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Ewing sarcoma origination

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https://www.readbyqxmd.com/read/28508996/hyalinizing-clear-cell-carcinoma-of-the-bronchus
#1
Maryam Shahi, Michelle Dolan, Paari Murugan
Hyalinizing clear cell carcinoma (HCCC) is an uncommon low-grade minor salivary gland neoplasm that usually arises in the head and neck region. We report a 55-year-old man who presented with a 2.5 cm lung mass that was partially obstructing the right bronchus intermedius. The tumor consisted of cords and nests of clear and eosinophilic cells in a hyalinized stromal background. The neoplastic cells expressed cytokeratin (CK) 7, CK 5/6, high-molecular weight cytokeratin (34BE12), p63 and p40, while TTF-1, napsin A, CK20, S100, smooth muscle actin, synaptophysin and chromogranin were negative...
May 16, 2017: Head and Neck Pathology
https://www.readbyqxmd.com/read/28474974/primary-spinal-epidural-cic-dux4-undifferentiated-sarcoma-in-a-child
#2
John E Donahue, Evgeny Yakirevich, Shan Zhong, Diana O Treaba, Nelli S Lakis, Siraj M Ali, Suzanne M de la Monte, Shamlal Mangray
Primitive round- or spindle-cell EWSR1-negative undifferentiated sarcomas harboring CIC-DUX4 gene fusion are the most common form of Ewing-like sarcomas. These tumors primarily occur in peripheral soft tissues, but examples have been described within viscera and the brain. As far as we are aware, CIC-DUX4 positive primary epidural spinal sarcoma has not been reported. Herein, we describe a T5-T6 epidural tumor in a 15-year-old girl in which many neoplastic cells had moderate and focally abundant cytoplasm, including plasmacytoid or rhabdoid cells, rather than the more common Ewing-like morphology described in the majority of such tumors...
January 1, 2017: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/28472564/entering-the-double-digits-ewing-sarcoma-in-adult-maxilla
#3
Mala Kamboj, Anju Devi, Virendra Singh, Sunita Singh
Ewing sarcoma is a rare malignant pediatric sarcoma of bone and soft tissues, which is more unusual in head and neck region. Although the exact histogenesis is still unknown, various cells have been proposed as cells of origin namely, endothelial, hematopoietic, fibroblastic, mesenchymal stem cells or neural derived mesenchymal stem cells. ES is more common in younger age group and among the gnathic bones, mandible is affected more than maxilla. This paper presents the first case of Ewing Sarcoma in the maxilla being reported in the adult age group from India and among the very few reported worldwide...
May 2017: Journal of Experimental Therapeutics & Oncology
https://www.readbyqxmd.com/read/28293503/near-complete-humerus-reconstruction-in-the-pediatric-patient-with-vascularized-free-fibula-transfer
#4
John Shuck, Benjamin C Wood, Christopher Zarella, Albert K Oh, Robert M Henshaw, Gary F Rogers
Vascularized free fibula transfer remains the gold standard for reconstruction of large segmental diaphyseal defects of the upper extremity. In the pediatric patient, before skeletal maturity, free fibula transfer with the fibular head provides an active physis for growth and an articular interface for glenohumeral joint reconstruction. Clinical and cadaveric studies have demonstrated that the vascular supply to the fibular head originates, in most cases, from the anterior tibial system. However, anatomical variation exists, and we report a case in which a vascularized fibula autograft including the physis was transferred on the peroneal artery in a 5-year-old patient with Ewing's sarcoma...
December 2016: Plastic and Reconstructive Surgery. Global Open
https://www.readbyqxmd.com/read/28289810/non-odontogenic-tumors-of-the-facial-bones-in-children-and-adolescents-role-of-multiparametric-imaging
#5
REVIEW
Minerva Becker, Salvatore Stefanelli, Anne-Laure Rougemont, Pierre Alexandre Poletti, Laura Merlini
Tumors of the pediatric facial skeleton represent a major challenge in clinical practice because they can lead to functional impairment, facial deformation, and long-term disfigurement. Their treatment often requires a multidisciplinary approach, and radiologists play a pivotal role in the diagnosis and management of these lesions. Although rare, pediatric tumors arising in the facial bones comprise a wide spectrum of benign and malignant lesions of osteogenic, fibrogenic, hematopoietic, neurogenic, or epithelial origin...
April 2017: Neuroradiology
https://www.readbyqxmd.com/read/28129857/discovering-novel-oncogenic-pathways-and-new-therapies-using-zebrafish-models-of-sarcoma
#6
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
#7
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
#8
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
#9
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...
February 2017: Pathology, Research and Practice
https://www.readbyqxmd.com/read/27898190/primary-ewing-s-sarcoma-of-the-sinonasal-tract-in-adults-a-challenging-disease
#10
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
#11
REVIEW
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
#12
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
#13
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
#14
REVIEW
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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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