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Ewings sarcoma

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https://www.readbyqxmd.com/read/28724045/spinal-tumors-in-children
#1
Andrei Fernandes Joaquim, Enrico Ghizoni, Marcelo Gomes Cordeiro Valadares, Simone Appenzeller, Simone Dos Santos Aguiar, Helder Tedeschi
Introduction: Spinal tumors are rare in the pediatric population, presenting many specific peculiarities when compared to adults. We have performed a broad narrative review to describe the most common spinal tumors in children, discussing their main characteristics and management options. Method: The authors have performed an extensive review of the peer-reviewed literature addressing the aforementioned objectives. Results: Multimodality radiological studies (plain films, 3D computed tomography scan and magnetic resonance imaging) are necessary for proper evaluation and differential diagnosis of spinal tumors in children...
May 2017: Revista da Associação Médica Brasileira
https://www.readbyqxmd.com/read/28722673/expression-and-clinical-association-of-programmed-cell-death-1-programmed-death-ligand-1-and-cd8-lymphocytes-in-primary-sarcomas-is-subtype-dependent
#2
Anke E M van Erp, Yvonne M H Versleijen-Jonkers, Melissa H S Hillebrandt-Roeffen, Laurens van Houdt, Mark A J Gorris, Laura S van Dam, Thomas Mentzel, Marije E Weidema, C Dilara Savci-Heijink, Ingrid M E Desar, Hans H M Merks, Max M van Noesel, Janet Shipley, Winette T A van der Graaf, Uta E Flucke, Friederike A G Meyer-Wentrup
In order to explore the potential of immune checkpoint blockade in sarcoma, we investigated expression and clinical relevance of programmed cell death-1 (PD-1), programmed death ligand-1 (PD-L1) and CD8 in tumors of 208 sarcoma patients. Primary untreated osteosarcoma (n = 46), Ewing sarcoma (n = 32), alveolar rhabdomyosarcoma (n = 20), embryonal rhabdomyosarcoma (n = 77), synovial sarcoma (n = 22) and desmoplastic small round cell tumors (DSRCT) (n = 11) were examined immunohistochemically. PD-L1 expression was predominantly detected in alveolar and embryonal rhabdomyosarcomas (15% and 16%, respectively)...
July 7, 2017: Oncotarget
https://www.readbyqxmd.com/read/28720588/ews-fli-is-a-master-regulator-of-metabolic-reprogramming-in-ewing-sarcoma
#3
Jason M Tanner, Claire Bensard, Peng Wei, Nathan M Krah, John C Schell, Jamie D Gardiner, Joshua D Schiffman, Stephen L Lessnick, Jared Rutter
Ewing sarcoma is a bone malignancy driven by a translocation event resulting in the fusion protein EWS/FLI1 (EF). EF functions as an aberrant and oncogenic transcription factor that misregulates the expression of thousands of genes. Previous work has focused principally on determining important transcriptional targets of EF, as well as characterizing important regulatory partnerships in EF-dependent transcriptional programs. Less is known, however, about EF-dependent metabolic changes or their role in Ewing sarcoma biology...
July 18, 2017: Molecular Cancer Research: MCR
https://www.readbyqxmd.com/read/28718313/massive-embryonal-rhabdomyosarcoma-of-the-hand-in-an-infant-with-metastasis-at-birth-management-dilemma
#4
Jayakrishnan K Narayana Kurup, Vinay C Kamble, Ashwath M Acharya, Anil K Bhat
BACKGROUND: Rhabdomyosarcomas are malignant tumors arising from striated muscle but can be often confused with primitive neuroectodermal tumors and Ewing sarcoma. They are often classified based on age of presentation and histological features. Three major types of rhabdomyosarcomas are embryonal, alveolar, and pleomorphic with characteristic presentations. METHOD: Here, we present a case of embryonal rhabdomyosarcoma in the hand of a 5-month-old child with lymphatic metastasis, the age, site, and metastasis being unusual feature for this type of rhabdomyosarcoma...
January 1, 2017: Hand: Official Journal of the American Association for Hand Surgery
https://www.readbyqxmd.com/read/28716733/ml327-induces-apoptosis-and-sensitizes-ewing-sarcoma-cells-to-tnf-related-apoptosis-inducing-ligand
#5
Eric J Rellinger, Chandrasekhar Padmanabhan, Jingbo Qiao, Andrew Appert, Alex G Waterson, Craig W Lindsley, R Daniel Beauchamp, Dai H Chung
Ewing sarcomas are rare mesenchymal-derived bone and soft tissue tumors in children. Afflicted children with distant metastases have poor survival despite aggressive therapeutics. Epithelial-to-mesenchymal transition in epithelial carcinomas is associated with loss of E-cadherin and resistance to apoptosis. ML327 is a novel small molecule that we have previously shown to reverse epithelial-to-mesenchymal transition features in both epithelial and neural crest-derived cancers. Herein, we sought to evaluate the effects of ML327 on mesenchymal-derived Ewing sarcoma cells, hypothesizing that ML327 initiates growth arrest and sensitizes to TNF-related apoptosis-inducing ligand...
