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Metastatic ewing's sarcoma

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https://www.readbyqxmd.com/read/30477937/efficacy-and-safety-of-regorafenib-in-adult-patients-with-metastatic-osteosarcoma-a-non-comparative-randomised-double-blind-placebo-controlled-phase-2-study
#1
Florence Duffaud, Olivier Mir, Pascaline Boudou-Rouquette, Sophie Piperno-Neumann, Nicolas Penel, Emanuelle Bompas, Corinne Delcambre, Elsa Kalbacher, Antoine Italiano, Olivier Collard, Christine Chevreau, Esma Saada, Nicolas Isambert, Jessy Delaye, Camille Schiffler, Corinne Bouvier, Vincent Vidal, Sylvie Chabaud, Jean-Yves Blay
BACKGROUND: Regorafenib has proven activity in patients with pretreated gastrointestinal stromal tumours and colorectal and hepatocellular carcinoma. We designed REGOBONE to assess the efficacy and safety of regorafenib for patients with progressive metastatic osteosarcoma and other bone sarcomas. This trial comprised four parallel independent cohorts: osteosarcoma, Ewing sarcoma, chondrosarcoma, and chordoma. In this Article, we report the results of the osteosarcoma cohort. METHODS: In this non-comparative, double-blind, placebo-controlled, phase 2 trial, patients aged 10 years or older with histologically confirmed osteosarcoma whose disease had progressed after treatment with one to two previous lines of chemotherapy for metastatic disease and an Eastern Cooperative Oncology Group performance status of 0 or 1 were enrolled...
November 23, 2018: Lancet Oncology
https://www.readbyqxmd.com/read/30470587/mir-708-5p-is-inversely-associated-with-ews-fli1-ewing-sarcoma-but-does-not-represent-a-prognostic-predictor
#2
Gabriela Molinari Roberto, Gabriela Maciel Vieira, Lara Elis Alberici Delsin, Marcela de Oliveira Silva, Rodrigo Guedes Hakime, Edgard Eduard Engel, Carlos Alberto Scrideli, Luiz Gonzaga Tone, María Sol Brassesco
BACKGROUND: Overall survival of Ewing sarcoma (EWS) remains poor and less than 30% of patients with metastatic or recurrent disease survive despite current treatments. Thus, there is a constant search for new biomarkers for diagnosis, prognosis and prediction of therapy. Numerous studies have reported the abnormal expression of miR-708-5p in tumors of different origins. However, its role in EWS remains unclear. PROCEDURE: qRT-PCR was performed in nineteen consecutive EWS samples and twelve non-tumor bone samples from age-matched controls...
November 13, 2018: Cancer Genetics
https://www.readbyqxmd.com/read/30449071/ewing-s-sarcoma-of-the-head-and-neck-margins-are-not-just-for-surgeons
#3
Jebrane Bouaoud, Stephane Temam, Nathalie Cozic, Louise Galmiche-Rolland, Kahina Belhous, Frederic Kolb, Francois Bidault, Stephanie Bolle, Sarah Dumont, Valerie Laurence, Dominique Plantaz, Marie-Dominique Tabone, Perrine Marec-Berard, Quentin Quassemyar, Vincent Couloigner, Arnaud Picard, Anne Gomez-Brouchet, Marie-Cécile Le Deley, Céline Mahier-Ait Oukhatar, Natacha Kadlub, Nathalie Gaspar
BACKGROUND, METHODS: To describe the characteristics, treatments (systemic/local), and outcome (oncological/functional) of French patients with head and neck Ewing's sarcomas (HNES) registered in the Euro-Ewing 99 (EE99) database. Specific patient-level data were reviewed retrospective. RESULTS: Forty-seven HNES patients in the EE99 database had a median age of 11 years, 89% had bone tumors (skull 55%, mandible 21%, maxilla 11%), 89% had small tumors (<200 mL), and they were rarely metastatic (9%)...
