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Ewing sarcoma

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https://www.readbyqxmd.com/read/29227121/-what%C3%A2-s%C3%A2-new-in-ewing-like-sarcoma-family-soft-tissue-and-bone-sarcomas-with-cic-bcor-rearrangement-review-of-the-literature-and-first-personal-experience
#1
Zdeněk Kinkor, Petr Grossmann, Magdaléna Dubová, David Bludovský, Andrea Černá, Lenka Krsková, Petr Lhoták
The literature is reviewed regarding of a rare molecularly defined group of sarcomas with rearrangement of both CIC and BCOR genes, which were originally placed into the EWSR1wt Ewing-like category. Personal experience with three cases demonstrating difficulties of this issue is added. Both groups of lesions differ not only by age and topography, but also vary in both the prognostic and the predictive parameters. CIC-rearranged tumors are very aggressive and almost never occur in the skeleton; in contrary, the BCOR-rearranged ones are predominantly bone tumors in young males behaving even better than classical Ewing sarcoma...
2017: Ceskoslovenská Patologie
https://www.readbyqxmd.com/read/29225486/desmoplastic-small-round-blue-cell-tumor-a-review-of-treatment-and-potential-therapeutic-genomic-alterations
#2
REVIEW
Ajaz Bulbul, Bridget Noel Fahy, Joanne Xiu, Sadaf Rashad, Asrar Mustafa, Hatim Husain, Andrea Hayes-Jordan
Desmoplastic small round blue cell tumors (DSRCTs) originate from a cell with multilineage potential. A molecular hallmark of DSRCT is the EWS-WT1 reciprocal translocation. Ewing sarcoma and DSRCT are treated similarly due to similar oncogene activation pathways, and DSRCT has been represented in very limited numbers in sarcoma studies. Despite aggressive therapy, median survival ranges from 17 to 25 months, and 5-year survival rates remain around 15%, with higher survival reported among those undergoing removal of at least 90% of tumor in the absence of extraperitoneal metastasis...
2017: Sarcoma
https://www.readbyqxmd.com/read/29221844/gabapentin-as-an-adjuvant-therapy-for-prevention-of-acute-phantom-limb-pain-in-pediatric-patients-undergoing-amputation-for-malignant-bone-tumors-a-prospective-double-blind-randomized-controlled-trial
#3
Xiandi Wang, Ying Yi, Dingbo Tang, Yi Chen, Yanhua Jiang, Jun Peng, Jiwei Xiao
CONTEXT: Gabapentin is reported to have an analgesic effect of reducing phantom-limb pain(PLP) in adult patients. There's no study on preoperative use of gabapentin in pediatric population in term of PLP prevention. OBJECTIVE: To determine whether gabapentin could be used as an adjuvant agent of opioid-based pain control to lower the rate of phantom-limb pain in pediatric patients undergoing amputation for malignant bone tumors in observation period of 60 days post-operatively...
December 5, 2017: Journal of Pain and Symptom Management
https://www.readbyqxmd.com/read/29220292/rhabdomyosarcoma-ewing-sarcoma-and-other-round-cell-sarcomas
#4
Alberto S Pappo, Uta Dirksen
Several recent advances have been made in the diagnosis and therapy of malignant small round cell tumors that affect children, particularly in rhabdomyosarcoma, Ewing sarcoma, and other round cell sarcomas. These advances have provided new insights into the pathologic, histologic, and genomic characterization of specific tumor subtypes, which has led to the identification of novel therapeutic targets and improved stratification of risk. This has, in turn, led to improved efficacy in clinical trials of new drug combinations, thereby increasing the survival of patients with newly diagnosed and refractory or recurrent round cell sarcomas...
December 8, 2017: Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology
https://www.readbyqxmd.com/read/29219959/primary-orbital-ewing-sarcoma-family-of-tumors-a-study-of-12-cases
#5
S Kaliki, S G Rathi, V A R Palkonda
PurposeThe purpose of this study is to discuss the clinical presentation, management, and outcomes of patients with primary orbital Ewing sarcoma family of tumors (ESFTs).Patients and methodsRetrospective study of 12 patients with biopsy-proven primary orbital ESFT.ResultsThe mean age at presentation of primary orbital ESFT was 12 years (median, 8 years; range, 5 months to 28 years). There were seven (58%) females and five (42%) males. The presenting complaints included proptosis (n=10; 83%) and swelling in the upper eyelid (n=2; 17%)...