July 14, 2017: Biochemical and Biophysical Research Communications
https://www.readbyqxmd.com/read/28710342/vincristine-ifosfamide-and-doxorubicin-for-initial-treatment-of-ewing-sarcoma-in-adults
#6
Michael J Wagner, Vancheswaran Gopalakrishnan, Vinod Ravi, J Andrew Livingston, Anthony P Conley, Dejka Araujo, Neeta Somaiah, Maria A Zarzour, Ravin Ratan, Wei-Lien Wang, Shreyaskumar R Patel, Alexander Lazar, Joseph A Ludwig, Robert S Benjamin
BACKGROUND: There are no clinical trials specifically addressing chemotherapy for adults with Ewing sarcoma (ES). Five-year event-free survival (EFS) of adults on pediatric studies of ES (44%-47%) is worse than that of children treated with the same therapy (69%). The object of this study was to review the results of therapy with vincristine, ifosfamide, and doxorubicin (VID) in the multidisciplinary treatment of adults with ES at our institution. MATERIALS AND METHODS: Charts for adults treated for ES from 1995 to 2011 were retrospectively reviewed...
July 14, 2017: Oncologist
https://www.readbyqxmd.com/read/28697168/pretreatment-neutrophil-to-lymphocyte-ratio-and-lymphocyte-recovery-independent-prognostic-factors-for-survival-in-pediatric-sarcomas
#7
Liliana Vasquez, Esmeralda León, Brady Beltran, Ivan Maza, Monica Oscanoa, Jenny Geronimo
BACKGROUND: Pretreatment neutrophil-to-lymphocyte ratio (NLR) and absolute lymphocyte count (ALC) recovery have been shown to be associated with prognosis in several types of cancer in adults. However, evidence in pediatric cancer is scarce. The aim of our study was to evaluate whether pretreatment NLR and lymphocyte recovery are prognostic factors in pediatric sarcomas. MATERIALS AND METHODS: Study participants were identified from a retrospective cohort of 100 children with osteosarcoma (n=55), rhabdomyosarcoma (n=22), and Ewing sarcoma (n=23)...
July 10, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28696044/practice-patterns-of-palliative-radiation-therapy-in-pediatric-oncology-patients-in-an-international-pediatric-research-consortium
#8
Avani Dholakia Rao, Qinyu Chen, Ralph P Ermoian, Sara R Alcorn, Maria Luisa S Figueiredo, Michael J Chen, Karin Dieckmann, Shannon M MacDonald, Matthew M Ladra, Daria Kobyzeva, Alexey V Nechesnyuk, Kristina Nilsson, Eric C Ford, Brian A Winey, Rosangela C Villar, Stephanie A Terezakis
BACKGROUND/OBJECTIVES: The practice of palliative radiation therapy (RT) is based on extrapolation from adult literature. We evaluated patterns of pediatric palliative RT to describe regimens used to identify opportunity for future pediatric-specific clinical trials. DESIGN/METHODS: Six international institutions with pediatric expertise completed a 122-item survey evaluating patterns of palliative RT for patients ≤21 years old from 2010 to 2015. Two institutions use proton RT...
July 11, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28691904/synthetic-lethality-between-the-cohesin-subunits-stag1-and-stag2-in-diverse-cancer-contexts
#9
Petra van der Lelij, Simone Lieb, Julian Jude, Gordana Wutz, Catarina P Santos, Katrina Falkenberg, Andreas Schlattl, Jozef Ban, Raphaela Schwentner, Thomas Hoffmann, Heinrich Kovar, Francisco X Real, Todd Waldman, Mark A Pearson, Norbert Kraut, Jan-Michael Peters, Johannes Zuber, Mark Petronczki
Recent genome analyses have identified recurrent mutations in the cohesin complex in a wide range of human cancers. Here we demonstrate that the most frequently mutated subunit of the cohesin complex, STAG2, displays a strong synthetic lethal interaction with its paralog STAG1. Mechanistically, STAG1 loss abrogates sister chromatid cohesion in STAG2 mutated but not in wild-type cells leading to mitotic catastrophe, defective cell division and apoptosis. STAG1 inactivation inhibits the proliferation of STAG2 mutated but not wild-type bladder cancer and Ewing sarcoma cell lines...