November 17, 2018: Cancer Medicine
https://www.readbyqxmd.com/read/30412903/risk-factors-for-detectable-metastatic-disease-at-presentation-in-ewing-sarcoma-an-analysis-of-the-seer-registry
#4
Dipak B Ramkumar, Niveditta Ramkumar, Benjamin J Miller, Eric R Henderson
BACKGROUND: Ewing family of tumors (EFT) represents the second-most common primary bone malignancy in children and adolescents. Approximately 25% of patients have radiographically detectable metastatic disease at presentation and experience poorer five-year survival, yet risk factors for metastatic disease at presentation are poorly characterized. We sought to study patient characteristics associated with metastatic disease upon presentation for patients with EFT. METHODS: We identified EFT cases in the Surveillance, Epidemiology, and End Results Program (SEER) registry from 2004 to 2012...
November 6, 2018: Cancer Epidemiology
https://www.readbyqxmd.com/read/30365478/spinal-ewing-sarcoma-metastatic-to-bilateral-orbits
#5
Lawrence Chan, Marez Megalla, Jenna M Kim, Renelle Pointdujour-Lim
A 39-year-old woman with primary spinal Ewing sarcoma and known lung metastases presented with painless bilateral decreased visual acuity over a 1-month period. Examination revealed bilateral disc edema. MRI of the brain/orbits showed metastatic lesions to the dura and bilateral orbits. Venous sinus thrombosis extending to the jugular vein was also noted. To the authors' knowledge, this is the first case of bilateral orbital metastasis and papilledema secondary to Ewing sarcoma and related hypercoagulability...
October 24, 2018: Ophthalmic Plastic and Reconstructive Surgery
https://www.readbyqxmd.com/read/30339522/clinical-and-radiographic-presentation-of-pelvic-sarcoma-in-children
#6
Muayad Kadhim, Nariman Abol Oyoun, Richard B Womer, John P Dormans
INTRODUCTION: Pelvic sarcomas may present with vague symptoms. The aim of this study was to examine the characteristics and clinical presentations of different types of pelvic sarcoma in children. METHODS: This is a retrospective cohort study of patients up to 21 years of age with the diagnosis of pelvic sarcoma between January 2000 and June 2013. Data on demographics, tumor type and location, and clinical presentation were collected from the medical records. RESULTS: A total of 59 patients [37 males (62...
2018: SICOT-J
https://www.readbyqxmd.com/read/30275176/spinal-ewing-sarcoma-debuting-with-cord-compression-have-we-discovered-the-thread-of-ariadne
#7
REVIEW
Stergios Boussios, Catherine Hayward, Deirdre Cooke, Nikolaos Zakynthinakis-Kyriakou, Alexandros K Tsiouris, Aikaterini A Chatziantoniou, Foivos S Kanellos, Afroditi Karathanasi
Ewing's sarcoma (ES) of the spine with cord or radicular compression as an initial sign is infrequent. It is unclear, in alleviating a neurological deficit, whether decompressive laminectomy is preferred over chemotherapy. Herein, a literature review of the treatment approaches to the primary or metastatic ES of the spine has been performed. Collected data included clinical features of the patients, treatment, and outcome. There are reported 69 cases with initial presentation of cord or radicular compression of spinal cord, arising from primary or metastatic ES, treated either with initial chemotherapy and/or radiotherapy (RT) (33...
October 2018: Anticancer Research
https://www.readbyqxmd.com/read/30173130/oesophageal-narrowing-during-combination-chemotherapy-in-ewing-s-sarcoma-is-vincristine-a-culprit
#8
Kunal Das, Santosh Singh, BrahmaPrakash Kalra, Nitika Agrawal
Vincristine is a widely used chemotherapeutic agent in paediatric oncology. A 7-year-old boy was diagnosed with non-metastatic Ewing's sarcoma of the pelvis. He was started on chemotherapy with vincristine-cyclophosphamide-adriamycin alternate with ifosfamide-etoposide. He developed recurrent vomiting after three cycles of chemotherapy. Evaluation showed oesophageal stricture involving the middle and lower third part. Biopsy was non-conclusive. His symptoms improved with dilatation. A chemotherapy-induced neuropathic dysmotility was suspected, and his chemotherapy was continued with serial dilatation...
