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https://www.readbyqxmd.com/read/29352211/hdl-nanoparticles-targeting-sonic-hedgehog-subtype-medulloblastoma
#1
Jonathan B Bell, Jonathan S Rink, Frank Eckerdt, Jessica Clymer, Stewart Goldman, C Shad Thaxton, Leonidas C Platanias
Medulloblastoma is the most common paediatric malignant brain cancer and there is a need for new targeted therapeutic approaches to more effectively treat these malignant tumours, which can be divided into four molecular subtypes. Here, we focus on targeting sonic hedgehog (SHH) subtype medulloblastoma, which accounts for approximately 25% of all cases. The SHH subtype relies upon cholesterol signalling for tumour growth and maintenance of tumour-initiating cancer stem cells (CSCs). To target cholesterol signalling, we employed biomimetic high-density lipoprotein nanoparticles (HDL NPs) which bind to the HDL receptor, scavenger receptor type B-1 (SCARB1), depriving cells of natural HDL and their cholesterol cargo...
January 19, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29340997/immunohistochemical-biomarkers-of-mesenchymal-neoplasms-in-endocrine-organs-diagnostic-pitfalls-and-recent-discoveries
#2
Yin P Hung, Jason L Hornick
Mesenchymal neoplasms rarely present in or adjacent to endocrine organs. In this context, the recognition of these rare tumor types can be challenging, with significant potential for misdiagnosis as sarcomatoid carcinomas (i.e., anaplastic thyroid carcinoma and sarcomatoid adrenal cortical carcinoma) or neuroendocrine carcinomas, depending upon the dominant histologic patterns. In this review, we address potential pitfalls in diagnosing selected mesenchymal neoplasms arising within or near endocrine organs, including dedifferentiated liposarcoma, synovial sarcoma, angiosarcoma, PEComa, proximal-type epithelioid sarcoma, Ewing sarcoma, and neuroblastoma...
January 16, 2018: Endocrine Pathology
https://www.readbyqxmd.com/read/29335623/sinusoidal-obstruction-syndrome-veno-occlusive-disease-after-high-dose-intravenous-busulfan-melphalan-conditioning-therapy-in-high-risk-ewing-sarcoma
#3
Massimo Eraldo Abate, Anna Paioli, Sivlia Cammelli, Marilena Cesari, Alessandra Longhi, Emanuela Palmerini, Stefano Ferrari, Elisa Carretta, Piero Picci, Fabio Piscaglia
This mono-institutional observational study was conducted to determine incidence, severity, risk factors, and outcome of sinusoidal obstruction syndrome/veno-occlusive disease (SOS/VOD) in high-risk Ewing sarcoma (ES) patients treated with intravenous busulfan and melphalan (BU-MEL) followed by autologous stem cell transplantation (ASCT). During the past 10 years, 75 consecutive ES patients resulted evaluable for the analysis. After diagnosis of SOS/VOD, defibrotide therapy was started as soon as the medication was available...
January 15, 2018: Bone Marrow Transplantation
https://www.readbyqxmd.com/read/29333027/ewing-s-sarcoma-of-the-calcaneum
#4
P Ajmal Sherif, A Santa
Ewing's sarcoma of the calcaneum is rare. Radiological features of this tumor can be misinterpreted as other benign bone tumors due to its rarity. The overall prognosis of Ewing's sarcoma of calcaneum is inferior compared to other sites of this tumor. Hence, these tumors should have extensive radiological evaluation and histological confirmation as misdiagnosis and treatment delays will have detrimental outcomes.
October 2017: Indian Journal of Medical and Paediatric Oncology
https://www.readbyqxmd.com/read/29333015/a-profile-of-pediatric-solid-tumors-a-single-institution-experience-in-kashmir
#5
Namita Sharma, Ayesha Ahmad, Gull M Bhat, Sheikh A Aziz, Mohammad Maqbool Lone, Nisar A Bhat
Aims: The purpose of this retroprospective study was to study the epidemiological characteristics and outcomes of children with solid tumors at our institution. Subjects and Methods: Three hundred and three pediatrics patients registered at Regional Cancer Centre (RCC), Sher-i-Kashmir Institute of Medical Sciences (SKIMS), Srinagar, Kashmir, between January 2008 and June 2014, were analyzed with regard to demographic status, presenting complaints, investigations, treatment, morbidity, and outcomes...
