)...
2018: Cancer Cell International
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