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JOURNAL ARTICLE
Vernon Justice Ebegboni, Tamara L Jones, Tayvia Brownmiller, Patrick X Zhao, Erica C Pehrsson, Soumya Sundara Rajan, Natasha J Caplen
The mechanistic basis for the metastasis of Ewing sarcomas remains poorly understood, as these tumors harbor few mutations beyond the chromosomal translocation that initiates the disease. Instead, the epigenome of Ewing sarcoma (EWS) cells reflects the regulatory state of genes associated with the DNA binding activity of the fusion oncoproteins EWSR1::FLI1 or EWSR1::ERG. In this study, we examined the EWSR1::FLI1/ERG's repression of transcription factor genes, concentrating on those that exhibit a broader range of expression in tumors than in EWS cell lines...
April 8, 2024: Molecular Cancer Research: MCR
#2
Ahmed D Khudair, Aiman D Khudair, Thuraiya Al-Rawahia, Rachel A Marshall, Khalifa Albenjasim, Mahera Roohi, Ziad Al Naib
Primary Ewing sarcoma of the kidney (ESK) is a rare and aggressive entity, with a poor prognosis. It often presents as metastatic disease with the lungs being the most common site. In adults, the occurrence of these tumors is uncommon, with patients exhibiting non-specific symptoms such as weight loss, flank pain, hematuria, and an abdominal mass. The combination of these vague clinical symptoms and the rarity of these tumors often results in a delayed diagnosis, leading to poorer outcomes for these patients...
January 2024: Curēus
#3
JOURNAL ARTICLE
Lea Dewi Schlieben, Maria Giulia Carta, Evgeny A Moskalev, Robert Stöhr, Markus Metzler, Manuel Besendörfer, Norbert Meidenbauer, Sabine Semrau, Rolf Janka, Robert Grützmann, Stefan Wiemann, Arndt Hartmann, Abbas Agaimy, Florian Haller, Fulvia Ferrazzi
Small blue round cell sarcomas (SBRCSs) are a heterogeneous group of tumors with overlapping morphologic features but markedly varying prognosis. They are characterized by distinct chromosomal alterations, particularly rearrangements leading to gene fusions, whose detection currently represents the most reliable diagnostic marker. Ewing sarcomas (ESs) are the most common SBRCSs, defined by gene fusions involving EWSR1 and transcription factors of the ETS gene family, while the most frequent non-EWSR1-rearranged SBRCSs harbor a CIC rearrangement...
February 21, 2024: Journal of Molecular Diagnostics: JMD
#4
Ariunaa Bayanjargal, Cenny Taslim, Iftekhar A Showpnil, Julia Selich-Anderson, Jesse C Crow, Stephen L Lessnick, Emily R Theisen
Ewing sarcoma is the second most common bone cancer in children and young adults. In 85% of patients, a translocation between chromosomes 11 and 22 results in a potent fusion oncoprotein, EWS::FLI. EWS::FLI is the only genetic alteration in an otherwise unaltered genome of Ewing sarcoma tumors. The EWS portion of the protein is an intrinsically disordered domain involved in transcriptional regulation by EWS::FLI. The FLI portion of the fusion contains a DNA binding domain shown to bind core GGAA motifs and GGAA repeats...
January 31, 2024: bioRxiv
#5
JOURNAL ARTICLE
Josephine K Dermawan, Emily Slotkin, William D Tap, Paul Meyers, Leonard Wexler, John Healey, Fabio Vanoli, Chad M Vanderbilt, Cristina R Antonescu
Chromoplexy is a phenomenon defined by large-scale chromosomal chained rearrangements. A previous study observed chromoplectic events in a subset of Ewing sarcomas (ES), which was linked to an increased relapse rate. Chromoplexy analysis could potentially facilitate patient risk stratification, particularly if it could be detected with clinically applied targeted next-generation sequencing (NGS) panels. Using DELLY, a structural variant (SV) calling algorithm that is part of the MSK-IMPACT pipeline, we characterized the spectrum of SVs in EWSR1-fused round cell sarcomas, including 173 ES and 104 desmoplastic small round cell tumors (DSRCT), to detect chromoplexy and evaluate its association with clinical and genomic features...