October 2017: Indian Journal of Medical and Paediatric Oncology
https://www.readbyqxmd.com/read/29328472/combined-analysis-of-gene-expression-and-genome-binding-profiles-identified-potential-therapeutic-targets-of-ciclopirox-in-ewing-sarcoma
#6
Baisheng Yuan, Wei Ji, Haipeng Xia, Jianmin Li
Ciclopirox (CPX) is a synthetic antifungal drug that is mainly used to treat dermatomycoses. The aim of the present study was to determine whether CPX could influence Ewing sarcoma progression. The present study suggested that CPX treatment may inhibit Ewing sarcoma (ES) progression through Ewing sarcoma breakpoint region 1‑Friend leukemia integration 1 (EWS‑FLI1), a common fusion transcript structure in patients with ES. To determine the underlying mechanisms of ES progression, cross analysis was conducted on three high‑throughput genome or transcript me datasets from the Gene Expression Omnibus...
January 10, 2018: Molecular Medicine Reports
https://www.readbyqxmd.com/read/29327709/insm1-expression-and-its-diagnostic-significance-in-extraskeletal-myxoid-chondrosarcoma
#7
Akihiko Yoshida, Naohiro Makise, Susumu Wakai, Akira Kawai, Nobuyoshi Hiraoka
Extraskeletal myxoid chondrosarcoma is a rare subtype of sarcoma that affects the soft tissue and bones in middle-aged and elderly adults. Its diagnosis can be challenging, with the differential diagnoses including a wide variety of mesenchymal tumors. The line of differentiation of extraskeletal myxoid chondrosarcoma has been controversial, but recent evidence suggests a neuroendocrine phenotype. INSM1 is a zinc-finger transcription factor that plays a pivotal role in neuroendocrine differentiation, and has been proposed as a promising immunohistochemical marker of neuroendocrine carcinoma...
January 12, 2018: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
https://www.readbyqxmd.com/read/29327554/-ewing-s-sarcoma-misdiagnosed-as-nonossifying-fibroma-a-case-report
#8
Hong-Tao Li, Dong-Dong Li, Xiao-di Yang, Yong-Zhi Yang, Gui-Shan Gu
No abstract text is available yet for this article.
January 25, 2017: Zhongguo Gu Shang, China Journal of Orthopaedics and Traumatology
https://www.readbyqxmd.com/read/29321818/ews-fli-1-creates-a-cell-surface-microenvironment-conducive-to-igf-signaling-by-inducing-pappalysin-1
#9
Panneerselvam Jayabal, Peter J Houghton, Yuzuru Shiio
Ewing sarcoma is an aggressive cancer of bone and soft tissue in children with poor prognosis. It is characterized by the chromosomal translocation between EWS and an Ets family transcription factor, most commonly FLI-1. EWS-FLI-1 fusion accounts for 85% of Ewing sarcoma cases. EWS-FLI-1 regulates the expression of a number of genes important for sarcomagenesis, can transform NIH3T3 and C3H10T1/2 cells, and is necessary for proliferation and tumorigenicity of Ewing sarcoma cells, suggesting that EWS-FLI-1 is the causative oncoprotein...
November 2017: Genes & Cancer
https://www.readbyqxmd.com/read/29313488/replication-study-systematic-identification-of-genomic-markers-of-drug-sensitivity-in-cancer-cells
#10
John P Vanden Heuvel, Ewa Maddox, Samar W Maalouf, Elizabeth Iorns, Rachel Tsui, Alexandria Denis, Nicole Perfito, Timothy M Errington
In 2016, as part of the Reproducibility Project: Cancer Biology, we published a Registered Report (Vanden Heuvel et al., 2016), that described how we intended to replicate selected experiments from the paper 'Systematic identification of genomic markers of drug sensitivity in cancer cells' (Garnett et al., 2012). Here we report the results. We found Ewing's sarcoma cell lines, overall, were more sensitive to the PARP inhibitor olaparib than osteosarcoma cell lines; however, while the effect was in the same direction as the original study (Figure 4C; Garnett et al...
