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Ewings sarcoma

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https://www.readbyqxmd.com/read/29152060/inhibition-of-chk1-sensitizes-ewing-sarcoma-cells-to-the-ribonucleotide-reductase-inhibitor-gemcitabine
#1
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
#2
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
https://www.readbyqxmd.com/read/29148079/insm1-a-novel-nuclear-marker-in-merkel-cell-carcinoma-cutaneous-neuroendocrine-carcinoma
#3
Patrick S Rush, Jason N Rosenbaum, Madhuchhanda Roy, Rebecca M Baus, Daniel D Bennett, Ricardo V Lloyd
Merkel cell carcinoma (MCC) is a rare, clinically aggressive, cutaneous neuroendocrine (NE) neoplasm. As a tumor with small, round, blue cells, the histologic differential diagnosis for MCC can include melanoma, metastatic small cell carcinoma (SCC), nodular hematopoietic tumors, basal cell carcinoma (BCC), atypical variants of squamous carcinoma, and the uncommon occurrence of primary cutaneous Ewing sarcoma. In cases with atypical histology or without the classic immunophenotype the diagnosis can be challenging...
November 17, 2017: Journal of Cutaneous Pathology
https://www.readbyqxmd.com/read/29143983/growth-of-doxorubicin-resistant-undifferentiated-spindle-cell-sarcoma-pdox-is-arrested-by-metabolic-targeting-with-recombinant-methioninase
#4
Kentaro Igarashi, Shukuan Li, Qinghong Han, Yuying Tan, Kei Kawaguchi, Takashi Murakami, Tasuku Kiyuna, Kentaro Miyake, Yunfeng Li, Scott D Nelson, Sarah M Dry, Arun S Singh, Irmina A Elliott, Tara A Russell, Mark A Eckardt, Norio Yamamoto, Katsuhiro Hayashi, Hiroaki Kimura, Shinji Miwa, Hiroyuki Tsuchiya, Fritz C Eilber, Robert M Hoffman
Undifferentiated spindle-cell sarcoma (USCS) is a recalcitrant -cancer in need of individualized therapy. A high-grade USCS from a striated muscle of a patient was grown orthotopically in the right biceps femoris muscle of nude mice to establish a patient-derived orthotopic xenograft (PDOX) model. In a previous study, we evaluated the efficacy of standard first-line chemotherapy of doxorubicin (DOX), gemcitabine (GEM) combined with docetaxel (DOC), compared to pazopanib (PAZ), a multi-targeting tyrosine-kinase inhibitor, in an USCS PDOX model...
November 16, 2017: Journal of Cellular Biochemistry
https://www.readbyqxmd.com/read/29136686/-clinicopathologic-and-molecular-characteristics-of-malignant-gastrointestinal-neuroectodermal-tumors
#5
M Zhao, T W Zhao, J Ma, C Y Wu, L Chen, G Q Ru, X L He
Objective: To investigate the clinicopathologic and molecular characteristics, diagnostic, differential diagnostic and prognostic features of malignant gastrointestinal neuroectodermal tumor. Methods: Two cases of malignant gastrointestinal neuroectodermal tumor were retrieved; the clinical and radiologic features, histomorphology, immunophenotype, molecular genetics and prognosis were analyzed and the relevant literature reviewed. Results: Case 1 was a 57-year-old male, presented with recurrent abdominal pain and melena...
November 8, 2017: Zhonghua Bing Li Xue za Zhi Chinese Journal of Pathology
https://www.readbyqxmd.com/read/29124298/can-fdg-pet-ct-replace-blind-bone-marrow-biopsy-of-the-posterior-iliac-crest-in-ewing-sarcoma
#6
Ömer Kasalak, Andor W J M Glaudemans, Jelle Overbosch, Paul C Jutte, Thomas C Kwee
OBJECTIVE: To determine and compare the value of (18)F-fluoro-2-deoxy-D-glucose positron emission tomography/computed tomography (FDG-PET/CT) to blind bone marrow biopsy (BMB) of the posterior iliac crest in detecting metastatic bone marrow involvement in newly diagnosed Ewing sarcoma. MATERIALS AND METHODS: This retrospective study included 20 patients with newly diagnosed Ewing sarcoma who underwent pretreatment FDG-PET/CT and a total of 38 blind BMBs (two unilateral and 18 bilateral) of the posterior iliac crest...
