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Ewing sarcoma

Maryam Abolhasani, Sareh Salarinejad, Mohammad Kazem Moslemi
INTRODUCTION: Ewing sarcoma/Primitive neuroectodermal tumor of the kidney (ES/PNET) is a member of Ewing's sarcoma family, occurring in young adults and has aggressive clinical behavior and poor prognosis. However, its discrimination from the renal cell carcinoma (RCC) is very difficult preoperatively. We present three cases of this rare disease that were managed in two academic centers. PRESENTATION OF CASES: Herein we report three cases of ES/PNET of the kidney, 2 young men complaining of right flank pain and gross hematuria and one young woman complaining of left subcostal pain...
October 18, 2016: International Journal of Surgery Case Reports
Miguel Flores, Anthony Caram, Edward Derrick, John D Reith, Laura Bancroft, Kurt Scherer
Ewing sarcoma (ES) is a primary malignant bone tumor which most commonly arises in children and young adults. The common clinical presentation with ES includes nighttime pain or pain related to activity, though patients may also present with a combination of localized swelling, a palpable mass, pathologic fracture, and constitutional symptoms. Clinical diagnosis may be delayed when a patient presents with clinical or imaging findings that overlap with non-malignant etiologies, such as fibrous dysplasia (FD) or osteomyelitis...
September 18, 2016: Curēus
Tao He, Didier Surdez, Juha K Rantala, Saija Haapa-Paananen, Jozef Ban, Maximilian Kauer, Eleni Tomazou, Vidal Fey, Javier Alonso, Heinrich Kovar, Olivier Delattre, Kristiina Iljin
A translocation leading to the formation of an oncogenic EWS-ETS fusion protein defines Ewing sarcoma. The most frequent gene fusion, present in 85 percent of Ewing sarcomas, is EWS-FLI1. Here, a high-throughput RNA interference screen was performed to identify genes whose function is critical for EWS-FLI1 driven cell viability. In total, 6781 genes were targeted by siRNA molecules and the screen was performed both in presence and absence of doxycycline-inducible expression of the EWS-FLI1 shRNA in A673/TR/shEF Ewing sarcoma cells...
October 16, 2016: Gene
Alberto Caballero Vázquez, Paula García Flores, Ángela Herrera Chilla
No abstract text is available yet for this article.
October 15, 2016: Archivos de Bronconeumología
M Beth McCarville, Sara M Federico, Michael W Bishop, Alberto S Pappo, Barry L Shulkin
No abstract text is available yet for this article.
November 2016: Radiology
Nana Zhang, Guanjun Yue, Yan Zhang, Jiangfeng You, Hua Wang
[This corrects the article DOI: 10.1371/journal.pone.0153546.].
2016: PloS One
Christopher M Runyan, Vishal Sharma, David A Staffenberg, Jamie P Levine, Lawrence E Brecht, Leonard H Wexler, David L Hirsch
BACKGROUND: Reconstruction of maxillary defects following tumor extirpation is challenging because of combined aesthetic and functional roles of the maxilla. One-stage reconstruction combining osseous free flaps with immediate osseointegrated implants are becoming the standard for mandibular defects, and have similar potential for maxillary reconstruction. METHODS: A woman with maxillary Ewing sarcoma successfully treated at age 9 with neoadjuvant chemotherapy, right hemimaxillectomy, and obturator prosthetic reconstruction presented for definitive reconstruction, complaining of poor obturator fit, and hypernasality...
October 14, 2016: Journal of Craniofacial Surgery
Wei Wan, Yan Lou, Zhiqi Hu, Ting Wang, Jinsong Li, Yu Tang, Zhipeng Wu, Leqin Xu, Xinghai Yang, Dianwen Song, Jianru Xiao
Little information has been published in the literature regarding survival outcomes of patients with Ewing's sarcoma family tumors (ESFTs) of the spine. The purpose of this study is to explore factors that may affect the prognosis of patients with non-metastatic spinal ESFTs. A retrospective analysis of survival outcomes was performed in patients with non-metastatic spinal ESFTs. Univariate and multivariate analyses were employed to identify prognostic factors for recurrence and survival. Recurrence-free survival (RFS) and overall survival (OS) were defined as the date of surgery to the date of local relapse and death...
October 17, 2016: Journal of Neuro-oncology
Maureen O'Sullivan
Since its foundation by remarkably talented and insightful individuals, prominently including Pepper Dehner, pediatric soft tissue tumor pathology has developed at an immense rate. The morphologic classification of tumoral entities has extensively been corroborated, but has also evolved with refinement or realignment of these classifications, through accruing molecular data, with many derivative ancillary diagnostic assays now already well-established. Tumors of unclear histogenesis, classically morphologically undifferentiated, are prominent amongst pediatric sarcomas, however, the classes of undifferentiated round- or spindle-cell-tumors-not-otherwise-specified are being dismantled gradually with the identification of their molecular underpinnings...
September 5, 2016: Seminars in Diagnostic Pathology
Hongjiu Yu, Yonggui Ge, Lianying Guo, Lin Huang
Ewing's sarcoma (ES) is a highly aggressive and metastatic tumor in children and young adults caused by a chromosomal fusion between the Ewing sarcoma breakpoint region 1 (EWSR1) gene and the transcription factor FLI1 gene. ES is managed with standard treatments, including chemotherapy, surgery and radiation. Although the 5-year survival rate for primary ES has improved, the survival rate for ES patients with metastases or recurrence remains low. Several novel molecular targets in ES have recently been identified and investigated in preclinical and clinical settings, and targeting the function of receptor tyrosine kinases (RTKs), the fusion protein EWS-FLI1 and mTOR has shown promise...
