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Ewings sarcoma

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https://www.readbyqxmd.com/read/28429277/primary-ewing-sarcoma-primitive-neuroectodermal-tumor-of-the-kidney-a-clinicopathologic-study-of-23-cases
#1
Paari Murugan, Priya Rao, Pheroze Tamboli, Bogdan Czerniak, Charles C Guo
Primary Ewing sarcoma / primitive neuroectodermal tumor (ES) of the kidney is a rare neoplasm with limited clinicopathologic data. We report 23 such cases with no history of ES elsewhere in the body. The patients included 13 male and 10 female, aged 8-70 years (mean, 31 years). The average tumor size was 11.7 cm (range, 5-20 cm). Microscopic analysis showed predominantly lobular growth (n = 14), with focal papillary (n = 3), alveolar (n = 1), and hemangiopericytoma-like (n = 1) patterns. Several tumors (n = 11) exhibited robust mitotic activity (>10 mitoses/10 high-power fields)...
April 20, 2017: Pathology Oncology Research: POR
https://www.readbyqxmd.com/read/28420319/bcor-ccnb3-undifferentiated-sarcoma-does-immunohistochemistry-help-in-the-identification
#2
Kathrin Ludwig, Rita Alaggio, Angelica Zin, Marica Peron, Vincenza Guzzardo, Stefania Benini, Alberto Righi, Marco Gambarotti
Recent methodology has enabled the identification of some new genetic subgroups within the melting pot of lesions presently classified by the 2013 WHO classification as "undifferentiated/unclassified sarcomas". One of these subgroups is characterized by a paracentric inversion of the X chromosome with consequent formation of a BCOR-CCNB3 fusion. Clinical and pathological features of these tumors overlap with the Ewing sarcoma family as well as other soft tissue sarcomas, thus making them difficult to diagnose...
January 1, 2017: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/28420037/does-surgery-or-radiation-provide-the-best-overall-survival-in-ewing-s-sarcoma-a-review-of-the-national-cancer-data-base
#3
Benjamin J Miller, Yubo Gao, Kyle R Duchman
BACKGROUND AND OBJECTIVES: There is continuing debate regarding the ideal modality for local control of the primary tumor for patients with Ewing's sarcoma. The primary aim of this study is to investigate the impact of the method of local control on overall survival in patients with Ewing's sarcoma. METHODS: The National Cancer Data Base was used to identify patients <40 years of age with high-grade Ewing's sarcoma of bone. A Kaplan-Meier survival analysis was performed at 2, 5, and 10 years...
April 18, 2017: Journal of Surgical Oncology
https://www.readbyqxmd.com/read/28419662/ewing-sarcoma-of-the-sternal-manubrium-first-report-of-a-case-in-australasia-and-review-of-the-literature
#4
Danus Ravindran, Yassar Alamri, Samantha Gerrie, Andrew Brainard
No abstract text is available yet for this article.
April 16, 2017: ANZ Journal of Surgery
https://www.readbyqxmd.com/read/28417853/the-role-of-18-f-fdg-pet-ct-in-pediatric-sarcoma
#5
REVIEW
Douglas J Harrison, Marguerite T Parisi, Barry L Shulkin
Considerable debate remains regarding how best to incorporate (18)F-FDG-PET/CT into clinical practice for pediatric sarcomas. Although there is a clear role for (18)F-FDG-PET/CT in staging pediatric sarcoma, the value of (18)F-FDG-PET/CT in prognostication for pediatric sarcomas remains unclear. In osteosarcoma, Ewing sarcoma, and rhabdomyosarcoma, (18)F-FDG-PET/CT may be most useful in the identification of skeletal metastases, where the literature consistently suggests that it has improved sensitivity and specificity as compared to bone scintigraphy...
May 2017: Seminars in Nuclear Medicine
https://www.readbyqxmd.com/read/28417551/morbidity-and-mortality-after-treatment-of-ewing-sarcoma-a-single-institution-experience
#6
Danielle Novetsky Friedman, Katherine Chastain, Joanne F Chou, Chaya S Moskowitz, Roberto Adsuar, Leonard H Wexler, Alexander J Chou, Amelia DeRosa, Joanne Candela, Heather Magnan, Shawn Pun, Tamara Kahan, Suzanne L Wolden, Paul A Meyers, Kevin C Oeffinger
BACKGROUND: Children, adolescents, and young adults treated for Ewing sarcoma (ES) are at risk for disease-related and treatment-related complications. We aimed to describe early and late overall mortality, cause-specific mortality, and key adverse health outcomes in a large, single-institutional cohort of patients with ES. METHODS: Patients with ES diagnosed at age less than 40 years and treated at Memorial Sloan Kettering between 1974 and 2012 were included. Overall survival was estimated using Kaplan-Meier methods...