November 9, 2017: Skeletal Radiology
https://www.readbyqxmd.com/read/29121995/round-cell-sarcoma-of-the-colon-with-cic-rearrangement
#7
H Maghrebi, R Batti, A Zehani, N Chrait, H Rais, A Makni, A Haddad, M Ayadi, A Daghfous, M Jrad, N Kchir, Z Bensafta, A Mezlini
BACKGROUND: The CIC-rearranged sarcoma is a very rare highly aggressive malignant soft tissue group of tumors. It has recently been described as highly aggressive soft tissue tumors of children and young adults sharing similar morphological features with the Ewing sarcoma. The digestive localization is exceptional. CASE PRESENTATION: A 14-year-old male presented with a history of abdominal pain for 1 year, which increased in intensity over the last 2 months. Imaging findings showed a large heterogeneous mesenteric mass on the left flank of the abdomen...
November 9, 2017: BMC Research Notes
https://www.readbyqxmd.com/read/29117845/restoration-of-the-active-forearm-rotation-using-vascularized-free-fibular-graft-and-radial-head-arthroplasty-for-a-wide-defect-of-the-proximal-radius
#8
Ryosuke Sato, Yoshitaka Hamada, Naohito Hibino, Toshihiko Nishisho, Ichiro Tonogai, Kenji Endo, Koichi Sairyo
In this report we present a case of re-stabilization of the proximal radius using vascularized free fibular graft combined with anconeus arthroplasty to stabilize the transplanted fibula in a patient with large loss of the proximal radius due to Ewing's sarcoma. A 20-year-old woman had Ewing's sarcoma in the proximal three-fourths of the radius. The resection of large part of the radius containing radial head were necessary for removal of the sarcoma. Reconstruction of the proximal radius was performed using vascularized free fibular graft and the transplanted fibula was stabilized by attaching the anconeus to the proximal edge of the fibula...
December 2017: Journal of Hand Surgery Asian-Pacific Volume
https://www.readbyqxmd.com/read/29117758/primary-tumor-volume-measurements-in-ewing-sarcoma-mri-inter-and-intraobserver-variability-and-comparison-with-fdg-pet
#9
Ömer Kasalak, Jelle Overbosch, Andor W J M Glaudemans, Ronald Boellaard, Paul C Jutte, Thomas C Kwee
BACKGROUND: Primary tumor volume is as an important and independent prognostic factor in Ewing sarcoma. However, the observer variability of magnetic resonance imaging (MRI)-based primary tumor volume measurements in newly diagnosed Ewing sarcoma has never been investigated. Furthermore, it is unclear how MRI-based volume measurements compare to (18)F-fluoro-2-deoxy-D-glucose (FDG) positron emission tomography (PET)-based volume measurements. This study aimed to determine the observer variability of simplified MRI-based primary tumor volume measurements in newly diagnosed treatment-naive Ewing sarcoma and to compare them to the actual primary tumor volume at MRI and the FDG-PET-based metabolically active tumor volume (MATV)...
November 9, 2017: Acta Oncologica
https://www.readbyqxmd.com/read/29114282/multicompartmental-primary-spinal-extramedullary-tumors-value-of-an-interdisciplinary-approach
#10
Guruprasad Bettaswamy, Paurush Ambesh, Raj Kumar, Rabi Narayan Sahu, Kuntal Kanti Das, Awadhesh Kumar Jaiswal, Arun Kumar Srivastava, Sanjay Behari
Background: Primary extramedullary tumors involving multiple compartments around the spine are a technically demanding group of tumors whose extent traverses beyond the normal confines of those anatomical regions which fall in the common domain of neurosurgeons. In the following series, we present 12 patients who were diagnosed with primary spinal extramedullary tumors with multicompartmental extension, and whose surgical management was facilitated by a combined multidisciplinary approach involving surgeons of other superspecialties...
