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https://www.readbyqxmd.com/read/27920700/primary-ewing-sarcoma-primitive-neuroectodermal-tumor-of-the-stomach
#1
Safi Khuri, Hayim Gilshtein, Sa'd Sayidaa, Bishara Bishara, Yoram Kluger
Ewing sarcoma/primitive neuroectodermal tumor (ES/PNET) is a tumor of small round cells arising in skeletal tissues. These tumors rarely arise in the stomach. We present a 31-year-old healthy female patient who was admitted to our surgical ward due to upper gastrointestinal hemorrhage. Upper endoscopy revealed a large ulcerated bleeding mass originating from the lesser curvature. Biopsy revealed tumor cell immunoreactivity positive for CD99, vimentin, and Ki67 (an index of proliferation). These findings were compatible with gastric ES/PNET...
September 2016: Case Reports in Oncology
https://www.readbyqxmd.com/read/27919577/primary-cic-dux4-round-cell-sarcoma-of-the-kidney-a-treatment-refractory-tumor-with-poor-outcome
#2
Sébastien Bergerat, Philippe Barthelemy, Pascal Mouracade, Hervé Lang, Christian Saussine, Véronique Lindner, Didier Jacqmin
The CIC-DUX4 sarcoma is a subset of the undifferentiated small round cell sarcoma family, presently recognized as a new clinicopathological entity. It is a rare and highly aggressive tumor usually arising in the soft parts of the limbs and the trunk. Only a very few cases of primitive visceral CIC-DUX4 have been hitherto described. We report the case of a 29 year-old male patient with a primary CIC-DUX4 sarcoma of the kidney with lung metastasis. The outcome of the disease was rapidly unfavorable. Despite radical nephrectomy, the patient experienced an early local retroperitoneal recurrence associated with lung and liver metastases...
November 24, 2016: Pathology, Research and Practice
https://www.readbyqxmd.com/read/27918348/ewing-sarcoma-of-the-bone-with-ews-fli1-translocation-after-successful-treatment-of-primary-osteosarcoma
#3
Noriko Yodoya, Shotaro Iwamoto, Akihiko Matsumine, Eiichi Azuma, Hidemi Toyoda, Yoshihiro Miura, Kaname Nakatani, Hiroshi Imai, Masahiro Hirayama, Yoshihiro Komada
Although prognosis in patients with localized osteosarcoma has been dramatically improved by the introduction of multiple chemotherapy agents known as combination chemotherapy, there is growing concern about the development of secondary malignant neoplasms. We report the case of a 13-year-old girl in whom the diagnosis of Ewing sarcoma of bone localized on the shaft of left femur was made 2 years after successful treatment without radiotherapy for osteosarcoma of right proximal femur. EWS-FLI1 fusion gene was detected by reverse transcriptase-polymerase chain reaction...
December 1, 2016: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/27914587/outcomes-of-chest-wall-resections-in-pediatric-sarcoma-patients
#4
Carmen Lopez, Arlene Correa, Ara Vaporciyan, Mary Austin, David Rice, Andrea Hayes-Jordan
PURPOSE: Chest wall tumors in pediatric patients are rare. This study evaluates outcomes in pediatric patients who have undergone chest wall resections secondary to sarcomas. METHODS: A retrospective review was performed for patients <19years old who underwent chest wall resections for sarcoma 1999-2014 at the University of Texas MD Anderson Cancer Center. RESULTS: Of 44 patients, Ewing's sarcoma (n=18) and osteosarcoma (n=16) were most common...
October 28, 2016: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/27913051/erg-expression-in-multiple-myeloma-a-potential-diagnostic-pitfall
#5
Juliana Knief, Katharina Reddemann, Jan Gliemroth, Swantje Brede, Tobias Bartscht, Christoph Thorns
INTRODUCTION: ERG expression has been described as a frequent event in prostate cancer indicating poor prognosis and promoting oncogenesis. It has also been demonstrated in Ewing's sarcoma, acute myeloid leukemia and acute T-lymphoblastic leukemia but could not be found in other epithelial tumors, Hodgkin's or Non-Hodgkin's lymphoma. We aimed to analyze ERG expression in multiple myeloma, following an index case of a patient with metastases of unknown origin in the spine strongly expressing ERG, which were thought to be of prostatic origin but turned out to be plasmacytic lesions...
