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Ewing sarcoma

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https://www.readbyqxmd.com/read/28647886/wnt-signaling-in-ewing-sarcoma-osteosarcoma-and-malignant-peripheral-nerve-sheath-tumors
#1
REVIEW
Matthew G Pridgeon, Patrick J Grohar, Matthew R Steensma, Bart O Williams
PURPOSE OF REVIEW: Wnt signaling plays a central role in development and homeostasis, and its dysregulation is a common event in many types of human cancer. Here we explore in detail the contributions of Wnt signaling to the initiation and maintenance of three types of saroma: Ewing sarcoma, osteosarcoma, and malignant peripheral nerve sheath tumors. This review provides an overview of the Wnt signaling pathway and explores in detail the current knowledge about its role in the initiation or maintenance of three tumor types: Ewing sarcoma, osteosarcoma, and malignant peripheral nerve sheath tumors...
June 24, 2017: Current Osteoporosis Reports
https://www.readbyqxmd.com/read/28647781/computer-navigation-assisted-surgery-for-musculoskeletal-tumors-a-closer-look-into-the-learning-curve
#2
Kevin Staats, Joannis Panotopoulos, Thomas M Tiefenboeck, Reinhard Windhager, Philipp T Funovics
BACKGROUND: Computer navigation-assisted surgery for musculoskeletal tumors has shown to reduce the risk of intra-lesional margins in resection. Experiences with this method are still limited to smaller case series. METHOD: We reviewed our first experiences in 24 patients in whom computer navigation-assisted surgery had been performed. In 7 of these patients (6 male and 1 female), this has influenced the surgical treatment plan and navigation was used for both tumor resection and reconstruction...
June 24, 2017: European Journal of Orthopaedic Surgery & Traumatology: Orthopédie Traumatologie
https://www.readbyqxmd.com/read/28645808/primary-undifferentiated-small-round-cell-sarcoma-of-the-deep-abdominal-wall-with-a-novel-variant-of-t-10-19-cic-dux4-gene-fusion
#3
Yoshitane Tsukamoto, Hiroyuki Futani, Shinichi Yoshiya, Takahiro Watanabe, Takako Kihara, Shohei Matsuo, Seiichi Hirota
We experienced a 38-year-old Japanese male with t(10;19) CIC-DUX4 -positive undifferentiated small round cell sarcoma in the deep abdominal wall. Three months before his first visit to our hospital, he noticed a mass in his right abdominal wall. Computed tomography on admission revealed a solid abdominal tumor 70×53mm in size and multiple small tumors in both lungs. The biopsy of the abdominal tumor revealed undifferentiated small round cell sarcoma, suggestive of Ewing sarcoma. Under the clinical diagnosis of Ewing-like sarcoma of the abdominal wall with multiple lung metastases, several cycles of ICE (ifosfamide, carboplatin and etoposide) therapy were performed...
June 6, 2017: Pathology, Research and Practice
https://www.readbyqxmd.com/read/28643791/ewsr1-fusion-proteins-mediate-pax7-expression-in-ewing-sarcoma
#4
Gregory W Charville, Wei-Lien Wang, Davis R Ingram, Angshumoy Roy, Dafydd Thomas, Rajiv M Patel, Jason L Hornick, Matt van de Rijn, Alexander J Lazar
PAX7 is a paired-box transcription factor that is required for the developmental specification of adult skeletal muscle progenitors in mice. We previously demonstrated PAX7 expression as a marker of skeletal muscle differentiation in rhabdomyosarcoma. Here, using analyses of published whole-genome gene expression microarray data, we identify PAX7 as a gene with significantly increased expression in Ewing sarcoma in comparison to CIC-DUX4 round cell sarcoma. Analysis of PAX7 in a large cohort of 103 Ewing sarcoma cases by immunohistochemistry revealed expression in 99...