July 10, 2017: ELife
https://www.readbyqxmd.com/read/28690908/primary-occipital-ewing-s-sarcoma-with-subsequent-spinal-seeding
#10
Ali Alqahtani, Roaa Amer, Eman Bakhsh
Ewing's sarcoma is a primary bone cancer that mainly affects the long bones. This malignancy is particularly common in pediatric patients. Primary cranial involvement accounts for 1% of cases, with occipital involvement considered extremely rare. In this case study, primary occipital Ewing's sarcoma with a posterior fossa mass and subsequent relapse resulting in spinal seeding is reported. A 3-year-old patient presented with a 1-year history of left-sided headaches, localized over the occipital bone with progressive torticollis...
2017: Case Reports in Pediatrics
https://www.readbyqxmd.com/read/28687003/inhibition-of-chk1-sensitizes-ewing-sarcoma-cells-to-the-ribonucleotide-reductase-inhibitor-gemcitabine
#11
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...
June 28, 2017: Oncotarget
https://www.readbyqxmd.com/read/28684229/reverse-shoulder-endoprosthesis-for-pathologic-lesions-of-the-proximal-humerus-a-minimum-3-year-follow-up
#12
Simon Maclean, Shahbaz S Malik, Scott Evans, Jonathan Gregory, Lee Jeys
BACKGROUND: The Bayley Walker (Stanmore Implants, Elstree, UK) reversed polarity, linked shoulder replacement is designed to provide stable function in the treatment of a painful shoulder with poor soft tissue coverage. We reviewed the results of the prosthesis in destructive pathologic lesions of the proximal humerus at a United Kingdom tumor center. METHODS: We identified 8 patients (2 men, 6 women) in our database. Clinical information and functional outcome scores were collected, including range of movement, Toronto Extremity Salvage Score, the Musculoskeletal Tumor Score...
July 3, 2017: Journal of Shoulder and Elbow Surgery
https://www.readbyqxmd.com/read/28681998/eribulin-regresses-a-doxorubicin-resistant-ewing-s-sarcoma-with-a-fus-erg-fusion-and-cdkn2a-deletion-for-the-patient-derived-orthotopic-xenograft-pdox-nude-mouse-model
#13
Kentaro Miyaki, Takashi Murakami, Tasuku Kiyuna, Kentaro Igarashi, Kei Kawaguchi, Yunfeng Li, Arun S Singh, Sarah M Dry, Mark A Eckardt, Yukihiko Hiroshima, Masashi Momiyama, Ryusei Matsuyama, Takashi Chishima, Itaru Endo, Fritz C Eilber, Robert M Hoffman
Ewing's sarcoma is a recalcitrant tumor greatly in need of more effective therapy. The aim of this study was to determine the efficacy of eribulin on a doxorubicin (DOX)-resistant Ewing's sarcoma patient derived orthotopic xenograft (PDOX) model. The Ewing's sarcoma PDOX model was previously established in the right chest wall of nude mice from the patient's right chest wall. In the previous study, the Ewing's sarcoma PDOX was resistant to DOX and sensitive to palbociclib and linsitinib. In the present study, the PDOX models were randomized into 3 groups when the tumor volume reached 60 mm(3) : G1, untreated control (n = 6); G2, DOX treated (n = 6), intraperitoneal (i...
July 6, 2017: Journal of Cellular Biochemistry
https://www.readbyqxmd.com/read/28680140/generation-of-novel-patient-derived-cic-dux4-sarcoma-xenografts-and-cell-lines
#14
Rieko Oyama, Mami Takahashi, Akihiko Yoshida, Marimu Sakumoto, Yoko Takai, Fusako Kito, Kumiko Shiozawa, Zhiwei Qiao, Yasuhito Arai, Tatsuhiro Shibata, Yoshihiro Araki, Makoto Endo, Akira Kawai, Tadashi Kondo
CIC-DUX4 sarcoma (CDS) is a group of rare, mesenchymal, small round cell tumours that harbour the unique CIC-DUX4 translocation, which causes aberrant gene expression. CDS exhibits an aggressive course and poor clinical outcome, thus novel therapeutic approaches are needed for CDS treatment. Although patient-derived cancer models are an essential modality to develop novel therapies, none currently exist for CDS. Thus, the present study successfully established CDS patient-derived xenografts and subsequently generated two CDS cell lines from the grafted tumours...
July 5, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28676226/late-toxicity-and-outcomes-following-radiation-therapy-for-chest-wall-sarcomas-in-pediatric-patients
#15
John T Lucas, Israel Fernandez-Pineda, Christopher L Tinkle, Michael W Bishop, Sue C Kaste, Rajiv Heda, Andrew M Davidoff, Matthew J Krasin
PURPOSE: To investigate the contribution of radiation therapy to acute and late toxicity in pediatric chest wall sarcoma patients and evaluate dosimetric correlates of higher incidence toxicities such as scoliosis and pneumonitis. METHODS AND MATERIALS: The data from 23 consecutively treated pediatric patients with chest wall sarcomas of various histologies (desmoid, Ewing, rhabdomyosarcoma, nonrhabdomyosarcoma-soft tissue sarcomas) were reviewed to evaluate the relationship between end-organ radiation dose, clinical factors, and the risk of subsequent late effects (scoliosis, pneumonitis)...
