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Ewings sarcoma

Masanori Kawano, Kazuhiro Tanaka, Ichiro Itonaga, Tatsuya Iwasaki, Hiroshi Tsumura
Background: MicroRNAs (miRNAs) are endogenous, small non-coding RNAs that play important roles in multiple biological processes. Here, we show that miRNAs play an important function in the down-regulation of FAS expression in Ewing's sarcoma (ES) cells. Methods: To identify and characterize possible oncogenic factors in ES, we employed a microarray-based approach to profile the changes in the expression of miRNAs and their target mRNAs in five ES cell lines and human mesenchymal stem cells (hMSCs)...
2018: Cancer Cell International
Ilan Kent, Evgeny Edelstein, Olga Levin, Yaron Wiener
No abstract text is available yet for this article.
March 2018: Journal of Gastrointestinal and Liver Diseases: JGLD
Kristy Pilbeam, Hongbo Wang, Elizabeth Taras, Rachel J Bergerson, Brianna Ettestad, Todd DeFor, Antonella Borgatti, Daniel A Vallera, Michael R Verneris
Children with high risk sarcoma have a poor prognosis despite surgical resection, irradiation and chemotherapy. Alternative therapies are urgently needed. Urokinase-type plasminogen activator receptor (uPAR) and epidermal growth factor receptor (EGFR) are surface proteins expressed by some pediatric sarcomas. We show for the first time that a de-immunized bispecific ligand toxin, EGFATFKDEL, directed against EGFR and uPAR, successfully targets pediatric sarcoma. Using flow cytometry, we identified a rhabdomyosarcoma (RMS) cell line, RH30, that expresses both uPAR and EGFR, and a Ewing sarcoma (EWS) cell line, TC-71, that expresses only uPAR...
February 23, 2018: Oncotarget
Bastien Provost, Gilles Missenard, Ciprian Pricopi, Olaf Mercier, Sacha Mussot, Dominique Fabre, Nathaniel Langer, Olivier Mir, Cécile Le Pechoux, Philippe Dartevelle, Elie Fadel
BACKGROUND: Radiotherapy has long been the treatment of choice for local control of Ewing sarcoma of the chest wall (ESCW). However, there is debate regarding the use of surgery versus RT. Our objective was to identify risk factors that may affect long-term outcomes of non-metastatic ESCW all treated with preoperative chemotherapy followed by en-bloc resection and adjuvant Chemotherapy or Chemoradiation. METHODS: Between 1996 and 2014, 30 patients with a median age of 25 years (SD +/-8...
March 15, 2018: Annals of Thoracic Surgery
(no author information available yet)
Transcriptional dysregulation promotes R-loops and impairs homologous recombination in Ewing sarcoma.
March 16, 2018: Cancer Discovery
Danny Mangual, Luis A Bisbal-Matos, Ricardo Jiménez-Lee, Román Vélez, Miguel Noy
The case of a 27-year-old Hispanic female who presented with an occipito-parietal tumor after suffering trauma to the area. A physical examination revealed no tenderness to palpation and with evidence of healing ulcerations. The biopsy was consistent with a synovial sarcoma. A wide excision of the mass (15cm x 14cm x 6cm) followed by a pericranial flap was performed. A follow-up CT showed recurrence involving the parietal sagittal sinus. After a second biopsy the mass was determined to be a small-cell sarcoma, consistent with Ewing's sarcoma...
March 2018: Puerto Rico Health Sciences Journal
Kyriakos Paraskeva, Dimitrios Giakoumettis, Ioannis Nikas, George Georgoulis, George Sfakianos, Marios S Themistocleous
INTRODUCTION: Neck pain and torticollis are common symptoms in the pediatric population that rarely requires further investigation. However, in case symptoms persist, then a more meticulously approach should be considered. Underlying conditions such as infections, neck injury, autoimmune disorders or even cervical spine cancer should be excluded from diagnosis. Cervical spine cancer is a rare neurosurgical entity in the pediatric population and even rarer is atlantoaxial Ewing's sarcoma...
March 3, 2018: Neurologia i Neurochirurgia Polska
Hanane Eddaoualline, Khadija Mazouz, Bouchra Rafiq, Ghizlane El Mghari Tabib, Nawal El Ansari, Rhizlane Belbaraka, Abdelhamid El Omrani, Mouna Khouchani
BACKGROUND: Ewing sarcoma/primitive neuroectodermal tumor is a family of highly malignant proliferation of neuroectodermal origin, most often skeletal, adrenal localization is extremely rare. Only few cases have been reported in the literature. Classical management includes radical surgery with adjuvant chemotherapy or radiotherapy or both. This case report is the only one where recurrence was surgically removed, and it confirms the importance of adjuvant treatment, and the efficacy of neoadjuvant chemotherapy...