February 9, 2024: Cancer Research
#6
Vernon Justice Ebegboni, Tamara L Jones, Tayvia Brownmiller, Patrick X Zhao, Erica C Pehrsson, Soumya Sundara Rajan, Natasha J Caplen
The mechanistic basis for the metastasis of Ewing sarcomas remains poorly understood, as these tumors harbor few mutations beyond the chromosomal translocation that initiates the disease. Instead, the epigenome of Ewing sarcoma (EWS) cells reflects the regulatory state of genes associated with the DNA binding activity of the fusion oncoproteins EWSR1::FLI1 or EWSR1::ERG. In this study, we examined the EWSR1::FLI1/ERG's repression of transcription factor genes, concentrating on those that exhibit a broader range of expression in tumors than in EWS cell lines...
December 23, 2023: bioRxiv
#7
Courtney N Johnson, Kandarp A Sojitra, Erich J Sohn, Alma K Moreno-Romero, Antoine Baudin, Xiaoping Xu, Jeetain Mittal, David S Libich
The FET family proteins, which includes FUS, EWS, and TAF15, are RNA chaperones instrumental in processes such as mRNA maturation, transcriptional regulation, and the DNA damage response. These proteins have clinical significance: chromosomal rearrangements in FET proteins are implicated in Ewing family tumors and related sarcomas. Furthermore, point mutations in FUS and TAF15 are associated with neurodegenerative conditions like amyotrophic lateral sclerosis and frontotemporal lobar dementia. The fusion protein EWS::FLI1, the causative mutation of Ewing sarcoma, arises from a genomic translocation that fuses the low-complexity domain (LCD) of EWS (EWS LCD ) with the DNA binding domain of the ETS transcription factor FLI1...
November 1, 2023: bioRxiv
#8
JOURNAL ARTICLE
Adam Rock, An Uche, Janet Yoon, Mark Agulnik, Warren Chow, Sherri Millis
Ewing Sarcoma (ES) is an aggressive, mesenchymal malignancy associated with a poor prognosis in the recurrent or metastatic setting with an estimated overall survival (OS) of <30% at 5 years. ES is characterized by a balanced, reciprocal chromosomal translocation involving the EWSR1 RNA-binding protein and ETS transcription factor gene ( EWS-FLI being the most common). Interestingly, murine ES models have failed to produce tumors phenotypically representative of ES. Genomic alterations (GA) in ES are infrequent and may work synergistically with EWS-ETS translocations to promote oncogenesis...
October 15, 2023: Journal of Personalized Medicine
#9
REVIEW
Maryne Dupuy, François Lamoureux, Mathilde Mullard, Anaïs Postec, Laura Regnier, Marc Baud'huin, Steven Georges, Bénédicte Brounais-Le Royer, Benjamin Ory, Françoise Rédini, Franck Verrecchia
In Europe, with an incidence of 7.5 cases per million, Ewing sarcoma (ES) is the second most common primary malignant bone tumor in children, adolescents and young adults, after osteosarcoma. Since the 1980s, conventional treatment has been based on the use of neoadjuvant and adjuvant chemotherapeutic agents combined with surgical resection of the tumor when possible. These treatments have increased the patient survival rate to 70% for localized forms, which drops drastically to less than 30% when patients are resistant to chemotherapy or when pulmonary metastases are present at diagnosis...
2023: Frontiers in Cell and Developmental Biology
#10
JOURNAL ARTICLE
Ying S Zou, Laura Morsberger, Melanie Hardy, Jen Ghabrial, Victoria Stinnett, Jaclyn B Murry, Patty Long, Andrew Kim, Christine A Pratilas, Nicolas J Llosa, Brian H Ladle, Kathryn M Lemberg, Adam S Levin, Carol D Morris, Lisa Haley, Christopher D Gocke, John M Gross
Ewing sarcomas (ES) are rare small round cell sarcomas often affecting children and characterized by gene fusions involving one member of the FET family of genes (usually EWSR1) and a member of the ETS family of transcription factors (usually FLI1 or ERG ). The detection of EWSR1 rearrangements has important diagnostic value. Here, we conducted a retrospective review of 218 consecutive pediatric ES at diagnosis and found eight patients having data from chromosome analysis, FISH/microarray, and gene-fusion assay...
May 24, 2023: Genes
#11
REVIEW
Sergey Tsibulnikov, Daria Fayzullina, Irina Karlina, Brett A Schroeder, Olga Karpova, Peter Timashev, Ilya Ulasov
Ewing sarcoma (ES) is an aggressive malignant tumor, characterized by non-random chromosomal translocations that produce fusion genes. Fusion genes and fusion protein products are promising targets for gene therapy. Therapeutic approaches and strategies vary based on target molecules (nucleotides, proteins) of interest. We present an extensive literature review of active molecules for gene therapy and methods of gene therapy delivery, both of which are necessary for successful treatment.