September 1, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/30148241/extraosseous-ewing-sarcoma-arising-in-a-chronically-lymphedematous-limb
#9
David John Tobias McArdle, Louise Nott, Robin Harle, John Patrick McArdle
Ewing sarcoma is a highly aggressive small round cell malignant neoplasm of bone and soft tissue that typically is manifested in children and young adults. It is most commonly a primary bone tumor; however, extraosseous cases have been increasingly reported. We report a case of metastatic extraosseous Ewing sarcoma with the primary lesion occurring within a limb affected by primary lymphedema. Lymphedema, in this case, played a role not only in the genesis of the tumor because of the relative local immunosuppression but also in masking the development of the lower limb mass...
September 2018: Journal of Vascular Surgery Cases and Innovative Techniques
https://www.readbyqxmd.com/read/30147091/analysis-of-bone-and-soft-tissue-sarcomas-registered-during-the-year-2012-at-tata-memorial-hospital-mumbai-with-clinical-outcomes
#10
Jyoti Bajpai, Nehal Khanna, Tushar Vora, Ashish Gulia, Siddhartha Laskar, Ajay Puri, Bhanupriya Sanduptla, Girish Chinnaswamy, Prakash Nayak, Shashikant L Juvekar, Amit Janu, Subhash Desai, Jaya Ghosh, Nilendu Purandare, Mukta Ramadwar, Venkatesh Rangarajan, Bharat Rekhi
Introduction: Primary bone and soft tissue sarcomas are rare, but diagnostically and therapeutically challenging group of tumors, requiring multidisciplinary management. There are limited documented studies from multidisciplinary teams , in the form of comprehensive analysis of these tumors, from our country. This study is an analysis of cases of osteosarcomas, Ewing sarcomas (ESs), chondrosarcomas (CSs), and soft-tissue sarcomas (STSs), registered at our institution during 2012. Methods: Clinical details, including outcomes of cases of bone and STSs, during the year 2012, were retrieved from the medical records of our institution and were further analyzed...
January 2018: Indian Journal of Cancer
https://www.readbyqxmd.com/read/30131449/usp6-confers-sensitivity-to-ifn-mediated-apoptosis-through-modulation-of-trail-signaling-in-ewing-sarcoma
#11
Ian C Henrich, Robert Young, Laura Quick, Andre M Oliveira, Margaret M Chou
Ewing sarcoma is the second most common sarcoma of the bone, afflicting predominantly the pediatric population. Although patients with localized disease exhibit favorable survival rates, patients with metastatic disease suffer a dismal 5-year rate of approximately 25%. Thus, there is a great need to develop treatments to combat the disseminated disease. Ubiquitin-specific protease 6 (USP6/TRE17) has been implicated as the key etiologic factor in several benign mesenchymal tumors, including nodular fasciitis and aneurysmal bone cyst (ABC)...
August 21, 2018: Molecular Cancer Research: MCR
https://www.readbyqxmd.com/read/30116912/sorafenib-in-patients-with-progressed-and-refractory-bone-tumors
#12
Anna Raciborska, Katarzyna Bilska
Patients with metastatic, progressive, or recurrent bone tumors have a dismal outcome. Sorafenib has been proposed as an effective salvage regimen for some malignancies. Thus, we sought to evaluate this approach for young patients with relapsed or refractory bone tumors. Twelve patients with refractory bone tumors (two with Ewing sarcoma, two with chondrosarcoma, and eight with osteosarcoma) received salvage treatment with sorafenib. All patients had standard tumor imaging and laboratory evaluation. All toxicities were documented...