December 8, 2017: Eye
https://www.readbyqxmd.com/read/29219854/reconstruction-of-the-scapula-in-pediatric-and-adolescent-patients-after-total-scapulectomy-a-report-of-10-patients-treated-by-extracorporeal-irradiation-and-reimplantation-of-the-scapula
#6
Ahmed M El Ghoneimy, Mohamed S Zaghloul, Iman Zaky, Hala Taha, Ahmed Elgammal, Magdy El Sherbiny, Nehal Kamal
BACKGROUND: Ewing sarcoma of the scapula is uncommon. Restoration of shoulder function after total scapulectomy is challenging. Extracorporeal irradiation and reimplantation is a well-known method of biological reconstruction in orthopaedic oncology; yet very few reports in the literature describe its specific use in tumors of the scapula. PATIENTS AND METHODS: Ten patients with the diagnosis of Ewing sarcoma of the scapula were surgically treated by total scapulectomy and extracorporeal irradiation and reimplantation of the scapula...
December 6, 2017: Journal of Pediatric Orthopedics
https://www.readbyqxmd.com/read/29207714/paediatric-peripheral-primitive-neuroectodermal-tumour-a-clinico-pathological-study-from-southern-india
#7
Rithika Rajendran, Leena Dennis Joseph, Thanka Johnson, Latha Magatha Sneha, Julius Xavier Scott, Satish Srinivasan
Introduction: Primitive Neuroectodermal Tumour (PNET)/Ewing Sarcomas (ES) are aggressive childhood malignancies with neuroectodermal differentiation. Aim: To study the clinical presentation, morphology, Immun-ohistochemistry (IHC), management and outcome of all the cases of paediatric pPNET/ES reported in our tertiary care centre over a period of six years. Materials and Methods: This was a retrospective study conducted at Sri Ramachandra Medical College and Research Institute, Chennai, India...
September 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/29204207/primary-ewing-s-sarcoma-of-cranium-in-a-pediatric-patient
#8
Ashutosh Das Sharma, Jayesh Singh, Jigna Bhattacharya
Ewing's sarcoma primarily involving the skull is an unusual occurrence (1% of all Ewing's sarcoma cases) with very few reported cases in the published literature. The challenge in such cases is to establish a definitive diagnosis before starting the multidisciplinary treatment approach. We report such a rare case in a 13-year-old boy who is being treated at our institute with an intention of adding to the limited information we currently have on diagnosis, management, and the outcome of such cases.
July 2017: Journal of Pediatric Neurosciences
https://www.readbyqxmd.com/read/29200689/acute-lymphoblastic-leukemia-as-secondary-malignancy-in-a-case-of-ewing-s-sarcoma-on-treatment
#9
Satyam Satyarth, Sonia Parikh, Asha Anand, Jyoti Sawhney, Harsha Panchal, Apurva Patel, Sandeep Shah
The survival of Ewing's sarcoma (ES) has improved due to advances in both local and systemic therapy. This has given rise to an increased detection of second malignant neoplasms which can be in the form of solid tumors and hematological malignancies. The most common hematological malignancies are acute myeloid leukemia/myelodysplastic syndrome. Acute lymphoblastic leukemia (ALL) is relatively uncommon in occurrence in this setting. Furthermore, the average refractory period for hematological malignancies varies from 3 to 5 years...
July 2017: Indian Journal of Medical and Paediatric Oncology
https://www.readbyqxmd.com/read/29200681/clinicopathological-features-and-treatment-outcomes-in-ewing-s-sarcoma-patients-a-10-year-experience-of-alexandria-clinical-oncology-department
#10
Asmaa Nazeer, Alaa Kandil, Omar Zahra, Maher Soliman
Background: Ewing's sarcoma (ES) is an aggressive tumor, which is usually associated with micrometastases in the circulation. Thus, systemic chemotherapy in addition to local control modality is essential to improve outcomes. The aim of this study was to evaluate clinicopathological features and treatment outcomes in patients with ES. Materials and Methods: Medical files of 74 patients with nonmetastatic ES treated at our centers between 2004 and 2014 were retrospectively evaluated...
July 2017: Indian Journal of Medical and Paediatric Oncology
https://www.readbyqxmd.com/read/29183875/navigating-chronic-pain-how-taking-a-few-wrong-turns-can-miss-sinister-pathology
#11
Jennifer Turnbull, Richard Dodds, Andy Wainwright, Athimalaipet Vaidyanathan Ramanan, Kate Wheeler
Bone sarcomas are rare in childhood, and their presentation can often mimic more benign complaints or chronic musculoskeletal pain. Ewing sarcomas in particular are often diagnosed after a significant delay from the onset of symptoms. At a population level, a long diagnostic delay is not necessarily associated with worse survival , as tumours that display slow growth also tend to be less aggressive. For any specific individual however, a delayed diagnosis can result in a larger tumour that is more difficult to treat...
