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Ewing sarcoma origination

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
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
Masako Ishiguro, Mutsumi Yuki, Tomoko Fukushige, Mikio Mizoguchi, Yasuhiko Kaneko, Takeshita Morishige, Hiroshi Iwasaki
Ewing's sarcoma/primitive neuroectodermal tumor/Askin's tumor (Ewing`s sarcoma family of tumors: ESFT) is the most common type of malignant tumor of bone and soft tissue in children and young adults, and morphologically is a member of a group of small round cell tumors. We report, here, on the establishment of two human ESFT cell lines, FU-PNET-3 and FU-PNET-4, from the iliac and the chest wall, respectively, the cells of both cell lines were tumorigenic in immunodeficient mice. Histologically, both original and xenograft tumors and cultured cells were composed of small round cells with positive immunoreactivity for CD99 and Nkx2...
September 9, 2016: Human Cell
Andrea Ferrari, Uta Dirksen, Stefan Bielack
The definition of soft tissue and bone sarcomas include a large group of several heterogeneous subtypes of mesenchymal origin that may occur at any age. Among the different sarcomas, rhabdomyosarcoma, synovial sarcoma, Ewing sarcoma and osteosarcoma are aggressive high-grade malignancies that often arise in adolescents and young adults. Managing these malignancies in patients in this age bracket poses various clinical problems, also because different therapeutic approaches are sometimes adopted by pediatric and adult oncologists, even though they are dealing with the same condition...
2016: Progress in Tumor Research
M M Dolmans, Y Iwahara, J Donnez, M Soares, J L Vaerman, C A Amorim, H Poirel
STUDY QUESTION: What is the risk of finding malignant cells in cryopreserved ovarian tissue from sarcoma patients? SUMMARY ANSWER: Minimal disseminated disease (MDD) was not detected in frozen-thawed ovarian tissue from 26 patients by any of the sensitive methods applied. WHAT IS KNOWN ALREADY: In case of leukemia, the risk of malignant cell transmission through the graft is well known and widely documented. However, for bone cancer, like Ewing sarcoma or osteosarcoma, only a small number of case reports, have been published...
October 2016: Human Reproduction
Camille Jacques, Lidia Rodriguez Calleja, Marc Baud'huin, Thibaut Quillard, Dominique Heymann, François Lamoureux, Benjamin Ory
Osteosarcoma and Ewing Sarcoma are the two most common types of Bone Sarcomas, principally localized at the long bones of the extremities and mainly affecting adolescents and young adults. Cisplatin is one of the current options in the therapeutic arsenal of drugs available to cure these aggressive cancers. Unfortunately, chemoresistance against this agent is still a major cause of patient relapse. Thus, a better understanding of the molecular pathways by which these drugs induce cancer cell death, together with a better delineation of the origins of chemoresistance are required to improve the success rate of current treatments...
July 29, 2016: Oncotarget
Song-Feng Xu, Xiu-Chun Yu, Ming Xu, Yong-Cheng Hu, Xiao-Ping Liu
OBJECTIVES: To demonstrate the functional results and emotional acceptance after scapulectomy for various malignant shoulder tumors. METHODS: Eight patients with malignant shoulder tumors who had undergone scapulectomy between April 2004 and March 2014 were retrospectively reviewed. They comprised seven men and one woman their mean age was 54 years (range, 24-69 years). All patients were diagnosed by pathological examination of biopsy specimens. The tumors were metastatic in four cases, having originated from a primary carcinoma of the liver in one patient, the lung in one patient and the kidney in two patients...
May 2016: Orthopaedic Surgery
Yang Zhang, Hongsheng Li, Zongjuan Li, Ming Liu, Linke Yang, Liyuan Fan, Chengsuo Huang, Baosheng Li
Ewing sarcomas (ES) and peripheral primitive neuroectodermal tumors (pPNET) are now thought to belong to the same tumor family. Ewing sarcoma family tumor (ESFT) members commonly originate in bones and soft tissues. However, a few published articles describe ESFT arising from cranial cavities. Pathologically, ES/pPNET are composed of small round cells. Unambiguous distinction between pPNET and other small round cell tumors, in particular central PNET, is of clinical significance. Definitive diagnoses of pPNET can be obtained through CD99 (MIC2 gene product) membrane positivities and molecular identifications of chromosomal rearrangements between EWS and ETS family genes...