April 10, 2023: Cancer Gene Therapy
#12
JOURNAL ARTICLE
Guangtao Han, Ting Liu, Pengde Kang
BACKGROUND: Ewing sarcoma has attracted more attention in recent years but has yet to be bibliometrically analyzed. Hence, this study investigated the trend of Ewing sarcoma over the past 30 years with bibliometric analysis. METHODS: Original publications related to Ewing sarcoma were obtained from the Science Citation Index Extension (SCI-E), Social Sciences Citation Index (SSCI), and Web of Science Core Collection (WoSCC) between 1993 and 2022. CiteSpace and VOSviewer were used to extract the countries/regions, institutions, authors, journals, references, and keywords involved in this topic to identify and analyze the research hotspots and trends in this field...
March 24, 2023: BMC Cancer
#13
JOURNAL ARTICLE
Kaya Atagi, Takashi Karashima, Keisuke Mizutani, Hideo Fukuhara, Satoshi Fukata, Yujiro Miura, Atsuyuki Mitsuishi, Kazuhiro Hanazaki, Sunao Uemura, Ryohei Miyazaki, Takashi Anayama, Mayuka Yamane, Mizu Sakai, Mitsuko Iguchi, Kenji Yorita, Keiji Inoue
BACKGROUND: Ewing's sarcoma is a malignant neoplasm that mainly occurs in skeletal tissue but can rarely arise in soft tissues. Recently, small round cell tumors (including Ewing's sarcoma) caused by chromosomal translocations have been collectively termed Ewing's sarcoma family of tumors. We report a rare case of primary adrenal Ewing's sarcoma family of tumors with tumor thrombus. CASE PRESENTATION: A 22-year-old Asian woman was referred to our hospital with a left retroperitoneal tumor 19 cm in diameter...
March 24, 2023: Journal of Medical Case Reports
#14
JOURNAL ARTICLE
Emily E Selig, Roohi Bhura, Matthew R White, Shivani Akula, Renee D Hoffman, Carmel N Tovar, Xiaoping Xu, Rachell E Booth, David S Libich
EWS is a member of the FET family of RNA/DNA binding proteins that regulate crucial phases of nucleic acid metabolism. EWS comprises an N-terminal low-complexity domain (LCD) and a C-terminal RNA-binding domain (RBD). The RBD is further divided into three RG-rich regions, which flank an RNA-recognition motif (RRM) and a zinc finger (ZnF) domain. Recently, EWS was shown to regulate R-loops in Ewing sarcoma, a pediatric bone and soft-tissue cancer in which a chromosomal translocation fuses the N-terminal LCD of EWS to the C-terminal DNA binding domain of the transcription factor FLI1...
March 17, 2023: Biopolymers
#15
Debajyoti Datta, Arunkumar Sekar, V G Parameshwar, Suvendu Purkait, Sumit Bansal
Ewing's sarcoma/peripheral primitive neuroectodermal tumor (ES/pPNET) belongs to the family of malignant small and blue round cell tumors. It usually occurs in children and young adults with 3/4th of the cases arising from bone and 1/4 from soft tissue. Here, we present two cases of intracranial ES/pPNET who presented with mass effect. Management consists of surgical excision followed by adjuvant chemotherapy. Intracranial ES/pPNETs are highly aggressive and rare malignancies, reported to comprise of 0.03% of all intracranial tumors...
2023: Journal of Neurosciences in Rural Practice
#16
JOURNAL ARTICLE
Sho Isoyama, Naomi Tamaki, Yutaka Noguchi, Mutsumi Okamura, Yuki Yoshimatsu, Tadashi Kondo, Takeshi Suzuki, Shin-Ichi Yaguchi, Shingo Dan
Translocation-related sarcomas (TRSs) harbor an oncogenic fusion gene generated by chromosome translocation and account for approximately one-third of all sarcomas; however, effective targeted therapies have yet to be established. We previously reported that a pan-phosphatidylinositol 3-kinase (PI3K) inhibitor, ZSTK474, was effective for the treatment of sarcomas in a phase I clinical trial. We also demonstrated the efficacy of ZSTK474 in a preclinical model, particularly in cell lines from synovial sarcoma (SS), Ewing's sarcoma (ES) and alveolar rhabdomyosarcoma (ARMS), all of which harbor chromosomal translocations...