January 9, 2018: ELife
https://www.readbyqxmd.com/read/29307936/retroperitoneal-ewing-s-sarcoma-embryonal-tumor-a-rare-differential-diagnosis-of-back-pain
#11
Fabiano Reis, Eduardo Macedo, Marcondes Cavalcanti França Junior, Eliane Ingrid Amstalden, Simone Appenzeller
No abstract text is available yet for this article.
November 2017: Radiologia Brasileira
https://www.readbyqxmd.com/read/29305692/cd99-at-the-crossroads-of-physiology-and-pathology
#12
REVIEW
Michela Pasello, Maria Cristina Manara, Katia Scotlandi
CD99 is a cell surface protein with unique features and only partly defined mechanisms of action. This molecule is involved in crucial biological processes, including cell adhesion, migration, death, differentiation and diapedesis, and it influences processes associated with inflammation, immune responses and cancer. CD99 is frequently overexpressed in many types of tumors, particularly pediatric tumors including Ewing sarcoma and specific subtypes of leukemia. Engagement of CD99 induces the death of malignant cells through non-conventional mechanisms...
January 6, 2018: Journal of Cell Communication and Signaling
https://www.readbyqxmd.com/read/29300189/bcor-ccnb3-fusion-positive-sarcomas-a-clinicopathologic-and-molecular-analysis-of-36-cases-with-comparison-to-morphologic-spectrum-and-clinical-behavior-of-other-round-cell-sarcomas
#13
Yu-Chien Kao, Adepitan A Owosho, Yun-Shao Sung, Lei Zhang, Yumi Fujisawa, Jen-Chieh Lee, Leonard Wexler, Pedram Argani, David Swanson, Brendan C Dickson, Christopher D M Fletcher, Cristina R Antonescu
BCOR-CCNB3 sarcoma (BCS) is a recently defined genetic entity among undifferentiated round cell sarcomas, which was initially classified as and treated similarly to the Ewing sarcoma (ES) family of tumors. In contrast to ES, BCS shows consistent BCOR overexpression, and preliminary evidence suggests that these tumors share morphologic features with other tumors harboring BCOR genetic alterations, including BCOR internal tandem duplication (ITD) and BCOR-MAML3. To further investigate the pathologic features, clinical behavior, and their relationship to other round cell sarcomas, we collected 36 molecularly confirmed BCSs for a detailed histologic and immunohistochemical analysis...
October 25, 2017: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/29298256/planning-for-bone-excision-in-ewing-sarcoma-post-chemotherapy-mri-more-accurate-than-pre-chemotherapy-mri-assessment
#14
Camille Thévenin-Lemoine, Louise Destombes, Julie Vial, Matthieu Wargny, Paul Bonnevialle, Yan Lefevre, Anne Gomez Brouchet, Jérome Sales de Gauzy
BACKGROUND: In determining the level of bone resection in Ewing sarcoma, the most suitable time at which to perform magnetic resonance imaging (MRI) remains controversial. Current guidelines recommend that surgical planning be based on MRI performed prior to neoadjuvant chemotherapy. The goal of this study was to determine whether pre-chemotherapy or post-chemotherapy MRI provides greater accuracy of tumor limits for planning bone excision in the management of Ewing sarcoma. METHODS: This was a single-center, retrospective study...
January 3, 2018: Journal of Bone and Joint Surgery. American Volume
https://www.readbyqxmd.com/read/29287925/ewing-s-sarcoma-of-the-maxillofacial-region-in-greek-children-report-of-6-cases-and-literature-review
#15
Ioannis Iatrou, Nadia Theologie-Lygidakis, Ourania Schoinohoriti, Fotios Tzermpos, Anastassios I Mylonas
BACKGROUND: The rarity of Ewing's sarcoma (ES) in the maxillofacial region of children, coupled with the technical challenge of resection and associated functional and cosmetic impairment has resulted in deficient data regarding the optimal local control of the disease. OBJECTIVE: To describe our experience in the management of primary maxillofacial ES in children, focusing on the therapeutic modalities for local control of the disease. STUDY DESIGN: Single institution observational study...