August 16, 2018: Medical Oncology
https://www.readbyqxmd.com/read/30085939/primary-ewing-family-of-tumor-arising-in-the-ovary-a-case-report
#13
Yi-Ping Li, Koping Chang, Tom Wei-Wu Chen, Shu-Ping Lee, Chi-An Chen, Wen-Fang Cheng
Ewing sarcoma and peripheral primitive neuroectodermal tumor constitute the Ewing family of tumors (EFT). EFTs primarily arising in the ovary are extremely rare. We report the case of a 22-yr-old nulliparous woman with a primary EFT in the ovary that initially presented as a 3-cm teratoma-like ovarian tumor, with rapid progression to a 15-cm-sized tumor with liver metastasis in 3 mo. The patient underwent suboptimal debulking surgery and salvage chemotherapy with vincristine, doxorubicin, and cyclophosphamide alternating with ifosfamide and etoposide...
August 6, 2018: International Journal of Gynecological Pathology
https://www.readbyqxmd.com/read/30084137/impact-of-the-interdisciplinary-tumor-board-of-the-cooperative-ewing-sarcoma-study-group-on-local-therapy-and-overall-survival-of-ewing-sarcoma-patients-after-induction-therapy
#14
Justus Kreyer, Andreas Ranft, Beate Timmermann, Heribert Juergens, Susanne Jung, Karsten Wiebe, Tobias Boelling, Andreas Schuck, Volker Vieth, Arne Streitbuerger, Jendrik Hardes, Melina Heinemann, Uta Dirksen
BACKGROUND: The Interdisciplinary Tumor Board (ITB) of the Cooperative Ewing Sarcoma Study (CESS) Group was investigated to assess its impact on the overall survival (OAS) of Ewing sarcoma (EwS) patients. The ITB functions as a reference center for the international institutions participating in the clinical trials of the CESS group, but is also available internationally to patients who have not been treated within an appropriate clinical trial. The value of tumor boards in terms of benefit for the patients and the health care system in general is not well documented and is also the subject of controversial discussions...
August 7, 2018: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/30080131/vincristine-irinotecan-and-temozolomide-treatment-for-refractory-relapsed-pediatric-solid-tumors-a-single-center-experience
#15
Sema Büyükkapu Bay, Rejin Kebudi, Omer Görgün, Bülent Zülfikar, Emin Darendeliler, Fatma B Çakır
Background Although the survival of pediatric cancer has increased dramatically in the last decades, the survival of refractory, relapsed, and metastatic cases is still dismal. The combination of irinotecan and temozolomide has shown activity against refractory/relapsed pediatric solid tumors. Method Thirty-four children with refractory/relapsed solid tumors who had previously been heavily pretreated and who were given vincristine, irinotecan, and temozolomide as third- or further line chemotherapy during 2004-2015 were evaluated...
August 6, 2018: Journal of Oncology Pharmacy Practice
https://www.readbyqxmd.com/read/30077790/short-hypofractionated-radiation-therapy-in-palliation-of-pediatric-malignancies-outcomes-and-toxicities
#16
Stanislav Lazarev, Brian H Kushner, Suzanne L Wolden
PURPOSE: Treatment strategies in palliation of pediatric cancer remain a significant challenge. In this study, we aimed to assess the efficacy and safety of a short course of hypofractionated radiation therapy (RT) for metastatic or recurrent childhood tumors. METHODS AND MATERIALS: A total of 104 lesions in 62 pediatric patients with metastatic or recurrent cancer were treated with a short hypofractionation schedule (>1 but ≤5 fractions; ≥3 Gy per fraction) between 2007 and 2017 in our institution...
