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Ewing sarcoma origination

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https://www.readbyqxmd.com/read/28129857/discovering-novel-oncogenic-pathways-and-new-therapies-using-zebrafish-models-of-sarcoma
#1
M N Hayes, D M Langenau
Sarcoma is a type of cancer affecting connective, supportive, or soft tissue of mesenchymal origin. Despite rare incidence in adults (<1%), over 15% of pediatric cancers are sarcoma. Sadly, both adults and children with relapsed or metastatic disease have devastatingly high rates of mortality. Current treatment options for sarcoma include surgery, radiation, and/or chemotherapy; however, significant limitations exist with respect to the efficacy of these strategies. Strong impetus has been placed on the development of novel therapies and preclinical models for uncovering mechanisms involved in the development, progression, and therapy resistance of sarcoma...
2017: Methods in Cell Biology
https://www.readbyqxmd.com/read/28060373/small-round-blue-cell-tumors-of-the-sinonasal-tract-a-differential-diagnosis-approach
#2
Lester Dr Thompson
One of the most challenging diagnostic categories within tumors of the sinonasal tract is the small round blue cell tumors. Biopsies are usually small and limited, resulting in considerable diagnostic difficulty for practicing surgical pathologists. These tumors share several overlapping histologic and immunophenotypic findings while also showing considerable variation within and between cases. Specific tumor site of origin, imaging findings, and clinical findings must be combined with the histology and pertinent ancillary studies if the correct diagnosis is to be reached...
January 2017: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
https://www.readbyqxmd.com/read/27920700/primary-ewing-sarcoma-primitive-neuroectodermal-tumor-of-the-stomach
#3
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/27913051/erg-expression-in-multiple-myeloma-a-potential-diagnostic-pitfall
#4
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/27898190/primary-ewing-s-sarcoma-of-the-sinonasal-tract-in-adults-a-challenging-disease
#5
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/27751600/pediatric-soft-tissue-tumor-pathology-a-happy-morpho-molecular-union
#6
Maureen O'Sullivan
Since its foundation by remarkably talented and insightful individuals, prominently including Pepper Dehner, pediatric soft tissue tumor pathology has developed at an immense rate. The morphologic classification of tumoral entities has extensively been corroborated, but has also evolved with refinement or realignment of these classifications, through accruing molecular data, with many derivative ancillary diagnostic assays now already well-established. Tumors of unclear histogenesis, classically morphologically undifferentiated, are prominent amongst pediatric sarcomas, however, the classes of undifferentiated round- or spindle-cell-tumors-not-otherwise-specified are being dismantled gradually with the identification of their molecular underpinnings...
November 2016: Seminars in Diagnostic Pathology
https://www.readbyqxmd.com/read/27703957/primary-renal-primitive-neuroectodermal-tumor-ewing-s-sarcoma-imaging-and-pathologic-findings-of-a-patient-with-a-nine-year-eight-month-disease-free-period-case-report-and-review-of-literature
#7
Ivan Zokalj, Jasminka Igrec, Antonio Plesnar
INTRODUCTION: Primitive neuroectodermal tumor (PNET)/Ewing's sarcoma (EWS) belongs to a family of neoplasms that are presumed to originate from the neuroectodermal crest. PNET/EWSs are highly aggressive malignancies that usually present in the form of bone or soft tissue masses and usually affect adolescents and young adults. Primary PNET/EWS of the kidney is very rare. CASE PRESENTATION: We present the case of a 32-year-old female patient with primary renal PNET/EWS diagnosed nine years and eight months earlier...
July 2016: Nephro-urology Monthly
https://www.readbyqxmd.com/read/27613543/molecular-cytogenetic-characterization-of-two-established-esft-cell-lines
#8
Masako Ishiguro, Mutsumi Yuki, Tomoko Fukushige, Mikio Mizoguchi, Yasuhiko Kaneko, Takeshita Morishige, Hiroshi Iwasaki
Ewing's sarcoma/primitive neuroectodermal tumor/Askin's tumor (Ewing`s sarcoma family of tumors: ESFT) is the most common type of malignant tumor of bone and soft tissue in children and young adults, and morphologically is a member of a group of small round cell tumors. We report, here, on the establishment of two human ESFT cell lines, FU-PNET-3 and FU-PNET-4, from the iliac and the chest wall, respectively, the cells of both cell lines were tumorigenic in immunodeficient mice. Histologically, both original and xenograft tumors and cultured cells were composed of small round cells with positive immunoreactivity for CD99 and Nkx2...
January 2017: Human Cell
https://www.readbyqxmd.com/read/27595362/sarcomas-of-soft-tissue-and-bone
#9
Andrea Ferrari, Uta Dirksen, Stefan Bielack
The definition of soft tissue and bone sarcomas include a large group of several heterogeneous subtypes of mesenchymal origin that may occur at any age. Among the different sarcomas, rhabdomyosarcoma, synovial sarcoma, Ewing sarcoma and osteosarcoma are aggressive high-grade malignancies that often arise in adolescents and young adults. Managing these malignancies in patients in this age bracket poses various clinical problems, also because different therapeutic approaches are sometimes adopted by pediatric and adult oncologists, even though they are dealing with the same condition...