March 16, 2018: Journal of Medical Case Reports
Paula Marrano, Mary Shago, Gino R Somers, Paul S Thorner
Osteogenic sarcoma (OS) is the most common malignant bone tumor in children and adolescents. Despite advances in molecular genetic characterization of pediatric and adult tumors, the diagnosis of OS still depends almost entirely on light microscopy. The lack of consistent genetic changes in OS has greatly hindered the development of any diagnostic molecular test. Recently, whole-genome sequencing has shown that ~50% of cases of OS have a translocation involving the TP53 gene with breakpoints confined to the first intron...
March 14, 2018: American Journal of Surgical Pathology
Paul J Choi, Joe Iwanaga, R Shane Tubbs, Emre Yilmaz
Owing to its rarity, rhabdomyosarcoma of the head and neck (HNRMS) has seldom been discussed in the literature. As most of the data is based only on the retrospective experiences of tertiary healthcare centers, there are difficulties in formulating a standard treatment protocol. Moreover, the disease is poorly understood at its pathological, genetic, and molecular levels. For instance, 20% of all histological assessment is inaccurate; even an experienced pathologist can confuse rhabdomyosarcoma (RMS) with neuroblastoma, Ewing's sarcoma, and lymphoma...
January 9, 2018: Curēus
Conglin Ye, Xiaolong Yu, Xuqiang Liu, Min Dai, Bin Zhang
MicroRNAs (miRNAs) are small, single-stranded, non-coding RNA molecules involved in cancer initiation and progression. The present study aimed to determine the effect of miRNA-30d (miR-30d) on the growth, malignant phenotype, and apoptosis of Ewing's sarcoma (ES) SK-ES-1 cells, and to elucidate the underlying molecular mechanism and signaling pathway involved. Cell proliferation, invasion, migration, morphological changes, cell cycle distribution and apoptosis were investigated. Furthermore, the expression of matrix metalloproteinase (MMP)-2, MMP-9, B-cell lymphoma 2 (Bcl-2), Bcl-2-associated X protein (Bax), caspase-3 and poly (ADP-ribose) polymerase (PARP) were examined, as was the activity of the mitogen-activated protein kinase kinase (MEK)/extracellular signal-regulated kinase (ERK) and phosphoinositide 3-kinase (PI3K)/Akt pathways...
April 2018: Oncology Letters
Alexandra Okihiro, Rachana Hasija, Lillia Fung, Bonnie Cameron, Brian M Feldman, Ronald Laxer, Rayfel Schneider, Earl Silverman, Lynn Spiegel, Rae S M Yeung, Shirley M L Tse
BACKGROUND: Anti-TNF (Tumor necrosis factor) therapy is effective in treating pediatric patients with refractory rheumatic disease. There is however a concern that anti-TNF usage may increase the risk of malignancy. Reports on specific types of malignancy in this patient population have been emerging over the past decade, but there is a need for additional malignancy reports, as these events are rare. Therefore, a retrospective chart review was performed on the biologic database of pediatric rheumatology patients at The Hospital for Sick Children (SickKids) from 1997 to 2013 for neoplasms, patient demographic information and rheumatologic treatment course...
March 14, 2018: Pediatric Rheumatology Online Journal
Nathália Kersting, Bárbara Kunzler Souza, Igor Araujo Vieira, Rafael Pereira Dos Santos, Danielly Brufatto Olguins, Lauro José Gregianin, André Tesainer Brunetto, Algemir Lunardi Brunetto, Rafael Roesler, Caroline Brunetto de Farias, Gilberto Schwartsmann
OBJECTIVE: Ewing sarcoma (ES) is a type of childhood cancer probably arising from stem mesenchymal or neural crest cells. The epidermal growth factor receptor (EGFR) acts as a driver oncogene in many types of solid tumors. However, its involvement in ES remains poorly understood. METHODS: Human SK-ES-1 and RD-ES ES cells were treated with EGF, the EGFR inhibitor tyrphostin (AG1478), or phosphoinositide 3-kinase (PI3K) or extracellular-regulated kinase (ERK)/mitogen-activated kinase (MAPK) inhibitors...
March 14, 2018: Oncology
Wen-Tong Zhang, Wen-Wen Zhang, Zhen-Yu He, Jia-Yuan Sun, Lei Zhang, Qing Xia, San-Gang Wu
BACKGROUND: To investigate the optimal local treatment strategies for patients with non-metastatic Ewing sarcoma (ES) of bone. METHODS: Patients with ES of bone were identified using the Surveillance Epidemiology and End Results database. Kaplan-Meier log-rank test and Cox regression models were performed to assess the effect of the types of local treatment strategies on cause-specific survival and overall survival. RESULTS: 560 patients were included with a median age of 16 years...