February 27, 2023: Cell Death & Disease
#17
JOURNAL ARTICLE
Olivia W Lee, Calvin Rodrigues, Shu-Hong Lin, Wen Luo, Kristine Jones, Derek W Brown, Weiyin Zhou, Eric Karlins, Sairah M Khan, Sylvain Baulande, Virginie Raynal, Didier Surdez, Stephanie Reynaud, Rebeca Alba Rubio, Sakina Zaidi, Sandrine Grossetête, Stelly Ballet, Eve Lapouble, Valérie Laurence, Gaelle Pierron, Nathalie Gaspar, Nadège Corradini, Perrine Marec-Bérard, Nathaniel Rothman, Casey L Dagnall, Laurie Burdett, Michelle Manning, Kathleen Wyatt, Meredith Yeager, Raj Chari, Wendy M Leisenring, Andreas E Kulozik, Jennifer Kriebel, Thomas Meitinger, Konstantin Strauch, Thomas Kirchner, Uta Dirksen, Lisa Mirabello, Margaret A Tucker, Franck Tirode, Gregory T Armstrong, Smita Bhatia, Leslie L Robison, Yutaka Yasui, Laura Romero-Pérez, Wolfgang Hartmann, Markus Metzler, W Ryan Diver, Adriana Lori, Neal D Freedman, Robert N Hoover, Lindsay M Morton, Stephen J Chanock, Thomas G P Grünewald, Olivier Delattre, Mitchell J Machiela
Ewing sarcoma (EwS) is a rare bone and soft tissue malignancy driven by chromosomal translocations encoding chimeric transcription factors, such as EWSR1-FLI1, that bind GGAA motifs forming novel enhancers that alter nearby expression. We propose that germline microsatellite variation at the 6p25.1 EwS susceptibility locus could impact downstream gene expression and EwS biology. We performed targeted long-read sequencing of EwS blood DNA to characterize variation and genomic features important for EWSR1-FLI1 binding...
February 8, 2023: American Journal of Human Genetics
#18
REVIEW
Le Yu, Ian J Davis, Pengda Liu
Ewing sarcoma is the second most common bone tumor in childhood and adolescence. Currently, first-line therapy includes multidrug chemotherapy with surgery and/or radiation. Although most patients initially respond to chemotherapy, recurrent tumors become treatment refractory. Pathologically, Ewing sarcoma consists of small round basophilic cells with prominent nuclei marked by expression of surface protein CD99. Genetically, Ewing sarcoma is driven by a fusion oncoprotein that results from one of a small number of chromosomal translocations composed of a FET gene and a gene encoding an ETS family transcription factor, with ~85% of tumors expressing the EWSR1::FLI1 fusion...
January 6, 2023: Cancers
#19
JOURNAL ARTICLE
Ramona Palombo, Ilaria Passacantilli, Francesca Terracciano, Alessia Capone, Alessandro Matteocci, Simon Tournier, Antonio Alberdi, Valerio Chiurchiù, Elisabetta Volpe, Maria Paola Paronetto
Ewing sarcomas are aggressive pediatric tumors of bone and soft tissues driven by in frame chromosomal translocations that yield fusion proteins guiding the oncogenic program. Promising alternative strategies to ameliorate current treatments involve inhibition of the PI3K/AKT/mTOR pathway. In this study, we identified the activating transcription factor 3 (ATF3) as an important mediator of the PI3K/AKT/mTOR pathway in Ewing sarcoma cells. ATF3 exerted its pro-tumoral activity through modulation of several chemokine-encoding genes, including CXCL8...
February 28, 2023: Cancer Letters
#20
Tharamel M Muhuesein, Gurubharath Ilangovan, Alex Daniel Prabhu Arul Pitchai, Ealai A Parthasarathy, Rajamani Anand, Alam Khalil-Khan
Ewing's sarcoma (ES) is the second most common osseous tumor in young patients after osteosarcoma. All primitive neuroectodermal tumors (PNET) and Askin tumors are members of Ewing's sarcoma family of tumors (ESFT), which all have aberrant translocations between the 11th and 22nd chromosomes. Only one in five cases of Ewing's sarcoma occurs as extraskeletal. In this report, we describe a young female with a palpable lump on her spine who presented with paravertebral and thoracic extraskeletal Ewing's sarcoma (EES)...
October 2022: Curēus
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