2016: OncoTargets and Therapy
Inmaculada Hernandez-Muñoz, Elisabeth Figuerola, Sara Sanchez-Molina, Eva Rodriguez, Ana Isabel Fernández-Mariño, Carlos Pardo-Pastor, María Isabel Bahamonde, José M Fernández-Fernández, Daniel J García-Domínguez, Lourdes Hontecillas-Prieto, Cinzia Lavarino, Angel M Carcaboso, Carmen de Torres, Oscar M Tirado, Enrique de Alava, Jaume Mora
Ewing sarcoma (ES) is an aggressive tumor defined by EWSR1 gene fusions that behave as an oncogene. Here we demonstrate that RING1B is highly expressed in primary ES tumors, and its expression is independent of the fusion oncogene. RING1B-depleted ES cells display an expression profile enriched in genes functionally involved in hematological development but RING1B depletion does not induce cellular differentiation. In ES cells, RING1B directly binds the SCN8A sodium channel promoter and its depletion results in enhanced Nav1...
June 15, 2016: Oncotarget
Hiroto Kawano, Naoki Nitta, Mitsuaki Ishida, Tadateru Fukami, Kazuhiko Nozaki
BACKGROUND: Primary Ewing's sarcoma originating in the pericranium is an extremely rare disease entity. CASE DESCRIPTION: A 9-year-old female patient was admitted to our department due to a left temporal subcutaneous mass. The mass was localized under the left temporal muscle and attached to the surface of the temporal bone. Head computed tomography revealed a mass with bony spicule formation on the temporal bone, however, it did not show bone destruction or intracranial invasion...
2016: Surgical Neurology International
Thomas Cash, Elizabeth McIlvaine, Mark D Krailo, Stephen L Lessnick, Elizabeth R Lawlor, Nadia Laack, Joel Sorger, Neyssa Marina, Holcombe E Grier, Linda Granowetter, Richard B Womer, Steven G DuBois
BACKGROUND: The prognostic significance of having extraskeletal (EES) versus skeletal Ewing sarcoma (ES) in the setting of modern chemotherapy protocols is unknown. The purpose of this study was to compare the clinical characteristics, biologic features, and outcomes for patients with EES and skeletal ES. METHODS: Patients had localized ES and were treated on two consecutive protocols using five-drug chemotherapy (INT-0154 and AEWS0031). Patients were analyzed based on having an extraskeletal (n = 213) or skeletal (n = 826) site of tumor origin...
October 2016: Pediatric Blood & Cancer
Wan-Shan Li, I-Chuang Liao, Mei-Chin Wen, Howard Haw-Chang Lan, Shih-Chen Yu, Hsuan-Ying Huang
AIMS: BCOR-CCNB3 sarcoma is a genetically defined undifferentiated malignancy with Ewing sarcoma (ES)-like round cells and preferentially affects the bones of male adolescents. Sarcomas harbouring BCOR-CCNB3 rarely arise from soft tissues; therefore, we reported four cases to expand the clinicopathological spectrum. METHODS AND RESULTS: Through RT-PCR and confirmatory sequencing, we detected a BCOR-CCNB3 transcript in primary undifferentiated sarcomas of the deep musculature of four male patients, comprising two teenagers (14 and 17 y) and two adults (34 and 44 y)...
May 26, 2016: Histopathology
Anita P Javalgi, Mahesh H Karigoudar, Katyayani Palur
Ewing's sarcoma is a highly malignant tumour of osseous or non-osseous origin, tremed as extra-skeletal Ewings sarcoma if arising from soft tissue. It is rare occurrence tumor most commonly occurring in paravertebral area, chest wall, head & neck and retroperitoneum. Reporting an interesting case of retroperitoneal Ewing's sarcoma in 39 years old female. Patient had complains of abdominal discomfort & vague pain since 2 months, following weakness in lower limb and loss of weight. On detail history and examination she was further referred to detail pathological and radiological investigations...