March 14, 2018: Expert Review of Anticancer Therapy
Mary Carroll Shapiro, Tien Tang, Atreyi Dasgupta, Lyazat Kurenbekova, Ryan Shuck, M Waleed Gaber, Jason T Yustein
PURPOSE: Radiation therapy (RT) is a viable therapeutic option for Ewing sarcoma (ES) patients. However, little progress has been made to elucidate the mechanisms of radioresistance. This study establishes a novel ES irradiation-adapted model designed to assess molecular and18 F fluorodeoxyglucose (FDG) positron emission tomography (PET) alterations secondary to RT. METHODS AND MATERIALS: Radiation-adapted cell lines (RACLs) were created in vitro by exposing ES human cell lines to fractionated doses of radiation...
February 5, 2018: International Journal of Radiation Oncology, Biology, Physics
Abdullah Alholle, Marie Karanian, Anna T Brini, Mark R Morris, Vinodh Kannappan, Stefania Niada, Angela Niblett, Dominique Ranchère-Vince, Daniel Pissaloux, Christophe Delfour, Aurelie Maran Gonzalez, Cristina R Antonescu, Vaiyapuri Sumathi, Franck Tirode, Farida Latif
In recent years, undifferentiated small round cell sarcoma (USRCS) have been divided into a variety of new, rare, sarcoma subtypes including the group of Ewing-like sarcoma that has the morphological appearance of Ewing sarcoma but carries CIC-DUX4, BCOR-CCNB3 and other gene fusions different from the classical EWSR1-ETS. Using high-throughput RNA-seq analyses we identified a novel recurrent gene fusion, CRTC1-SS18, in two cases of USRCS that lacked any known translocation. RNA-seq results were confirmed by RT-PCR, long-range PCR and FISH...
March 13, 2018: Journal of Pathology
Isidro Machado, Carlos Santonja, Victoria Huerta, Julia Cruz, Celia Requena, Luis Requena, Antonio Llombart-Bosch
Neuroendocrine differentiation or aberrant expression of neuroendocrine markers is very uncommon in angiosarcomas (AS) and creates a challenging differential diagnosis with other superficial or soft tissue tumors. Herein, we report a new case of superficial AS presenting as a tumor lesion on the little finger of the right hand of a 52-year-old man. The tumor displayed CD56, chromogranin-A, and synaptophysin immunoreactivity. Tumor cells were positive for vascular markers (CD31, FLI1, ERG, D2-40, VE-cadherin, VEGR1,2, and 3), CD99, and EMA, but were negative for S100, CK (AE1/AE3), CK20, polyomavirus, and myogenic (desmin and myogenin) and melanocyte markers (melan-A and HMB45)...
February 27, 2018: American Journal of Dermatopathology
E Palmerini, R L Jones, E Setola, P Picci, E Marchesi, R Luksch, G Grignani, M Cesari, A Longhi, M E Abate, A Paioli, Z Szucs, L D'ambrosio, K Scotlandi, F Fagioli, S Asaftei, S Ferrari
BACKGROUND: Data on temozolomide (TEM) and irinotecan (IRI) activity in recurrent Ewing sarcoma (EWS), especially in adult patients, are limited. METHODS: Patients receiving TEM 100 mg/m2 /day oral, and IRI 40 mg/m2 /day intravenous, days 1-5, every 21 days, were included in this multi-institutional retrospective study. Disease control rate (DCR) [overall response rate (ORR) [complete response (CR) + partial response (PR)] + stable disease (SD)], 6-months progression-free survival (6-mos PFS) and 1-year overall survival (OS) were assessed...
March 13, 2018: Acta Oncologica
Christina Enciso Holm, Christian Bardram, Anja Falk Riecke, Peter Horstmann, Michael Mørk Petersen
PURPOSE: Previous studies reported variable outcome and failure rates after mega-prosthetic reconstructions in the lower extremities. The purpose of this study was to make a long-term single-center evaluation of patients treated with limb-sparing surgery and reconstruction with mega-prostheses in the lower extremities. METHODS: We identified 50 patients (osteosarcoma (n = 30), chondrosarcoma (n = 9), osteoclastoma (n = 6), Ewing sarcoma (n = 4), angiosarcoma (n = 1)), who underwent limb-sparing reconstruction of the lower extremities (proximal femur (n = 9), distal femur (n = 29), proximal tibia (n = 9), and the entire femur (n = 3)) between 1985 and 2005...
March 12, 2018: International Orthopaedics
Matthew J Thompson, John C Shapton, Stephanie E Punt, Christopher N Johnson, Ernest U Conrad
BACKGROUND: The quantitative accuracy of MRI in predicting the intraosseous extent of primary sarcoma of bone has not been definitively confirmed, although MRI is widely accepted as an accurate tool to plan limb salvage resections. Because inaccuracies in MRI determination of tumor extent could affect the ability of a tumor surgeon to achieve negative margins and avoid local recurrence, we thought it important to assess the accuracy of MR-determined tumor extent to the actual extent observed pathologically from resected specimens in pediatric patients treated for primary sarcomas of bone...
March 2018: Clinical Orthopaedics and related Research
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