October 2017: Asian Journal of Neurosurgery
https://www.readbyqxmd.com/read/29113210/analysis-of-imaging-characteristics-of-primary-malignant-bone-tumors-in-children
#11
Yingwei Sun, Xueyong Liu, Shinong Pan, Chunbo Deng, Xiaohan Li, Qiyong Guo
The present study aimed to investigate the imaging characteristics of primary malignant bone tumors in children. The imaging results of 34 children with primary malignant bone tumors confirmed by histopathological diagnosis between March 2008 and January 2014 were retrospectively analyzed. In total, 25 patients had osteosarcoma, with radiography and computed tomography (CT) showing osteolytic bone destruction or/and osteoblastic bone sclerosis, an aggressive periosteal reaction, a soft-tissue mass and cancerous bone...
November 2017: Oncology Letters
https://www.readbyqxmd.com/read/29108279/size-based-detection-of-sarcoma-circulating-tumor-cells-and-cell-clusters
#12
Masanori Hayashi, Peixuan Zhu, Gregory McCarty, Christian F Meyer, Christine A Pratilas, Adam Levin, Carol D Morris, Catherine M Albert, Kyle W Jackson, Cha-Mei Tang, David M Loeb
Metastatic disease is the most important factor in determining the survival of sarcoma patients. Since sarcoma metastasis is predominantly hematogenous, we hypothesized that detection and quantification of circulating tumor cells (CTCs) could reflect response to therapy and risk of metastatic relapse. We evaluated the presence of CTCs using a novel animal model and in the blood of patients with high grade sarcomas utilizing the CellSieve™ size-based low pressure microfiltration system. Sarcoma CTCs were identified based on antibody staining patterns and nuclear morphology...
October 3, 2017: Oncotarget
https://www.readbyqxmd.com/read/29108227/inhibition-of-porcupine-prolongs-metastasis-free-survival-in-a-mouse-xenograft-model-of-ewing-sarcoma
#13
Masanori Hayashi, Alissa Baker, Seth D Goldstein, Catherine M Albert, Kyle W Jackson, Gregory McCarty, Ulf D Kahlert, David M Loeb
The most pressing unmet clinical need for patients with Ewing sarcoma (ES) is the prevention and treatment of metastasis. The Wnt signaling pathway regulates a number of cellular functions associated with metastasis, including proliferation, motility, and stem cell self-renewal. Functional interaction between Wnt ligands and their receptors requires palmitoylation by Porcupine (Porcn), making this an ideal therapeutic target. We studied the effect of WNT974, a potent, selective Porcn inhibitor, on ES metastasis...
October 3, 2017: Oncotarget
https://www.readbyqxmd.com/read/29107752/staging-and-follow-up-of-a-ewing-sarcoma-patient-using-18-f-fdg-pet-ct
#14
J R Garcia, A Castañeda, A Morales La Madrid, P Bassa, M Soler, E Riera
No abstract text is available yet for this article.
October 26, 2017: Revista Española de Medicina Nuclear e Imagen Molecular
https://www.readbyqxmd.com/read/29104083/development-and-evaluation-of-a-pan-sarcoma-fusion-gene-detection-assay-using-the-nanostring-ncounter-platform
#15
Kenneth T E Chang, Angela Goytain, Tracy Tucker, Aly Karsan, Cheng-Han Lee, Torsten O Nielsen, Tony L Ng
The NanoString nCounter assay is a high-throughput hybridization technique using target-specific probes that can be customized to test for numerous fusion transcripts in a single assay using RNA from formalin-fixed, paraffin-embedded material. We designed a NanoString assay targeting 174 unique fusion junctions in 25 sarcoma types. The study cohort comprised 212 cases, 96 of which showed fusion gene expression by the NanoString assay, including all 20 Ewing sarcomas, 11 synovial sarcomas, and 5 myxoid liposarcomas tested...