June 23, 2017: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
https://www.readbyqxmd.com/read/28640941/phox2b-reliably-distinguishes-neuroblastoma-among-small-round-blue-cell-tumors
#5
Yin P Hung, John P Lee, Andrew M Bellizzi, Jason L Hornick
AIMS: Neuroblastoma shows considerable histologic overlap with other small round blue cell tumors. PHOX2B, a transcription factor essential for autonomic nervous system development, has been reported as an immunohistochemical marker for neuroblastoma. The purpose of this study was to validate the specificity and diagnostic utility of PHOX2B for peripheral neuroblastic tumors. METHODS AND RESULTS: We evaluated 240 cases (133 in whole-tissue sections; 107 in tissue microarrays), including 76 peripheral neuroblastic tumors [median age 2 years; including 4 adults] and 164 other tumors: 44 Wilms tumors; 20 Ewing sarcomas; 10 each CIC-rearranged round cell sarcomas, poorly differentiated synovial sarcomas, lymphoblastic lymphomas, alveolar rhabdomyosarcomas, embryonal rhabdomyosarcomas, mesenchymal chondrosarcomas, Merkel cell carcinomas, olfactory neuroblastomas, and melanomas; 5 each NUT midline carcinomas and desmoplastic small round cell tumors...
June 22, 2017: Histopathology
https://www.readbyqxmd.com/read/28639531/outcome-of-bone-recycling-using-liquid-nitrogen-as-bone-reconstruction-procedure-in-malignant-and-recurrent-benign-aggressive-bone-tumour-of-distal-tibia-a-report-of-four-cases
#6
Eka Wiratnaya I Gede, Arrisna Artha Ida Ayu, Yudhi Setiawan I Gn, Wien Aryana Ign, Suyasa I Ketut, Siki Kawiyana I Ketut, Astawa Putu
Amputation still considered as primary choice of malignancy treatment in distal tibia. Bone recycling with liquid nitrogen for reconstruction following resection of malignant bone tumours offers many advantages. We presented four patients with osteosarcoma, Ewing sarcoma, adamantinoma and recurrent giant cell tumour over distal tibia. All of the patients underwent wide excision and bone recycling using liquid nitrogen as bone reconstruction. The mean functional Musculoskeletal Tumor Society (MSTS) score was 75% with no infection and local recurrent...
May 2017: Journal of Orthopaedic Surgery
https://www.readbyqxmd.com/read/28638739/ewing-sarcoma-partial-regression-without-gvhd-by-chondromodulin-i-hla-a-02-01-specific-allorestricted-t-cell-receptor-transgenic-t-cells
#7
Uwe Thiel, Sebastian J Schober, Ingo Einspieler, Andreas Kirschner, Melanie Thiede, David Schirmer, Katja Gall, Franziska Blaeschke, Oxana Schmidt, Susanne Jabar, Andreas Ranft, Rebeca Alba Rubío, Uta Dirksen, Thomas G P Grunewald, Poul H Sorensen, Günther H S Richter, Irene Teichert von Lüttichau, Dirk H Busch, Stefan E G Burdach
Background: Chondromodulin-I (CHM1) sustains malignancy in Ewing sarcoma (ES). Refractory ES carries a dismal prognosis and patients with bone marrow (BM) metastases do not survive irrespective of therapy. We assessed HLA-A*02:01/CHM1-specific allorestricted T cell receptor (TCR) wild-type and transgenic cytotoxic (CD8(+)) T cells against ES. Patients and Methods: Three refractory HLA-A2(+) ES patients were treated with HLA-A*02:01/peptide-specific allorepertoire-derived (i.e., allorestricted) CD8(+) T cells...
2017: Oncoimmunology
https://www.readbyqxmd.com/read/28630846/chronic-recurrent-multifocal-osteomyelitis-a-case-report-with-atypical-presentation
#8
Miguel Pádua Figueiredo, Marco Pato, Fernando Amaral
INTRODUCTION: Chronic recurrent multifocal osteomyelitis (CRMO) is a rare autoinflammatory condition. The clinical picture consists of sterile osteomyelitis, typically with multiple-site lesions in the metaphysis of long bones and not uncommonly, symmetrical bone involvement. It is a poorly understood entity, whose prognosis, etiology and ideal treatment are still controversial. The authors report a case of unifocal presentation with an atypical location. CASE REPORT: A previously healthy 12-year-old Caucasian girl came to our institution due to progressive pain on her left thigh for the previous 3 months...
January 2017: Journal of Orthopaedic Case Reports
https://www.readbyqxmd.com/read/28630763/malignant-tumours-of-the-foot-and-ankle
#9
E Mascard, N Gaspar, L Brugières, C Glorion, S Pannier, A Gomez-Brouchet
Most of tumours of the foot are tumour-like (synovial cyst, foreign body reactions and epidermal inclusion cyst) or benign conditions (tenosynovial giant cells tumours, planta fibromatosis). Malignant tumours of the soft-tissue and skeleton are very rare in the foot and their diagnosis is often delayed with referral to specialised teams after initial inappropriate procedures or unplanned excisions. The adverse effect of these misdiagnosed tumours is the increasing rate of amputation or local recurrences in the involved patients...