April 18, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28412026/primary-bone-sarcomas-in-patients-over-40-years-of-age-a-retrospective-study-using-data-from-the-bone-tumor-registry-of-japan
#7
Akihito Nagano, Daichi Ishimaru, Yutaka Nishimoto, Haruhiko Akiyama, Akira Kawai
BACKGROUND: Optimal treatments of patients aged >40 years with osteosarcoma and Ewing sarcoma and of patients with other types of bone sarcoma have not yet been established. METHODS: The clinical characteristics of 746 patients with primary bone sarcomas aged >40 years listed in the nationwide tumor registry (Bone and Soft Tissue Tumor Registry of Japan) were investigated. Patients with Ewing sarcoma, conventional chondrosarcoma, multiple myeloma, and malignant lymphoma were excluded...
April 12, 2017: Journal of Orthopaedic Science: Official Journal of the Japanese Orthopaedic Association
https://www.readbyqxmd.com/read/28410188/like-or-dislike-impact-of-facebook-on-ewing-sarcoma-treatment
#8
Paul Ruckenstuhl, Michael Schippinger, Paul Liebmann, Andreas Leithner, Gerwin Bernhardt
BACKGROUND: An increasing number of patients are raising their voices in online forums to exchange health-related information. Facebook is the leading social media platform with more than 1 billion international daily users recorded in the summer of 2015. Facebook has a dynamic audience and is utilized in a number of ways, discussing medical issues being one of them. Ewing sarcoma mainly affects teenagers and young adults. Additionally, many individuals within this age group are regular users of Facebook...
August 25, 2016: JMIR Cancer
https://www.readbyqxmd.com/read/28405084/primitive-neuroectodermal-tumors-of-the-kidney
#9
Geetha Narayanan, Varun Rajan, T R Preethi
Ewing's sarcoma/primitive neuroectodermal tumor (ES/PNET) rarely occurs as a primary renal tumor. The disease affects young adults and children and has an aggressive course. The clinical presentation and imaging of these tumors are nonspecific, and they often present at an advanced stage. We present the clinical features, imaging, diagnosis, and treatment of 7 cases of renal PNET (4 men, 3 women; median age, 32 years). Common presenting symptoms were flank or abdominal pain and a mass in the abdomen. On imaging, a large heterogenous infiltrating renal mass with areas of calcification, hemorrhage, and necrosis and tumor thrombus can give a clue to the diagnosis of renal PNET...
April 2017: Proceedings of the Baylor University Medical Center
https://www.readbyqxmd.com/read/28404944/recombinant-methioninase-effectively-targets-a-ewing-s-sarcoma-in-a-patient-derived-orthotopic-xenograft-pdox-nude-mouse-model
#10
Takashi Murakami, Shukuan Li, Qinghong Han, Yuying Tan, Tasuku Kiyuna, Kentaro Igarashi, Kei Kawaguchi, Ho Kyoung Hwang, Kentaro Miyake, Arun S Singh, Scott D Nelson, Sarah M Dry, Yunfeng Li, Yukihiko Hiroshima, Thinzar M Lwin, Jonathan C DeLong, Takashi Chishima, Kuniya Tanaka, Michael Bouvet, Itaru Endo, Fritz C Eilber, Robert M Hoffman
Methionine dependence is due to the overuse of methionine for aberrant transmethylation reactions in cancer. Methionine dependence may be the only general metabolic defect in cancer. In order to exploit methionine dependence for therapy, our laboratory previously cloned L-methionine α-deamino-γ-mercaptomethane lyase [EC 4.4.1.11]). The cloned methioninase, termed recombinant methioninase, or rMETase, has been tested in mouse models of human cancer cell lines. Ewing's sarcoma is recalcitrant disease even though development of multimodal therapy has improved patients'outcome...