October 11, 2016: Oncotarget
Tahereh Javaheri, Zahra Kazemi, Jan Pencik, Ha Tt Pham, Maximilian Kauer, Rahil Noorizadeh, Barbara Sax, Harini Nivarthi, Michaela Schlederer, Barbara Maurer, Maximillian Hofbauer, Dave Nt Aryee, Marc Wiedner, Eleni M Tomazou, Malcolm Logan, Christine Hartmann, Jan P Tuckermann, Lukas Kenner, Mario Mikula, Helmut Dolznig, Aykut Üren, Günther H Richter, Florian Grebien, Heinrich Kovar, Richard Moriggl
Ewing sarcoma (ES) is the second most frequent childhood bone cancer driven by the EWS/FLI1 (EF) fusion protein. Genetically defined ES models are needed to understand how EF expression changes bone precursor cell differentiation, how ES arises and through which mechanisms of inhibition it can be targeted. We used mesenchymal Prx1-directed conditional EF expression in mice to study bone development and to establish a reliable sarcoma model. EF expression arrested early chondrocyte and osteoblast differentiation due to changed signaling pathways such as hedgehog, WNT or growth factor signaling...
October 13, 2016: Cell Death & Disease
Ian J Robertson, Fadel Bennani, Ronan S Ryan, Waqar Khan, M Kevin Barry
No abstract text is available yet for this article.
October 11, 2016: Diagnostic Pathology
Snehal J, Suresh Sr
No abstract text is available yet for this article.
January 2016: Journal of the Association of Physicians of India
Matthias Benndorf, Jakob Neubauer, Mathias Langer, Elmar Kotter
PURPOSE: In the diagnostic process of primary bone tumors, patient age, tumor localization and to a lesser extent sex affect the differential diagnosis. We therefore aim to develop a pretest probability calculator for primary malignant bone tumors based on population data taking these variables into account. METHODS: We access the SEER (Surveillance, Epidemiology and End Results Program of the National Cancer Institute, 2015 release) database and analyze data of all primary malignant bone tumors diagnosed between 1973 and 2012...
October 8, 2016: International Journal of Computer Assisted Radiology and Surgery
Huma Mansoori, Bushra Moiz
No abstract text is available yet for this article.
September 2016: Blood Research
Abha Soni, Shi Wei
Primary renal Ewing sarcoma is extremely rare and carries a dismal prognosis. It is particularly diagnostically challenging due to its ability to mimic other round cell tumors. Herein we report such a case in a 28-year-old white female. The diagnosis was confirmed by immunophenotyping, fluorescence in-situ hybridization and RT-PCR analyses.
October 6, 2016: Urology
Jason A Jarzembowski
Dr. Louis "Pepper" Dehner is an internationally renowned surgical pathologist, especially in the subspecialty of pediatric pathology. Although his clinical and academic expertise are broad, with over 400 published articles, some of his most intriguing contributions have been in the area of pediatric renal and genitourinary pathology. This review focuses on the entities in these following organ systems where he has focused his efforts: malignant rhabdoid tumor, renal medullary carcinoma, Ewing sarcoma/peripheral neuroectodermal tumor, and the DICER1-related lesions cystic nephroma, embryonal rhabdomyosarcoma of the uterine cervix, and Sertoli-Leydig cell tumor...
August 31, 2016: Seminars in Diagnostic Pathology
Ivan Zokalj, Jasminka Igrec, Antonio Plesnar
INTRODUCTION: Primitive neuroectodermal tumor (PNET)/Ewing's sarcoma (EWS) belongs to a family of neoplasms that are presumed to originate from the neuroectodermal crest. PNET/EWSs are highly aggressive malignancies that usually present in the form of bone or soft tissue masses and usually affect adolescents and young adults. Primary PNET/EWS of the kidney is very rare. CASE PRESENTATION: We present the case of a 32-year-old female patient with primary renal PNET/EWS diagnosed nine years and eight months earlier...
July 2016: Nephro-urology Monthly
Jorge Hernando-Cubero, Pilar Sanz-Moncasi, Alba Hernández-García, Isabel Pajares-Bernard, Javier Martínez-Trufero
The Ewing's sarcoma family of tumors (ESFT) comprises a number of rare malignant tumors. Standard first-line treatment for patients with these tumors includes chemotherapy with a five-drug regimen of vincristine, doxorubicin (Adriamycin(®)) and cyclophosphamide, alternating with ifosfamide and etoposide (VAC/IE). In cases of inadequate response, there are a number of second-line regimens available. However, further treatment options are required for those patients with disease unresponsive to standard treatment...
October 2016: Oncology Letters
Matt L Harlow, Nichole Maloney, Joseph Roland, María José Guillén-Navarro, Matthew K Easton, Susan M Kitchen-Goosen, Elissa A Boguslawski, Zachary B Madaj, Ben K Johnson, Megan J Bowman, Maurizio D'Incalci, Mary E Winn, Lisa Turner, Galen Hostetter, Carlos M Galmarini, Pablo M Aviles, Patrick J Grohar
There is a great need to develop novel approaches to target oncogenic transcription factors with small molecules. Ewing sarcoma is emblematic of this need, as it depends on the continued activity of the EWS-FLI1 transcription factor to maintain the malignant phenotype. We have previously shown that the small molecule trabectedin interferes with EWS-FLI1. Here we report important mechanistic advances and a second-generation inhibitor to provide insight into the therapeutic targeting of EWS-FLI1. We discovered that trabectedin functionally inactivated EWS-FLI1 by redistributing the protein within the nucleus to the nucleolus...
October 3, 2016: Cancer Research
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