2016: Progress in Tumor Research
https://www.readbyqxmd.com/read/27591237/evaluation-of-minimal-disseminated-disease-in-cryopreserved-ovarian-tissue-from-bone-and-soft-tissue-sarcoma-patients
#10
M M Dolmans, Y Iwahara, J Donnez, M Soares, J L Vaerman, C A Amorim, H Poirel
STUDY QUESTION: What is the risk of finding malignant cells in cryopreserved ovarian tissue from sarcoma patients? SUMMARY ANSWER: Minimal disseminated disease (MDD) was not detected in frozen-thawed ovarian tissue from 26 patients by any of the sensitive methods applied. WHAT IS KNOWN ALREADY: In case of leukemia, the risk of malignant cell transmission through the graft is well known and widely documented. However, for bone cancer, like Ewing sarcoma or osteosarcoma, only a small number of case reports, have been published...
October 2016: Human Reproduction
https://www.readbyqxmd.com/read/27486986/mirna-193a-5p-repression-of-p73-controls-cisplatin-chemoresistance-in-primary-bone-tumors
#11
Camille Jacques, Lidia Rodriguez Calleja, Marc Baud'huin, Thibaut Quillard, Dominique Heymann, François Lamoureux, Benjamin Ory
Osteosarcoma and Ewing Sarcoma are the two most common types of Bone Sarcomas, principally localized at the long bones of the extremities and mainly affecting adolescents and young adults. Cisplatin is one of the current options in the therapeutic arsenal of drugs available to cure these aggressive cancers. Unfortunately, chemoresistance against this agent is still a major cause of patient relapse. Thus, a better understanding of the molecular pathways by which these drugs induce cancer cell death, together with a better delineation of the origins of chemoresistance are required to improve the success rate of current treatments...
August 23, 2016: Oncotarget
https://www.readbyqxmd.com/read/27384727/functional-results-and-emotional-acceptance-after-scapulectomy-for-malignant-shoulder-tumors
#12
Song-Feng Xu, Xiu-Chun Yu, Ming Xu, Yong-Cheng Hu, Xiao-Ping Liu
OBJECTIVES: To demonstrate the functional results and emotional acceptance after scapulectomy for various malignant shoulder tumors. METHODS: Eight patients with malignant shoulder tumors who had undergone scapulectomy between April 2004 and March 2014 were retrospectively reviewed. They comprised seven men and one woman their mean age was 54 years (range, 24-69 years). All patients were diagnosed by pathological examination of biopsy specimens. The tumors were metastatic in four cases, having originated from a primary carcinoma of the liver in one patient, the lung in one patient and the kidney in two patients...
May 2016: Orthopaedic Surgery
https://www.readbyqxmd.com/read/27330312/synthetic-treatment-of-intracranial-peripheral-primitive-neuroectodermal-tumor-with-multiple-metastasis-a-case-report
#13
Yang Zhang, Hongsheng Li, Zongjuan Li, Ming Liu, Linke Yang, Liyuan Fan, Chengsuo Huang, Baosheng Li
Ewing sarcomas (ES) and peripheral primitive neuroectodermal tumors (pPNET) are now thought to belong to the same tumor family. Ewing sarcoma family tumor (ESFT) members commonly originate in bones and soft tissues. However, a few published articles describe ESFT arising from cranial cavities. Pathologically, ES/pPNET are composed of small round cells. Unambiguous distinction between pPNET and other small round cell tumors, in particular central PNET, is of clinical significance. Definitive diagnoses of pPNET can be obtained through CD99 (MIC2 gene product) membrane positivities and molecular identifications of chromosomal rearrangements between EWS and ETS family genes...
2016: OncoTargets and Therapy
https://www.readbyqxmd.com/read/27317769/ring1b-contributes-to-ewing-sarcoma-development-by-repressing-the-nav1-6-sodium-channel-and-the-nf-%C3%AE%C2%BAb-pathway-independently-of-the-fusion-oncoprotein
#14
Inmaculada Hernandez-Muñoz, Elisabeth Figuerola, Sara Sanchez-Molina, Eva Rodriguez, Ana Isabel Fernández-Mariño, Carlos Pardo-Pastor, María Isabel Bahamonde, José M Fernández-Fernández, Daniel J García-Domínguez, Lourdes Hontecillas-Prieto, Cinzia Lavarino, Angel M Carcaboso, Carmen de Torres, Oscar M Tirado, Enrique de Alava, Jaume Mora
Ewing sarcoma (ES) is an aggressive tumor defined by EWSR1 gene fusions that behave as an oncogene. Here we demonstrate that RING1B is highly expressed in primary ES tumors, and its expression is independent of the fusion oncogene. RING1B-depleted ES cells display an expression profile enriched in genes functionally involved in hematological development but RING1B depletion does not induce cellular differentiation. In ES cells, RING1B directly binds the SCN8A sodium channel promoter and its depletion results in enhanced Nav1...