April 2016: Journal of Clinical and Diagnostic Research: JCDR
George Galyfos, Georgios A Karantzikos, Nikolaos Kavouras, Argiri Sianou, Konstantinos Palogos, Konstantinos Filis
Extraosseous Ewing sarcomas (EESs) are rare tumours originating from soft tissues. Their clinical picture depends mainly on the primary site of the sarcoma. Patient characteristics and outcomes seem to be different in EES compared to patients with skeletal Ewing sarcoma, with implications for patient care and prognosis. However, multimodality therapeutic strategies are recommended for all types of the Ewing tumour family. The available diagnostic tools include ultrasonographic evaluation and computed tomography (CT) or magnetic resonance imaging as well as histopathologic and immunohistochemical tissue examination...
February 2016: Indian Journal of Surgery
Michael J Wagner, J Andrew Livingston, Shreyaskumar R Patel, Robert S Benjamin
The largest studies of chemotherapy for bone sarcomas are in the pediatric population. Although increasing age is often found to be an adverse prognostic factor in these clinical trials, few studies are aimed at assessing regimens specifically in the adult population. Osteosarcoma and Ewing sarcoma have peak incidences in the pediatric and young adult population but also occur in adults. Chondrosarcoma and giant cell tumor of bone are generally found in adults. In this review, we describe the current status of our knowledge about treating adults with cancers of bone origin...
March 2016: Journal of Oncology Practice
Kei Ijichi, Toyonori Tsuzuki, Makoto Adachi, Shingo Murakami
Primitive neuroectodermal tumors (PNETs) are malignant tumors comprised of small round cells of neuroectodermal origin. Current evidence indicates that peripheral PNETs (pPNETs), which arise in the non-central nervous system, possess histological similarity to Ewing's sarcoma. Though the occurrence of pPNETs in the head and neck region is rare, these are aggressive malignant tumors, and long-term survival rates following diagnosis remain poor. The current report presents a case of pPNET and evaluates its significance with regard to previous studies...
February 2016: Oncology Letters
Huijuan Xiao, Fengchang Bao, Hongna Tan, Bo Wang, Wei Liu, Jianbo Gao, Xianzheng Gao
OBJECTIVE: To describe the clinical, CT and pathological findings of paediatric peripheral primitive neuroectodermal tumours (pPNETs) to enhance the recognition of these rare tumours. METHODS: The clinical, CT and pathological findings of 18 paediatric patients with pPNETs confirmed by biopsy or surgical pathology were retrospectively reviewed. RESULTS: The age of these 18 paediatric patients with pPNETs ranged from 4 months to 15 years, with a mean age of 7...
2016: British Journal of Radiology
David J Elzi, Meihua Song, Peter J Houghton, Yidong Chen, Yuzuru Shiio
Ewing sarcoma is a cancer of bone and soft tissue in children that is characterized by a chromosomal translocation involving EWS and an Ets family transcription factor, most commonly FLI-1. The EWS-FLI-1 fusion oncogene is widely believed to play a central role in Ewing sarcoma. The EWS-FLI-1 gene product regulates the expression of a number of genes important for cancer progression, can transform mouse cells such as NIH3T3 and C3H10T1/2, and is necessary for proliferation and tumorigenicity of Ewing sarcoma cells, suggesting that EWS-FLI-1 is the causative oncogene...
November 2015: Genes & Cancer
Grigory A Raskin, Kazimir M Pozharisski, Aglaya G Iyevleva, Ivan V Rikov, Rashida V Orlova, Evgeny N Imyanitov
BACKGROUND: Use of molecular assays is gradually becoming a mandatory part of the clinical management of soft tissue tumors, however the choice and the interpretation of these tests may present a challenge. SUMMARY: This report demonstrates an unusual presentation of sarcoma, which was initially diagnosed as a tumor of unknown primary site. Given the presence of vimentin, Fli-1, CD99 and S100 markers, lack of immunostaining for melan A, HMB45, MITF, synaptophysin, CD56, myf4, CKAE1/3 and WT-1, as well as the presence of EWSR1 translocation determined by a break-apart FISH assay, Ewing's sarcoma (ES) diagnosis seemed to be well justified...
September 2015: Gastrointestinal Tumors
S Akbayram, M Başaranoglu, A Kaya, M Açıkgöz, L Üstyol, G A Taşkın, M Dogan
Ewing's sarcoma is one of the most aggressive primary bone tumours. Ewing's sarcoma arising from the bones of the head and neck region is extremely rare; only 4-9% of all Ewing's sarcoma originate in this region. We report a case of Ewing's sarcoma localized in the mandible because of its unusual presentation.
September 2015: West Indian Medical Journal
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