November 17, 2017: Journal of Molecular Diagnostics: JMD
https://www.readbyqxmd.com/read/29100387/metabolic-modulation-of-ewing-sarcoma-cells-inhibits-tumor-growth-and-stem-cell-properties
#16
Atreyi Dasgupta, Matteo Trucco, Nino Rainusso, Ronald J Bernardi, Ryan Shuck, Lyazat Kurenbekova, David M Loeb, Jason T Yustein
Ewing sarcoma (EWS) is a highly aggressive and metabolically active malignant tumor. Metabolic activity can broadly be characterized by features of glycolytic activity and oxidative phosphorylation. We have further characterized metabolic features of EWS cells to identify potential therapeutic targets. EWS cells had significantly more glycolytic activity compared to their non-malignant counterparts. Thus, metabolic inhibitors of glycolysis such as 2-deoxy-D-glucose (2DG) and of the mitochondrial respiratory pathway, such as metformin, were evaluated as potential therapeutic agents against a panel of EWS cell lines in vitro...
September 29, 2017: Oncotarget
https://www.readbyqxmd.com/read/29100369/initial-fdg-pet-ct-predicts-survival-in-adults-ewing-sarcoma-family-of-tumors
#17
Bastien Jamet, Thomas Carlier, Loic Campion, Emmanuelle Bompas, Sylvie Girault, Fanny Borrely, Ludovic Ferrer, Maxime Rousseau, Yann Venel, Françoise Kraeber-Bodéré, Caroline Rousseau
Purpose: The aim of this retrospective study was to determine, at baseline, the prognostic value of different FDG-PET/CT quantitative parameters in a homogenous Ewing Sarcoma Family of Tumors (ESFT) adult population, compared with clinically relevant prognostic factors. Methods: Adult patients from 3 oncological centers, all with proved ESFT, were retrospectively included. Quantitative FDG-PET/CT parameters (SUV (maximum, peak and mean), metabolic tumor volume (MTV) and total lesion glycolysis (TLG) of the primary lesion of each patient were recorded before treatment, as well as usual clinical prognostic factors (stage of disease, location, tumor size, gender and age)...
September 29, 2017: Oncotarget
https://www.readbyqxmd.com/read/29093183/therapeutic-targeting-of-pgbd5-induced-dna-repair-dependency-in-pediatric-solid-tumors
#18
Anton G Henssen, Casie Reed, Eileen Jiang, Heathcliff Dorado Garcia, Jennifer von Stebut, Ian C MacArthur, Patrick Hundsdoerfer, Jun Hyun Kim, Elisa de Stanchina, Yasumichi Kuwahara, Hajime Hosoi, Neil J Ganem, Filemon Dela Cruz, Andrew L Kung, Johannes H Schulte, John H Petrini, Alex Kentsis
Despite intense efforts, the cure rates of childhood and adult solid tumors are not satisfactory. Resistance to intensive chemotherapy is common, and targets for molecular therapies are largely undefined. We have found that the majority of childhood solid tumors, including rhabdoid tumors, neuroblastoma, medulloblastoma, and Ewing sarcoma, express an active DNA transposase, PGBD5, that can promote site-specific genomic rearrangements in human cells. Using functional genetic approaches, we discovered that mouse and human cells deficient in nonhomologous end joining (NHEJ) DNA repair cannot tolerate the expression of PGBD5...
November 1, 2017: Science Translational Medicine
https://www.readbyqxmd.com/read/29091716/identification-of-two-types-of-ggaa-microsatellites-and-their-roles-in-ews-fli-binding-and-gene-regulation-in-ewing-sarcoma
#19
Kirsten M Johnson, Cenny Taslim, Ranajeet S Saund, Stephen L Lessnick
Ewing sarcoma is a bone malignancy of children and young adults, frequently harboring the EWS/FLI chromosomal translocation. The resulting fusion protein is an aberrant transcription factor that uses highly repetitive GGAA-containing elements (microsatellites) to activate and repress thousands of target genes mediating oncogenesis. However, the mechanisms of EWS/FLI interaction with microsatellites and regulation of target gene expression is not clearly understood. Here, we profile genome-wide protein binding and gene expression...
2017: PloS One
https://www.readbyqxmd.com/read/29084055/satb2-and-tle1-expression-in-bcor-ccnb3-ewing-like-sarcoma-mimicking-small-cell-osteosarcoma-and-poorly-differentiated-synovial-sarcoma
#20
David Creytens
No abstract text is available yet for this article.
October 27, 2017: Applied Immunohistochemistry & Molecular Morphology: AIMM
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