November 3, 2016: Pathology, Research and Practice
https://www.readbyqxmd.com/read/27903134/o-glcnac-glycosylation-stoichiometry-of-the-fet-protein-family-only-ews-is-glycosylated-with-a-high-stoichiometry
#6
Kazuo Kamemura
Of the FET (fused in sarcoma [FUS]/Ewing sarcoma protein [EWS]/TATA binding protein-associated factor 15 [TAF15]) family of heterogeneous nuclear ribonucleoprotein particle proteins, FUS and TAF15 are consistently and EWS variably found in inclusion bodies in neurodegenerative diseases such as frontotemporal lobar degeneration associated with FUS. It is speculated that dysregulation of FET proteins at the post-translational level is involved in their cytoplasmic deposition. Here, the O-linked β-N-acetylglucosamine (O-GlcNAc) glycosylation stoichiometry of the FET proteins was chemoenzymatically analyzed, and it was found that only EWS is dynamically glycosylated with a high stoichiometry in the neural cell lines tested and in mouse brain...
December 1, 2016: Bioscience, Biotechnology, and Biochemistry
https://www.readbyqxmd.com/read/27900390/-is-postoperative-radiotherapy-necessary-in-localised-standard-risk-ewing-sarcoma-the-primary-tumor-bulk-decides-upon-adjuvant-radiotherapy-after-induction-chemotherapy-and-surgery
#7
Irenäus A Adamietz
No abstract text is available yet for this article.
November 29, 2016: Strahlentherapie und Onkologie: Organ der Deutschen Röntgengesellschaft ... [et Al]
https://www.readbyqxmd.com/read/27898190/primary-ewing-s-sarcoma-of-the-sinonasal-tract-in-adults-a-challenging-disease
#8
Davide Lombardi, Davide Mattavelli, Luca O Redaelli De Zinis, Remo Accorona, Maria L Morassi, Fabio Facchetti, Vittorio Ferrari, Davide Farina, Rossella Bertulli, Piero Nicolai
BACKGROUND: Sinonasal localization of Ewing's sarcoma in adults is an exceedingly rare event. METHODS: The clinical records of 5 patients with primary sinonasal Ewing's sarcoma treated from 1992 to 2012 were retrospectively analyzed. All pathologic slides were reviewed by 2 experienced pathologists. All patients underwent multimodality treatments. RESULTS: Median age was 36 years (range, 25-52 years). At referral, 2 patients had the original diagnosis changed by review of the histologic slides...
November 29, 2016: Head & Neck
https://www.readbyqxmd.com/read/27895949/primary-ewing-s-sarcoma-of-the-spine-in-a-two-year-old-boy
#9
Ali J Electricwala, Jaffer T Electricwala
Ewing's Sarcoma (ES) is a highly malignant bone tumour. It may involve any part of the skeleton but the most frequent parts are the ilium and diaphysis of femur and tibia (Alfeeli et al., 2005; Zhu et al., 2012). Primary ES of the spine is extremely rare (Yan et al., 2011). It accounts for only 3.5 to 14.9 percent of all primary bone sarcomas. The age of presentation ranges from 12 to 24 years (median 21 years) (Ferguson, 1999; Sharafuddin et al., 1992; Klimo Jr. et al., 2009). We report an unusual case of primary ES of the spine in a two-year-old boy, who presented to us with paraparesis and features of cauda equina syndrome...
2016: Case Reports in Orthopedics
https://www.readbyqxmd.com/read/27894957/dna-methylation-profiling-identifies-ptrf-cavin-1-as-a-novel-tumor-suppressor-in-ewing-sarcoma-when-co-expressed-with-caveolin-1
#10
Juan Huertas-Martínez, Franck Court, Santiago Rello-Varona, David Herrero-Martín, Olga Almacellas-Rabaiget, Miguel Sáinz-Jaspeado, Silvia Garcia-Monclús, Laura Lagares-Tena, Raquel Buj, Lourdes Hontecillas-Prieto, Ana Sastre, Daniel Azorin, Xavier Sanjuan, Roser López-Alemany, Sebastian Moran, Josep Roma, Soledad Gallego, Jaume Mora, Xavier García Del Muro, Paloma H Giangrande, Miquel A Peinado, Javier Alonso, Enrique de Alava, Dave Monk, Manel Esteller, Oscar M Tirado
Epigenetic modifications have been shown to be important in developmental tumors as Ewing sarcoma. We profiled the DNA methylation status of 15 primary tumors, 7 cell lines, 10 healthy tissues and 4 human mesenchymal stem cells lines samples using the Infinium Human Methylation 450k. Differential methylation analysis between Ewing sarcoma and reference samples revealed 1,166 hypermethylated and 864 hypomethylated CpG sites (Bonferroni p<0.05, δ-β-value with absolute difference of >0.20) corresponding to 392 and 470 genes respectively...