July 19, 2016: Oncotarget
https://www.readbyqxmd.com/read/27308095/primary-pericranial-ewing-s-sarcoma-on-the-temporal-bone-a-case-report
#15
Hiroto Kawano, Naoki Nitta, Mitsuaki Ishida, Tadateru Fukami, Kazuhiko Nozaki
BACKGROUND: Primary Ewing's sarcoma originating in the pericranium is an extremely rare disease entity. CASE DESCRIPTION: A 9-year-old female patient was admitted to our department due to a left temporal subcutaneous mass. The mass was localized under the left temporal muscle and attached to the surface of the temporal bone. Head computed tomography revealed a mass with bony spicule formation on the temporal bone, however, it did not show bone destruction or intracranial invasion...
2016: Surgical Neurology International
https://www.readbyqxmd.com/read/27297500/comparison-of-clinical-features-and-outcomes-in-patients-with-extraskeletal-versus-skeletal-localized-ewing-sarcoma-a-report-from-the-children-s-oncology-group
#16
Thomas Cash, Elizabeth McIlvaine, Mark D Krailo, Stephen L Lessnick, Elizabeth R Lawlor, Nadia Laack, Joel Sorger, Neyssa Marina, Holcombe E Grier, Linda Granowetter, Richard B Womer, Steven G DuBois
BACKGROUND: The prognostic significance of having extraskeletal (EES) versus skeletal Ewing sarcoma (ES) in the setting of modern chemotherapy protocols is unknown. The purpose of this study was to compare the clinical characteristics, biologic features, and outcomes for patients with EES and skeletal ES. METHODS: Patients had localized ES and were treated on two consecutive protocols using five-drug chemotherapy (INT-0154 and AEWS0031). Patients were analyzed based on having an extraskeletal (n = 213) or skeletal (n = 826) site of tumor origin...
October 2016: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/27228320/bcor-ccnb3-positive-soft-tissue-sarcoma-with-round-cell-and-spindle-cell-histology-a-series-of-four-cases-highlighting-the-pitfall-of-mimicking-poorly-differentiated-synovial-sarcoma
#17
Wan-Shan Li, I-Chuang Liao, Mei-Chin Wen, Howard Haw-Chang Lan, Shih-Chen Yu, Hsuan-Ying Huang
AIMS: BCOR-CCNB3 sarcoma is a genetically defined undifferentiated malignancy with Ewing sarcoma (ES)-like round cells, and preferentially affects the bones of male adolescents. Sarcomas harbouring BCOR-CCNB3 rarely arise from soft tissues; therefore, we aimed to report four cases to expand the clinicopathological spectrum. METHODS AND RESULTS: By reverse transcription polymerase chain reaction and confirmatory sequencing, we detected a BCOR-CCNB3 transcript in primary undifferentiated sarcomas of the deep musculature of four male patients, comprising two teenagers (aged 14 and 17 years) and two adults (aged 34 and 44 years)...
November 2016: Histopathology
https://www.readbyqxmd.com/read/27190820/blue-cell-tumour-at-unusual-site-retropritoneal-ewings-sarcoma
#18
Anita P Javalgi, Mahesh H Karigoudar, Katyayani Palur
Ewing's sarcoma is a highly malignant tumour of osseous or non-osseous origin, tremed as extra-skeletal Ewings sarcoma if arising from soft tissue. It is rare occurrence tumor most commonly occurring in paravertebral area, chest wall, head & neck and retroperitoneum. Reporting an interesting case of retroperitoneal Ewing's sarcoma in 39 years old female. Patient had complains of abdominal discomfort & vague pain since 2 months, following weakness in lower limb and loss of weight. On detail history and examination she was further referred to detail pathological and radiological investigations...
April 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/27186040/extraosseous-ewing-sarcoma-diagnosis-prognosis-and-optimal-management
#19
REVIEW
George Galyfos, Georgios A Karantzikos, Nikolaos Kavouras, Argiri Sianou, Konstantinos Palogos, Konstantinos Filis
Extraosseous Ewing sarcomas (EESs) are rare tumours originating from soft tissues. Their clinical picture depends mainly on the primary site of the sarcoma. Patient characteristics and outcomes seem to be different in EES compared to patients with skeletal Ewing sarcoma, with implications for patient care and prognosis. However, multimodality therapeutic strategies are recommended for all types of the Ewing tumour family. The available diagnostic tools include ultrasonographic evaluation and computed tomography (CT) or magnetic resonance imaging as well as histopathologic and immunohistochemical tissue examination...
February 2016: Indian Journal of Surgery
https://www.readbyqxmd.com/read/26962160/chemotherapy-for-bone-sarcoma-in-adults
#20
REVIEW
Michael J Wagner, J Andrew Livingston, Shreyaskumar R Patel, Robert S Benjamin
The largest studies of chemotherapy for bone sarcomas are in the pediatric population. Although increasing age is often found to be an adverse prognostic factor in these clinical trials, few studies are aimed at assessing regimens specifically in the adult population. Osteosarcoma and Ewing sarcoma have peak incidences in the pediatric and young adult population but also occur in adults. Chondrosarcoma and giant cell tumor of bone are generally found in adults. In this review, we describe the current status of our knowledge about treating adults with cancers of bone origin...
March 2016: Journal of Oncology Practice
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