December 16, 2017: Journal of Cranio-maxillo-facial Surgery
https://www.readbyqxmd.com/read/29285586/intracranial-ewing-sarcoma-four-pediatric-examples
#16
Michael J Yang, Ros Whelan, Jennifer Madden, Jean M Mulcahy Levy, B K Kleinschmidt-DeMasters, Todd C Hankinson, Nicholas K Foreman, Michael H Handler
BACKGROUND: Ewing sarcoma typically arises in bone and is unrelated to intraparenchymal small blue cell embryonal central nervous system (CNS) tumors previously designated primitive neuroectodermal tumors (PNETs). When the CNS is impacted, it is usually secondary to local extension from either the epidural space, skull, or intracranial or spinal metastases. Primary examples within the cranial vault are rare, usually dural-based, and are largely case reports in the literature. We detail four pediatric patients with solitary, primary intracranial Ewing sarcoma, all manifesting the archetypal EWRS1 gene rearrangement that confirms diagnosis...
December 28, 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/29279582/histopathological-diagnoses-in-soft-tissue-tumours-an-experience-from-a-tertiary-centre-in-malaysia
#17
N D Jalaludin, N Mohd Dusa, M R Hassan, N Abd Shukor
Soft tissue tumours are a group of remarkably diverse neoplasms that frequently pose significant diagnostic challenges to general pathologists. This study aimed to compare the agreement of histopathological diagnoses between general pathologists from various referral institutes and the referred soft tissue pathologist in a tertiary centre. The common discrepancies and their causes are also presented here. A retrospective study was conducted on 243 cases of potential soft tissue tumours referred to Hospital Kuala Lumpur, Malaysia over a period of 5 years...
December 2017: Malaysian Journal of Pathology
https://www.readbyqxmd.com/read/29278534/cohesin-mutations-in-myeloid-malignancies-made-simple
#18
Aaron D Viny, Ross L Levine
PURPOSE OF REVIEW: Recurrent loss of function mutations within genes of the cohesin complex have been identified in myelodysplastic syndrome (MDS) and acute myeloid leukemia (AML). STAG2 is the most commonly mutated cohesin member in AML as well as solid tumors. STAG2 is recurrently, mutated in Ewing's Sarcoma, bladder cancer, and glioblastoma, and is one of only ten genes known to be recurrently mutated in over four distinct tissue types of human cancer RECENT FINDINGS: The cohesin complex, a multiprotein ring, is canonically known to align and stabilize replicated chromosomes prior to cell division...
December 22, 2017: Current Opinion in Hematology
https://www.readbyqxmd.com/read/29273680/igf-system-in-sarcomas-a-crucial-pathway-with-many-unknowns-to-exploit-for-therapy
#19
Caterina Mancarella, Katia Scotlandi
The insulin-like growth factor (IGF) system has gained substantial interest due to its involvement in regulating cell proliferation, differentiation and survival during anoikis and after conventional and targeted therapies. However, results from clinical trials have been largely disappointing, with only a few but notable exceptions, such as trials targeting sarcomas, especially Ewing sarcoma. This review highlights key studies focusing on IGF signaling in sarcomas, specifically studies underscoring the properties that make this system an attractive therapeutic target, and identifies new relationships that may be exploited...
December 22, 2017: Journal of Molecular Endocrinology
https://www.readbyqxmd.com/read/29247978/advances-in-chromosomal-translocations-and-fusion-genes-in-sarcomas-and-potential-therapeutic-applications
#20
REVIEW
Xin Xiao, Cassandra C Garbutt, Francis Hornicek, Zheng Guo, Zhenfeng Duan
Chromosomal translocations and fusion genes are very common in human cancer especially in subtypes of sarcomas, such as rhabdomyosarcoma, Ewing's sarcoma, synovial sarcoma and liposarcoma. The discovery of novel chromosomal translocations and fusion genes in different tumors are due to the advancement of next-generation sequencing (NGS) technologies such as whole genome sequencing. Recently, many novel chromosomal translocations and gene fusions have been identified in different types of sarcoma through NGS approaches...
December 6, 2017: Cancer Treatment Reviews
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