November 28, 2017: Archives of Disease in Childhood. Education and Practice Edition
https://www.readbyqxmd.com/read/29178401/childhood-cancer-incidence-and-survival-in-japan-and-england-a-population-based-study-1993-2010
#12
Kayo Nakata, Yuri Ito, Winnie Magadi, Audrey Bonaventure, Charles A Stiller, Kota Katanoda, Tomohiro Matsuda, Isao Miyashiro, Kathy Pritchard-Jones, Bernard Rachet
This study aimed to compare cancer incidence and trends in survival for children diagnosed in Japan and England, using population-based cancer registry data. The analysis was based on 5,192 children with cancer (age 0-14 years) from six prefectural cancer registries in Japan and 21,295 children diagnosed in England during 1993-2010. Differences in incidence rates between the two countries were measured with Poisson regression models. Overall survival was estimated using the Kaplan-Meier method. Incidence rates for Hodgkin lymphoma, renal tumours, and Ewing sarcomas in England were more than twice as high as those in Japan...
November 24, 2017: Cancer Science
https://www.readbyqxmd.com/read/29177593/practical-recommendations-for-fertility-preservation-in-women-by-the-fertiprotekt-network-part-i-indications-for-fertility-preservation
#13
A N Schüring, T Fehm, K Behringer, M Goeckenjan, P Wimberger, M Henes, J Henes, M F Fey, M von Wolff
PURPOSE: Most guidelines about fertility preservation are predominantly focused on scientific evidence, but are less practically orientated. Therefore, practically oriented recommendations are needed to support the clinician in daily practice. METHODS: A selective literature search was performed based on the clinical and scientific experience of the authors, focussing on the most relevant diseases and gynaecological cancers. This article (Part I) provides information on topics that are essential for the fertility preservation indication, such as disease prognosis, disease therapy and its associated risks to fertility, recommending disease-specific fertility preservation measures...
November 24, 2017: Archives of Gynecology and Obstetrics
https://www.readbyqxmd.com/read/29176463/primary-ewing-sarcoma-of-the-mastoid-a-novel-case-mimicking-acute-mastoiditis
#14
Grace Egan, Joanna Pierro, Pillai Pallavi Madhusoodhan, Ghulam Ilyas, Benjamin Cohen, Teena Bhatla
Ewing sarcoma (EWS) is a primitive neuroectodermal tumor arising in bone or soft tissue. It is the second most common primary bone malignancy of children and adolescents, with a peak incidence in the second decade of life. It most often arises in the long bones of the extremities and pelvis. Here, we present a novel case of EWS arising from the mastoid bone in a 5-year-old African American male who presented with symptoms of acute mastoiditis. This unique presentation highlights the importance of considering EWS in a patient who presents with atypical mastoiditis or a rapidly growing mass in the postauricular region...
November 23, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29170560/clinicopathological-correlation-of-tumor-associated-macrophages-in-ewing-sarcoma
#15
Marek Handl, Marketa Hermanova, Sylva Hotarkova, Jiri Jarkovsky, Peter Mudry, Tetiana Shatokhina, Marcela Vesela, Jaroslav Sterba, Iva Zambo
AIMS: Tumor-associated macrophages (TAMs) are known markers playing complex roles in tumorigenesis. However, the function of TAMs in a variety of malignancies is not yet fully understood. The aim of this pilot study was to quantify the density of TAMs in Ewing sarcoma and to determine the correlation between TAMs and clinicopathological parameters. METHODS: Using immunohistochemistry, the expressions of CD68 and CD163 were examined in 24 tissue samples of Ewing sarcoma of bone...
November 21, 2017: Biomedical Papers of the Medical Faculty of the University Palacký, Olomouc, Czechoslovakia
https://www.readbyqxmd.com/read/29168368/a-congenital-soft-tissue-ewing-sarcoma-in-a-newborn-patient
#16
Mustafa Akçalı, Hacer Yapıcıoğlu, Eray Akay, Ferda Özlü, Banu Kozanoğlu, Kıvılcım Erdoğan, Gülfiliz Gönlüşen, Mehmet Satar
Akçalı M, Yapıcıoğlu H, Akay E, Özlü F, Kozanoğlu B, Erdoğan K, Gönlüşen G, Satar M. A congenital soft tissue Ewing sarcoma in a newborn patient. Turk J Pediatr 2017; 59: 76-79. < p < Congenital Ewing sarcoma is extremely rare. Here we present a newborn baby born with a mass on the left shoulder. Immunohistochemical staining showed congenital Ewing sarcoma. Chemotherapy and then surgical operation were planned, however the patient died before initiation of chemotherapy on the 30th day of life...