April 26, 2017: Practical Radiation Oncology
https://www.readbyqxmd.com/read/28672777/correction-the-parp-inhibitor-olaparib-enhances-the-sensitivity-of-ewing-sarcoma-to-trabectedin
#16
José Luis Ordóñez, Ana Teresa Amaral, Angel M Carcaboso, David Herrero-Martín, María Del Carmen García-Macías, Vicky Sevillano, Diego Alonso, Guillem Pascual-Pasto, Laura San-Segundo, Monica Vila-Ubach, Telmo Rodrigues, Susana Fraile, Cristina Teodosio, Agustín Mayo-Iscar, Miguel Aracil, Carlos María Galmarini, Oscar M Tirado, Jaume Mora, Enrique de Álava
No abstract text is available yet for this article.
June 27, 2017: Oncotarget
https://www.readbyqxmd.com/read/28671673/ews-fli1-perturbs-mrtfb-yap-1-tead-target-gene-regulation-inhibiting-cytoskeletal-autoregulatory-feedback-in-ewing-sarcoma
#17
A M Katschnig, M O Kauer, R Schwentner, E M Tomazou, C N Mutz, M Linder, M Sibilia, J Alonso, D N T Aryee, H Kovar
Ewing sarcoma (EWS) is a paediatric bone cancer with high metastatic potential. Cellular plasticity resulting from dynamic cytoskeletal reorganization, typically regulated via the Rho pathway, is a prerequisite for metastasis initiation. Here, we interrogated the role of the Ewing sarcoma driver oncogene EWS-FLI1 in cytoskeletal reprogramming. We report that EWS-FLI1 strongly represses the activity of the Rho-F-actin signal pathway transcriptional effector MRTFB, affecting the expression of a large number of EWS-FLI1-anticorrelated genes including structural and regulatory cytoskeletal genes...
July 3, 2017: Oncogene
https://www.readbyqxmd.com/read/28670073/ewing-s-sarcoma-of-the-trachea-in-an-adolescent-girl
#18
Jaisankar Puthusseri, Geetha Narayanan, T R Preethi, G Jayapriya
Primitive neuroectodermal tumors (PNET) are aggressive neoplasms of neuroectodermal origin. Although they are known to arise in a host of locations, involvement of the trachea has rarely been reported. We describe an adolescent girl who presented with stridor and was diagnosed with PNET of the trachea. She is in remission following treatment with combination chemotherapy and local radiotherapy.
July 2017: Proceedings of the Baylor University Medical Center
https://www.readbyqxmd.com/read/28670029/diagnostic-performance-of-magnetic-resonance-imaging-in-the-assessment-of-periosteal-reactions-in-bone-sarcomas-using-conventional-radiography-as-the-reference
#19
José Luiz de Sá Neto, Marcelo Novelino Simão, Michel Daoud Crema, Edgard Eduard Engel, Marcello Henrique Nogueira-Barbosa
OBJECTIVE: To evaluate the performance of magnetic resonance imaging (MRI) in detecting periosteal reactions and to compare MRI and conventional radiography (CR) in terms of the classification of periosteal reactions. MATERIALS AND METHODS: Retrospective study of 42 consecutive patients (mean age, 22 years; 20 men) with a confirmed diagnosis of osteosarcoma or Ewing's sarcoma, MRI and CR images having been acquired pretreatment. Three blinded radiologists detected periosteal reactions and evaluated each periosteal reaction subtype in CR and MRI images: Codman's triangle; laminated; and spiculated...
May 2017: Radiologia Brasileira
https://www.readbyqxmd.com/read/28660821/major-haemorrhage-requiring-transarterial-embolisation-following-open-biopsy-of-an-unusual-neck-mass
#20
M Walsh, M Supriya, N Railton
A 65-year-old man presented with a right supraclavicular neck mass and right arm pain. Magnetic resonance imaging revealed a 96mm lesion in the upper thoracic paraspinal region extending into the deep supraclavicular fossa. The presentation was consistent with a sarcoma or lymphoma but fine needle aspiration was inconclusive. During open biopsy of the lesion, the patient had a rapid intraoperative haemorrhage of 1l from the tumour. Haemostasis could only be achieved by transarterial embolisation of the feeding vessel and the biopsy result confirmed Ewing's sarcoma...
July 2017: Annals of the Royal College of Surgeons of England
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