November 25, 2016: Cancer Letters
https://www.readbyqxmd.com/read/27888797/tumorigenicity-of-ewing-sarcoma-is-critically-dependent-on-the-trithorax-proteins-mll1-and-menin
#11
Laurie K Svoboda, Natashay Bailey, Raelene A Van Noord, Melanie A Krook, Ashley Harris, Cassondra Cramer, Brooke Jasman, Rajiv M Patel, Dafydd Thomas, Dmitry Borkin, Tomasz Cierpicki, Jolanta Grembecka, Elizabeth R Lawlor
Developmental transcription programs are epigenetically regulated by the competing actions of polycomb and trithorax (TrxG) protein complexes, which repress and activate genes, respectively. Ewing sarcoma is a developmental tumor that is associated with widespread de-regulation of developmental transcription programs, including HOX programs. Posterior HOXD genes are abnormally over-expressed by Ewing sarcoma and HOXD13, in particular, contributes to the tumorigenic phenotype. In MLL1 fusion-driven leukemia, aberrant activation of HOXA genes is epigenetically mediated by the TrxG complex and HOXA gene expression and leukemogenesis are critically dependent on the protein-protein interaction between the TrxG proteins MLL1 and menin...
November 18, 2016: Oncotarget
https://www.readbyqxmd.com/read/27879517/dux4-immunohistochemistry-is-a-highly-sensitive-and-specific-marker-for-cic-dux4-fusion-positive-round-cell-tumor
#12
Bradford Siegele, Jon Roberts, Jennifer O Black, Erin Rudzinski, Sara O Vargas, Csaba Galambos
The histologic differential diagnosis of pediatric and adult round cell tumors is vast and includes the recently recognized entity CIC-DUX4 fusion-positive round cell tumor. The diagnosis of CIC-DUX4 tumor can be suggested by light microscopic and immunohistochemical features, but currently, definitive diagnosis requires ancillary genetic testing such as conventional karyotyping, fluorescence in situ hybridization, or molecular methods. We sought to determine whether DUX4 expression would serve as a fusion-specific immunohistochemical marker distinguishing CIC-DUX4 tumor from potential histologic mimics...
November 22, 2016: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/27875302/proteasomal-degradation-of-the-ews-fli1-fusion-protein-is-regulated-by-a-single-lysine-residue
#13
Maria E Gierisch, Franziska Pfistner, Laura A Lopez-Garcia, Lena Harder, Beat W Schäfer, Felix K Niggli
E-26 transformation specific (ETS) proteins are transcription factors directing gene expression through their conserved DNA-binding domain. They are implicated as truncated forms or interchromosomal rearrangements in a variety of tumors including Ewing sarcoma, a pediatric tumor of the bone. Tumor cells express the chimeric oncoprotein EWS-FLI1 from a specific t(22;11)(q24;12) translocation. EWS-FLI1 harbors a strong transactivation domain from EWSR1 and the DNA-binding ETS domain of FLI1 in the C-terminal part of the protein...
November 8, 2016: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/27869363/is-the-memorial-sloan-kettering-cancer-centre-mskcc-sarcoma-nomogram-useful-in-an-asian-population
#14
Deanna Wan Jie Ng, Grace Hwei Ching Tan, Claramae Shulyn Chia, Cindy Xindi Lim, Soo Khee Chee, Richard Hong Hui Quek, Mohamad Farid, Melissa Ching Ching Teo
AIM: A nomogram for prediction of 12-year sarcoma-specific survival has been developed based on patients with soft tissue sarcomas treated in Memorial Sloan Kettering Cancer Centre (MSKCC). We aim to evaluate the predictive accuracy of the MSKCC sarcoma nomogram in a cohort of patients treated at an Asian institution. This has not been validated in an Asian population and thus its universal applicability remains unproven. MATERIALS AND METHODS: Between 1990 and 2013, 840 adult patients underwent treatment for primary soft tissue sarcoma (STS) at the National Cancer Centre Singapore...
November 21, 2016: Asia-Pacific Journal of Clinical Oncology
https://www.readbyqxmd.com/read/27863422/in-silico-and-in-vitro-drug-screening-identifies-new-therapeutic-approaches-for-ewing-sarcoma
#15
Ziyan Y Pessetto, Bin Chen, Hani Alturkmani, Stephen Hyter, Colleen A Flynn, Michael Baltezor, Yan Ma, Howard G Rosenthal, Kathleen A Neville, Scott J Weir, Atul J Butte, Andrew K Godwin
The long-term overall survival of Ewing sarcoma (EWS) patients remains poor; less than 30% of patients with metastatic or recurrent disease survive despite aggressive combinations of chemotherapy, radiation and surgery. To identify new therapeutic options, we employed a multi-pronged approach using in silico predictions of drug activity via an integrated bioinformatics approach in parallel with an in vitro screen of FDA-approved drugs. Twenty-seven drugs and forty-six drugs were identified, respectively, to have anti-proliferative effects for EWS, including several classes of drugs in both screening approaches...