2017: Turkish Journal of Pediatrics
https://www.readbyqxmd.com/read/29168356/single-institution-experience-on-cancer-among-adolescents-15-19-years-of-age
#17
Begül Yağcı-Küpeli, Canan Akyüz, Bilgehan Yalçın, Ali Varan, Tezer Kutluk, Münevver Büyükpamukçu
Yağcı-Küpeli B, Akyüz C, Yalçın B, Varan A, Kutluk T, Büyükpamukçu M. Single institution experience on cancer among adolescents 15-19 years of age. Turk J Pediatr 2017; 59: 1-5. Adolescent cancers differ from other age groups in terms of cancer types, treatment and outcome. We aimed to present our institutional data on survival of certain types of cancer in adolescents. Hospital files were retrospectively evaluated for distribution of tumor types, clinical features, and overall and event-free survival (OS and EFS) rates in adolescents with malignant tumors...
2017: Turkish Journal of Pediatrics
https://www.readbyqxmd.com/read/29163353/primary-ewing-sarcoma-of-the-thyroid-eight-cases-in-a-decade-a-case-report-and-literature-review
#18
Paweł Kabata, Sonia Kaniuka-Jakubowska, Wanda Kabata, Joanna Lakomy, Wojciech Biernat, Krzysztof Sworczak, Janusz Jaśkiewicz, Maciej Świerblewski
Sarcomas represent less than 1% of all malignant tumors found in the thyroid. Of these, primary extraosseoussarcoma has been reported only a few times in the past decade. We present the case of a 34-year-old male who had a fast-growing hard mass in the lower left neck. FNA was inconclusive. Core needle biopsy revealed the diagnosis of an Ewing sarcoma/primitive neuroectodermal tumor. Mutation of EWSR1 was confirmed using the FISH method. Following treatment by neoadjuvant chemotherapy, we observed clinical, radiological, and finally histopathological remission...
2017: Frontiers in Endocrinology
https://www.readbyqxmd.com/read/29152060/inhibition-of-chk1-sensitizes-ewing-sarcoma-cells-to-the-ribonucleotide-reductase-inhibitor-gemcitabine
#19
Kelli L Goss, Stacia L Koppenhafer, Kathryn M Harmoney, William W Terry, David J Gordon
Ewing sarcoma is a bone and soft tissue sarcoma that occurs in children and young adults. The EWS-FLI1 gene fusion is the driver mutation in most Ewing sarcoma tumors and functions, in part, as an aberrant transcription factor. We recently identified that Ewing sarcoma cells are sensitive to inhibition of ribonucleotide reductase (RNR), which catalyzes the formation of deoxyribonucleotides from ribonucleotides. In this report, we show that Ewing sarcoma cells are sensitive to treatment with clofarabine, which is a nucleoside analogue and allosteric inhibitor of RNR...
October 20, 2017: Oncotarget
https://www.readbyqxmd.com/read/29150734/the-sirtuin-1-2-inhibitor-tenovin-1-induces-a-nonlinear-apoptosis-inducing-factor-dependent-cell-death-in-a-p53-null-ewing-s-sarcoma-cell-line
#20
Christian Marx, Lisa Marx-Blümel, Nora Lindig, René Thierbach, Doerte Hoelzer, Sabine Becker, Susan Wittig, Roland Lehmann, Hortense Slevogt, Thorsten Heinzel, Zhao-Qi Wang, James F Beck, Jürgen Sonnemann
The sirtuin 1/2 inhibitor tenovin-1 activates p53 and may have potential in the management of cancer. Here, we investigated the responsiveness of Ewing's sarcoma cells to tenovin-1. We examined its effects in two Ewing's sarcoma cell lines with different p53 status, i.e. in p53 wild-type and p53 null cells. Effects were assessed by flow cytometric analyses of cell death, mitochondrial membrane depolarization and reactive oxygen species (ROS) generation, by caspase 3/7 activity measurement, by mRNA expression profiling and by immunoblotting...
November 18, 2017: Investigational New Drugs
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