May 2017: EFORT open reviews
https://www.readbyqxmd.com/read/28627000/pencil-beam-scanned-protons-for-the-treatment-of-patients-with-ewing-sarcoma
#10
Damien C Weber, Fritz R Murray, Dora Correia, Alessandra Bolsi, Martina Frei-Welte, Alessia Pica, Antony J Lomax, Ralf Schneider, Barbara Bachtiary
BACKGROUND: Few data exist regarding the clinical outcome of patients with Ewing sarcoma (EWS) treated with pencil beam scanning proton therapy (PT). We report the outcome of children, adolescents and young adults (AYA) treated with PT at the Paul Scherrer Institute. MATERIALS: Thirty-eight patients (median age, 9.9 years) received a median dose of 54.9 Gy(RBE) (where RBE is relative biologic effectiveness). Size of the tumor ranged from 1.7 to 24 cm. Most common primary site was axial/pelvic (n = 27; 71%)...
June 19, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28626837/the-histogenesis-of-ewing-sarcoma
#11
Jian Tu, Zijun Huo, Julian Gingold, Ruiying Zhao, Jingnan Shen, Dung-Fang Lee
No abstract text is available yet for this article.
2017: Cancer Rep Rev
https://www.readbyqxmd.com/read/28626407/systemic-therapy-outcomes-in-adult-patients-with-ewing-sarcoma-family-of-tumors
#12
Mario Valdes, Garth Nicholas, Shailendra Verma, Timothy Asmis
BACKGROUND: The Ewing sarcoma family of tumors (ESFT) is a rare but curable bone neoplastic entity. The current standard of care involves chemotherapy and local disease control with surgery or radiation regardless of the extent of disease at presentation. Data that document the effectiveness of the current approach in the adult patient population are limited. METHODS: We performed a retrospective review including all ESFT patients older than 19 years of age who received systemic therapy between January 2002 and December 2013 at our institution...
May 2017: Case Reports in Oncology
https://www.readbyqxmd.com/read/28620142/magnetic-resonance-imaging-of-rrx-001-pharmacodynamics-in-preclinical-tumors
#13
Natarajan Raghunand, Jan Scicinski, Gerald P Guntle, Bhumasamudram Jagadish, Eugene A Mash, Elizabeth Bruckheimer, Bryan Oronsky, Ronald L Korn
RRx-001 is an anticancer agent that subjects cancer cells to reactive oxygen/nitrogen species (ROS/RNS) and acts as an epigenetic modifier. We have used a thiol-bearing MRI contrast agent, Gd-LC7-SH, to investigate the pharmacodynamics of RRx-001 in CHP-100 Ewing's Sarcoma, HT-29 colorectal carcinoma, and PANC-1 pancreatic carcinoma xenografts in SCID mice. Binding of Gd-LC7-SH to the Cys34 residue on plasma albumin prolongs retention in the tumor microenvironment and increases tumor enhancement on MRI. Mice were imaged by MRI and in vivo T1 maps acquired 50 min (T150 min) after injection of 0...
June 12, 2017: Oncotarget
https://www.readbyqxmd.com/read/28619077/what-is-the-impact-of-local-control-in-ewing-sarcoma-analysis-of-the-first-brazilian-collaborative-study-group-ewing1
#14
Ricardo G Becker, Lauro J Gregianin, Carlos R Galia, Reynaldo Jesus-Garcia Filho, Eduardo A Toller, Gerardo Badell, Suely A Nakagawa, Alexandre David, André M Baptista, Eduardo S Yonamime, Osvaldo A Serafini, Valter Penna, Julie Francine C Santos, Algemir L Brunetto
BACKGROUND: Relapse in localized Ewing sarcoma patients has been a matter of concern regarding poor prognosis. Therefore, we investigated the impact of local control modality (surgery, surgery plus radiotherapy, and radiotherapy) on clinical outcomes such as survival and recurrence in patients with non-metastatic Ewing sarcoma treated on the first Brazilian Collaborative Group Trial of the Ewing Family of Tumors (EWING1). METHODS: Seventy-three patients with localized Ewing sarcoma of bone aged < 30 years were included...