November 16, 2016: Oncotarget
https://www.readbyqxmd.com/read/27860482/long-noncoding-rna-ewsat1-promotes-osteosarcoma-cell-growth-and-metastasis-through-suppression-of-meg3-expression
#16
Lishan Sun, Cheng Yang, Juan Xu, Yanhong Feng, Liguo Wang, Tao Cui
Osteosarcoma (OS) is the most common primary bone tumor in children and adolescents. Long noncoding RNAs (lncRNAs) are a class of transcriptional products of the genome without protein-coding potential. Recently, lncRNA Ewing sarcoma-associated transcript 1 (EWSAT1) was functionally identified in Ewing sarcoma, a highly aggressive primary pediatric bone tumor. However, whether EWSAT1 plays a role in OS remains unclear. In the present study, gain- and loss-of-function assays demonstrated that EWSAT1 enhanced OS cell proliferation, migration, and invasion...
November 18, 2016: DNA and Cell Biology
https://www.readbyqxmd.com/read/27856382/prognostic-determinants-and-treatment-outcomes-analysis-of-osteosarcoma-and-ewing-sarcoma-of-the-spine
#17
Armin Arshi, Justin Sharim, Don Y Park, Howard Y Park, Hamed Yazdanshenas, Nicholas M Bernthal, Arya N Shamie
BACKGROUND CONTEXT: Osteosarcoma (OGS) and Ewing sarcoma (EWS) are the two classic primary malignant bone tumors. Due to the rarity of these tumors, evidence on demographics, survival determinants, and treatment outcomes for primary disease of the spine are limited and derived from small case series. PURPOSE: To use population-level data to determine the epidemiology and prognostic indicators in patients with OGS and EWS of the osseous spine. STUDY DESIGN/SETTING: Large-scale retrospective study...
November 14, 2016: Spine Journal: Official Journal of the North American Spine Society
https://www.readbyqxmd.com/read/27843394/trabectedin-followed-by-irinotecan-can-stabilize-disease-in-advanced-translocation-positive-sarcomas-with-acceptable-toxicity
#18
J Herzog, F von Klot-Heydenfeldt, S Jabar, A Ranft, C Rossig, U Dirksen, J Van den Brande, M D'Incalci, I von Luettichau, P J Grohar, W E Berdel, St Burdach
Background. Preclinical data indicate that trabectedin followed by irinotecan has strong synergistic effects on Ewing sarcoma. This is presumably due to hypersensitization of the tumor cells to the camptothecin as an effect of trabectedin in addition to synergistic suppression of EWS-FLI1 downstream targets. A strong effect was also reported in a human rhabdomyosarcoma xenograft. Procedure. Twelve patients with end-stage refractory translocation-positive sarcomas were treated with trabectedin followed by irinotecan within a compassionate use program...
2016: Sarcoma
https://www.readbyqxmd.com/read/27843262/ewing-s-sarcoma-of-mandible-an-impressive-case-of-spontaneous-mandible-regeneration
#19
Ioannis Chatzistefanou, Sotiria Kabesi, Konstantinos Paraskevopoulos, Dimitrios Koliouskas, Konstantinos Antoniades
: Ewing's sarcoma (ES) is an uncommon aggressive bone malignancy that mainly affects children and adolescents. Mandible involvement is quite rare and usually represents metastasis from another skeletal site. Combined therapy including wide surgical resection and preoperative and postoperative chemotherapy has been demonstrated as the mainstay of therapeutic approach. As improved therapeutic modalities have significantly increased survival over the last decades, functional and esthetic reconstruction of the postmandibulectomy defect is usually necessary for patient's total rehabilitation...
July 2016: International Journal of Clinical Pediatric Dentistry
https://www.readbyqxmd.com/read/27835596/cd99-triggering-induces-methuosis-of-ewing-sarcoma-cells-through-igf-1r-ras-rac1-signaling
#20
Maria Cristina Manara, Mario Terracciano, Caterina Mancarella, Marika Sciandra, Clara Guerzoni, Michela Pasello, Andrea Grilli, Nicoletta Zini, Piero Picci, Mario P Colombo, Andrea Morrione, Katia Scotlandi
CD99 is a cell surface molecule that has emerged as a novel target for Ewing sarcoma (EWS), an aggressive pediatric bone cancer. This report provides the first evidence of methuosis in EWS, a non-apoptotic form of cell death induced by an antibody directed against the CD99 molecule. Upon mAb triggering, CD99 induces an IGF-1R/RAS/Rac1 complex, which is internalized into RAB5-positive endocytic vacuoles. This complex is then dissociated, with the IGF-1R recycling to the cell membrane while CD99 and RAS/Rac1 are sorted into immature LAMP-1-positive vacuoles, whose excessive accumulation provokes methuosis...
November 7, 2016: Oncotarget
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