March 1, 2017: Oncotarget
https://www.readbyqxmd.com/read/28404587/cic-dux4-induces-small-round-cell-sarcomas-distinct-from-ewing-sarcoma
#11
Toyoki Yoshimoto, Miwa Tanaka, Mizuki Homme, Yukari Yamazaki, Yutaka Takazawa, Cristina R Antonescu, Takuro Nakamura
CIC-DUX4 sarcoma (CDS) or CIC-rearranged sarcoma is a  subcategory of small round cell sarcoma resembling the morphological phenotypes of Ewing sarcoma (ES). Hoever, recent clinicopathologic and molecular genetic analyses indicate that CDS is an independent disease entity from ES. Few ancillary markers have been used in the differential diagnosis of CDS, and additional CDS-specific biomarkers are needed for more definitive classification. Here we report the generation of an ex vivo mouse model for CDS by transducing embryonic mesenchymal cells (eMC) with human CIC-DUX4 cDNA...
April 12, 2017: Cancer Research
https://www.readbyqxmd.com/read/28389532/rna-binding-proteins-with-prion-like-domains-in-health-and-disease
#12
REVIEW
Alice Ford Harrison, James Shorter
Approximately 70 human RNA-binding proteins (RBPs) contain a prion-like domain (PrLD). PrLDs are low-complexity domains that possess a similar amino acid composition to prion domains in yeast, which enable several proteins, including Sup35 and Rnq1, to form infectious conformers, termed prions. In humans, PrLDs contribute to RBP function and enable RBPs to undergo liquid-liquid phase transitions that underlie the biogenesis of various membraneless organelles. However, this activity appears to render RBPs prone to misfolding and aggregation connected to neurodegenerative disease...
April 7, 2017: Biochemical Journal
https://www.readbyqxmd.com/read/28384876/primary-ewing-sarcoma-of-sphenoid-bone-with-intracranial-extension-a-common-tumour-at-an-uncommon-location
#13
Guddi Rani Singh, Vijayanand Choudhary, Rawi Agrawal
Primary Ewing Sarcoma of the cranial bone is rare, contributing to only 1% of all Ewing Sarcomas. Primary cranial Ewing Sarcoma occurs most commonly in temporal bone followed by parietal and occipital bones. Sphenoid bone is less commonly involved. We report a case of Ewing Sarcoma of the sphenoid bone with intra-cranial extension in a 20-month-old boy. On CT scan a provisional diagnosis of rhabdomyosarcoma was made. Fine Needle Aspiration Cytology (FNAC) and histopathological examination of core needle biopsy showed small round cell tumour...
February 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28382842/ewing-sarcoma-and-atypical-teratoid-rhabdoid-tumor
#14
M Cristina Pacheco, Michelle Dolan, Anne Bendel
Ewing sarcoma (ES) and atypical teratoid rhabdoid tumor (ATRT) are high-grade malignancies of childhood, each of which is associated with genetic abnormalities on chromosome 22. ES is typically characterized by rearrangement of the EWSR1 locus and ATRT by deletion of SMARCB1. We report a case with an unusual fluorescence in situ hybridization signal pattern consistent with EWSR1 rearrangement that was shown to have loss of INI1 expression by immunohistochemistry due to deletion in the long arm of one chromosome 22...
January 1, 2017: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/28377061/respiratory-gated-bilateral-pulmonary-radiotherapy-for-ewing-s-sarcoma-and-nephroblastoma-in-children-and-young-adults-dosimetric-and-clinical-feasibility-studies
#15
C Demoor-Goldschmidt, S Chiavassa, S Josset, M-A Mahé, S Supiot
PURPOSE: Bilateral pulmonary radiotherapy in children and young adults aims to reduce the recurrence of lung metastases. The radiation field includes liver tissue, which is sensitive to even low radiation doses. We investigated the feasibility of respiratory gating radiotherapy using voluntary deep inspiration breath hold and its toxicity in these patients. PATIENTS AND METHOD: A retrospective clinical review was conducted for all patients who had undergone bilateral pulmonary radiotherapy, with or without deep inspiration breath hold, treated in our institution between October 1999 and May 2012...
April 1, 2017: Cancer Radiothérapie: Journal de la Société Française de Radiothérapie Oncologique
https://www.readbyqxmd.com/read/28376845/rare-adrenal-gland-incidentaloma-an-unusual-ewing-s-sarcoma-family-of-tumor-presentation-and-literature-review
#16
Hui Guo, Shuaiqi Chen, Shukun Liu, Kaixuan Wang, Erpeng Liu, Faping Li, Yuchuan Hou
BACKGROUND: Members of the Ewing's sarcoma family of tumor (ESFT) are malignant neoplasms and rarely observed in the adrenal gland. CASE PRESENTATION: We report an extremely exceptional case of ESFT rising from the adrenal gland in a 57-year-old Chinese man. The patient was hospitalized with abdominal swelling for 2 months. Computed tomography (CT) scan revealed a nearly-circular mass measuring about 8.1 × 10.6 cm in the right adrenal region. The patient underwent right adrenal resection...