June 15, 2017: BMC Cancer
https://www.readbyqxmd.com/read/28616785/usefulness-of-nkx2-2-immunohistochemistry-for-distinguishing-ewing-sarcoma-from-other-sinonasal-small-round-blue-cell-tumors
#15
Austin McCuiston, Justin A Bishop
NKX2.2 is a new immunohistochemical marker that has been reported to be sensitive and specific for Ewing sarcoma (ES). It has not, however, been investigated specifically in the sinonasal small round blue cell tumor (SRBCT) differential diagnosis which includes many tumors specific to that site. It has also not been investigated in the newly recognized "adamantinoma-like" variant of ES. Immunohistochemistry for NKX2.2 was performed on 170 poorly differentiated sinonasal neoplasms: 73 squamous cell carcinomas (67 poorly differentiated, non-keratinizing, or basaloid types and 6 nasopharyngeal carcinomas), 46 olfactory neuroblastomas, 8 sinonasal undifferentiated carcinomas (SNUCs), 6 melanomas, 7 Ewing sarcomas, 6 SMARCB1-deficient carcinomas, 6 teratocarcinosarcomas, 5 alveolar rhabdomyosarcomas, 4 solid adenoid cystic carcinomas, 4 NK/T cell lymphomas, 3 NUT carcinomas, and 2 small cell carcinomas...
June 14, 2017: Head and Neck Pathology
https://www.readbyqxmd.com/read/28616622/a-case-of-primary-mediastinal-ewing-s-sarcoma-primitive-neuroectodermal-tumor-presenting-with-chest-pain
#16
Ural Koc, Erkan Duman
No abstract text is available yet for this article.
June 2017: Turkish Journal of Emergency Medicine
https://www.readbyqxmd.com/read/28612488/front-line-window-therapy-with-cisplatin-in-patients-with-primary-disseminated-ewing-sarcoma-a-study-by-the-associazione-italiana-di-ematologia-ed-oncologia-pediatrica-and-italian-sarcoma-group
#17
Roberto Luksch, Giovanni Grignani, Paolo D'Angelo, Arcangelo Prete, Nadia Puma, Marta Podda, Michela Casanova, Andrea Ferrari, Carlo Morosi, Franca Fagioli, Massimo Aglietta, Stefano Ferrari, Piero Picci, Maura Massimino
The aim was to assess the activity of cisplatin (CDDP) in Ewing sarcoma (ES). The study consisted of front-line window therapy with CDDP 120 mg/sqm every 3 weeks for two courses in children and young adults with primary disseminated ES. Response was assessed using the Response Evaluation Criteria in Solid Tumours criteria, and Simon's two-stage design was applied. Twelve consecutive patients were enrolled in stage 1. Only one objective response was observed. Since the target response rate was not achieved, accrual was stopped and CDDP as a single agent in ES was judged unworthy of further assessment...
June 14, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28605142/aberrant-expression-of-fli-1-in-melanoma
#18
Nisha Ramani, Phyu P Aung, Wen-Jen Hwu, Priyadharsini Nagarajan, Michael T Tetzlaff, Jonathan L Curry, Doina Ivan, Victor G Prieto, Carlos A Torres-Cabala
FLI-1 (Friend leukemia integration site 1) nuclear transcription factor has been proposed as a suitable tool in the differential diagnosis of small round cell sarcomas. It has also been described as nuclear marker of endothelial differentiation. Expression of FLI-1 has been demonstrated in Ewing's sarcoma/ primitive neuroectodermal tumor (ES/PNET) and vascular neoplasms. In the present study, we describe two cases of metastatic melanoma with small round blue cell morphology that showed strong nuclear expression of FLI-1...
June 12, 2017: Journal of Cutaneous Pathology
https://www.readbyqxmd.com/read/28601426/corrigendum-to-risk-stratification-and-pattern-of-cardiotoxicity-in-pediatric-ewing-sarcoma-j-egypt-natl-cancer-instit-29-2017-53-56
#19
Emad Moussa, Manal Zamzam, Ahmed Kamel, Zeinab Salah, Iman Attia, Lina Gaber, Ranin Soliman, Sameera Ezzat
No abstract text is available yet for this article.
June 7, 2017: Journal of the Egyptian National Cancer Institute
https://www.readbyqxmd.com/read/28589731/next-steps-in-ewing-sarcoma-epi-genomics
#20
Giuseppina Sannino, Martin F Orth, Thomas Gp Grünewald
No abstract text is available yet for this article.
June 7, 2017: Future Oncology
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