August 2, 2018: International Journal of Radiation Oncology, Biology, Physics
https://www.readbyqxmd.com/read/30048248/survival-outcome-and-prognostic-factors-after-pulmonary-metastasectomy-in-sarcoma-patients-a-18-year-experience-at-a-single-high-volume-referral-center
#17
Umberto Cariboni, Rita De Sanctis, Marta Giaretta, Emanuele Voulaz, Emanuela Morenghi, Piergiuseppe Colombo, Pierluigi Novellis, Edoardo Bottoni, Valentina Errico, Alessandro Crepaldi, Andrea Marrari, Pierina Navarria, Anna M Ascolese, Alberto Testori, Marta Scorsetti, Vittorio Quagliuolo, Armando Santoro, Marco Alloisio
BACKGROUND: Metastatic sarcoma patients have a poor prognosis with 3-year survival rate of 25%. About 30% of them present isolated lung metastases. We aimed to analyze the clinical outcome of sarcoma patients undergoing pulmonary metastasectomy (PM) and prognostic factors associated with local control, disease-free, and overall survival (OS). PATIENTS AND METHODS: All sarcoma patients undergoing PM at Humanitas Cancer Center were retrospectively reviewed. Analyzed variables included clinicopathologic, surgical, and survival data...
July 25, 2018: American Journal of Clinical Oncology
https://www.readbyqxmd.com/read/29977059/ewing-sarcoma
#18
REVIEW
Thomas G P Grünewald, Florencia Cidre-Aranaz, Didier Surdez, Eleni M Tomazou, Enrique de Álava, Heinrich Kovar, Poul H Sorensen, Olivier Delattre, Uta Dirksen
Ewing sarcoma is the second most frequent bone tumour of childhood and adolescence that can also arise in soft tissue. Ewing sarcoma is a highly aggressive cancer, with a survival of 70-80% for patients with standard-risk and localized disease and ~30% for those with metastatic disease. Treatment comprises local surgery, radiotherapy and polychemotherapy, which are associated with acute and chronic adverse effects that may compromise quality of life in survivors. Histologically, Ewing sarcomas are composed of small round cells expressing high levels of CD99...
July 5, 2018: Nature Reviews. Disease Primers
https://www.readbyqxmd.com/read/29949650/reconstructing-diaphyseal-tumors-using-radiated-50-gy-autogenous-tumor-bone-graft
#19
Ajay Puri, Suman Byregowda, Ashish Gulia, Vijayraj Patil, Saniya Crasto, Siddharth Laskar
AIMS: Establish oncologic safety using 50 Gy to sterilize tumor bearing bone before reimplantation in primary diaphyseal high grade extremity tumors, determine extracorporeal radiotherapy (ECRT) graft survival, and analyze factors that affect union at osteotomy sites. MATERIALS AND METHODS: Seventy non metastatic patients underwent reconstruction with intercalary ECRT grafts sterilized with 50 Gy. Diagnosis included osteosarcoma (38) and Ewing's sarcoma (32)...
July 2018: Journal of Surgical Oncology
https://www.readbyqxmd.com/read/29936064/phase-i-results-of-a-phase-i-ii-study-of-weekly-nab-paclitaxel-in-paediatric-patients-with-recurrent-refractory-solid-tumours-a-collaboration-with-innovative-therapies-for-children-with-cancer
#20
Lucas Moreno, Michela Casanova, Julia C Chisholm, Pablo Berlanga, Pascal B Chastagner, Sylvain Baruchel, Loredana Amoroso, Soledad Gallego Melcón, Nicolas U Gerber, Gianni Bisogno, Franca Fagioli, Birgit Geoerger, Julia L Glade Bender, Isabelle Aerts, Christophe Bergeron, Pooja Hingorani, Ileana Elias, Mathew Simcock, Stefano Ferrara, Yvan Le Bruchec, Ruta Slepetis, Nianhang Chen, Gilles Vassal
BACKGROUND: nab-Paclitaxel has demonstrated efficacy in adults with solid tumours and preclinical activity in paediatric solid tumour models. Results from phase I of a phase I/II study in paediatric patients with recurrent/refractory solid tumours treated with nab-paclitaxel are reported. PATIENTS AND METHODS: Patients with recurrent/refractory extracranial solid tumours received nab-paclitaxel on days 1, 8 and 15 every 4 weeks at 120, 150, 180, 210, 240, or 270 mg/m2 (rolling-6 dose-escalation) to establish the maximum tolerated dose (MTD) and recommended phase II dose (RP2D)...
September 2018: European Journal of Cancer
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