April 4, 2017: BMC Urology
https://www.readbyqxmd.com/read/28355116/quality-of-survivorship-in-a-rare-disease-clinicofunctional-outcome-and-physical-activity-in-an-observational-cohort-study-of-618-long-term-survivors-of-ewing-sarcoma
#17
Andreas Ranft, Corinna Seidel, Christiane Hoffmann, Michael Paulussen, Ann-Christin Warby, Henk van den Berg, Ruth Ladenstein, Claudia Rossig, Uta Dirksen, Dieter Rosenbaum, Herbert Juergens
Purpose Significantly improved survival rates in patients with Ewing sarcoma have raised interest in accessing the quality of long-term survivorship. In this study, subjective and objective measurement tools, preclassified as physical or mental scores, were used to assess clinicofunctional outcome and physical activity after intensive bone tumor treatment. Methods Long-term outcome of 618 survivors from consecutive Ewing sarcoma trials was assessed by the Toronto Extremity Salvage Score, Short-Form Health Survey (SF-36), Brief Symptom Inventory (BSI), and Rosenberg Self-Esteem Scale questionnaires and by the accelerometric StepWatch 3 Activity Monitor...
March 29, 2017: Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology
https://www.readbyqxmd.com/read/28346326/sarcomas-with-cic-rearrangements-are-a-distinct-pathologic-entity-with-aggressive-outcome-a-clinicopathologic-and-molecular-study-of-115-cases
#18
Cristina R Antonescu, Adepitan A Owosho, Lei Zhang, Sonja Chen, Kemal Deniz, Joseph M Huryn, Yu-Chien Kao, Shih-Chiang Huang, Samuel Singer, William Tap, Inga-Marie Schaefer, Christopher D Fletcher
CIC-DUX4 gene fusion, resulting from either a t(4;19) or t(10;19) translocation, is the most common genetic abnormality detected in EWSR1-negative small blue round cell tumors. Following their discovery it was debated if these tumors should be classified as variants of Ewing sarcoma (ie, atypical Ewing sarcoma) or as a stand-alone pathologic entity. As such the WHO classification temporarily grouped the CIC-rearranged tumors under undifferentiated sarcomas with round cell phenotype, until further clinical evidence was available...
March 24, 2017: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28345284/role-of-fine-needle-aspiration-cytology-in-the-diagnosis-of-a-rare-case-of-a-poorly-differentiated-synovial-sarcoma-with-rhabdoid-features-including-treatment-implications
#19
Bharat Rekhi, Omshree Shetty, Mukta Ramadwar, Venkatesh Rangarajan, Jyoti Bajpai
Synovial sarcoma is a high-grade, soft tissue sarcoma that is relatively chemosensitive. Its exact diagnosis is crucial, including differentiation from its closest diagnostic mimic, ie, Ewing sarcoma, in view of different treatment options, including chemotherapy regimens, for both these tumors. A 15-year-old girl presented with a recurrent soft tissue mass in her right popliteal region, which was diagnosed as Ewing sarcoma, based on positive immunoexpression of MIC2/CD99, Fli1 and negative expression of LCA and desmin...
March 27, 2017: Diagnostic Cytopathology
https://www.readbyqxmd.com/read/28344885/pappalysin-1-t-cell-receptor-transgenic-allo-restricted-t-cells-kill-ewing-sarcoma-in-vitro-and-in-vivo
#20
Andreas Kirschner, Melanie Thiede, Thomas G P Grünewald, Rebeca Alba Rubio, Günther H S Richter, Thomas Kirchner, Dirk H Busch, Stefan Burdach, Uwe Thiel
Pregnancy-associated plasma protein-A (PAPPA), also known as pappalysin, is a member of the insulin-like growth factor (IGF) family. PAPPA acts as a protease, cleaving IGF inhibitors, i.e., IGF binding proteins (IGFBPs), thereby setting free IGFs. The insulin/IGF-axis is involved in cancer in general and in Ewing sarcoma (ES) in particular. ES is a highly malignant bone tumor characterized by early metastatic spread. PAPPA is associated with various cancers. It is overexpressed and required for proliferation in ES...
2017: Oncoimmunology
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