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Ewing sarcoma origination

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https://www.readbyqxmd.com/read/29227121/-what%C3%A2-s%C3%A2-new-in-ewing-like-sarcoma-family-soft-tissue-and-bone-sarcomas-with-cic-bcor-rearrangement-review-of-the-literature-and-first-personal-experience
#1
Zdeněk Kinkor, Petr Grossmann, Magdaléna Dubová, David Bludovský, Andrea Černá, Lenka Krsková, Petr Lhoták
The literature is reviewed regarding of a rare molecularly defined group of sarcomas with rearrangement of both CIC and BCOR genes, which were originally placed into the EWSR1wt Ewing-like category. Personal experience with three cases demonstrating difficulties of this issue is added. Both groups of lesions differ not only by age and topography, but also vary in both the prognostic and the predictive parameters. CIC-rearranged tumors are very aggressive and almost never occur in the skeleton; in contrary, the BCOR-rearranged ones are predominantly bone tumors in young males behaving even better than classical Ewing sarcoma...
2017: Ceskoslovenská Patologie
https://www.readbyqxmd.com/read/29225486/desmoplastic-small-round-blue-cell-tumor-a-review-of-treatment-and-potential-therapeutic-genomic-alterations
#2
REVIEW
Ajaz Bulbul, Bridget Noel Fahy, Joanne Xiu, Sadaf Rashad, Asrar Mustafa, Hatim Husain, Andrea Hayes-Jordan
Desmoplastic small round blue cell tumors (DSRCTs) originate from a cell with multilineage potential. A molecular hallmark of DSRCT is the EWS-WT1 reciprocal translocation. Ewing sarcoma and DSRCT are treated similarly due to similar oncogene activation pathways, and DSRCT has been represented in very limited numbers in sarcoma studies. Despite aggressive therapy, median survival ranges from 17 to 25 months, and 5-year survival rates remain around 15%, with higher survival reported among those undergoing removal of at least 90% of tumor in the absence of extraperitoneal metastasis...
2017: Sarcoma
https://www.readbyqxmd.com/read/28928879/-18-f-fdg-pet-ct-as-an-indicator-of-survival-in-ewing-sarcoma-of-bone
#3
Usama Salem, Behrang Amini, Hubert H Chuang, Najat C Daw, Wei Wei, Tamara Miner Haygood, John E Madewell, Colleen M Costelloe
Objective: The existing literature of 18 F-FDG PET/CT in Ewing sarcoma investigates mixed populations of patients with both soft tissue and bone primary tumors. The aim of our study was to evaluate whether the maximum standardized uptake value (SUVmax) obtained with 18F-FDG PET/CT before and after induction chemotherapy can be used as an indicator of survival in patients with Ewing sarcoma originating exclusively in the skeleton. Materials and Methods: A retrospective database search from 2004-2011 identified 28 patients who underwent 18 F-FDG PET/CT before (SUV1, n= 28) and after (SUV2, n=23) induction chemotherapy...
2017: Journal of Cancer
https://www.readbyqxmd.com/read/28882943/tissue-directed-implantation-using-ultrasound-visualization-for-development-of-biologically-relevant-metastatic-tumor-xenografts
#4
Raelene A VAN Noord, Tina Thomas, Melanie Krook, Sahiti Chukkapalli, Mark J Hoenerhoff, Jonathan R Dillman, Elizabeth R Lawlor, Valerie P Opipari, Erika A Newman
BACKGROUND: Advances in cancer therapeutics depend on reliable in vivo model systems. To develop biologically relevant xenografts, ultrasound was utilized for tissue-directed implantation of neuroblastoma (NB) cell line and patient-derived tumors in the adrenal gland, and for renal subcapsular engraftment of Ewing's sarcoma (ES). MATERIALS AND METHODS: NB xenografts were established by direct adrenal injection of luciferase-transfected NB cell lines (IMR32, SH-SY5Y, SK-N-BE2) or NB patient-derived tumor cells (UMNBL001, UMNBL002)...
September 2017: In Vivo
https://www.readbyqxmd.com/read/28844303/calcaneal-ewing-s-sarcoma-with-skip-metastases-to-tarsals-and-lymph-node-involvement-a-case-report
#5
Koray Şahin, Serkan Bayram, Ahmet Salduz
Ewing's sarcoma (ES) represents the second most common primary malignant tumor of bone of children and occurs rarely in the bones of the foot. Dissemination to regional lymph nodes and skip metastases to adjacent bones are thought to be uncommon. We report a case of a 19-year-old female with the diagnosis of ES of the right calcaneus. Six months earlier, she had presented to the hospital with a history of ankle sprain and was treated with analgesics and ice application. Despite the treatment, the pain over the ankle persisted, her foot swelled progressively, and a mass evolved on the lateral side of the foot...
August 22, 2017: Journal of Foot and Ankle Surgery: Official Publication of the American College of Foot and Ankle Surgeons
https://www.readbyqxmd.com/read/28716733/ml327-induces-apoptosis-and-sensitizes-ewing-sarcoma-cells-to-tnf-related-apoptosis-inducing-ligand
#6
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...
September 16, 2017: Biochemical and Biophysical Research Communications
https://www.readbyqxmd.com/read/28680140/generation-of-novel-patient-derived-cic-dux4-sarcoma-xenografts-and-cell-lines
#7
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/28670073/ewing-s-sarcoma-of-the-trachea-in-an-adolescent-girl
#8
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/28630763/malignant-tumours-of-the-foot-and-ankle
#9
E Mascard, N Gaspar, L Brugières, C Glorion, S Pannier, A Gomez-Brouchet
Most of tumours of the foot are tumour-like (synovial cyst, foreign body reactions and epidermal inclusion cyst) or benign conditions (tenosynovial giant cells tumours, planta fibromatosis). Malignant tumours of the soft-tissue and skeleton are very rare in the foot and their diagnosis is often delayed with referral to specialised teams after initial inappropriate procedures or unplanned excisions. The adverse effect of these misdiagnosed tumours is the increasing rate of amputation or local recurrences in the involved patients...
May 2017: EFORT Open Reviews
https://www.readbyqxmd.com/read/28508996/hyalinizing-clear-cell-carcinoma-of-the-bronchus
#10
Maryam Shahi, Michelle Dolan, Paari Murugan
Hyalinizing clear cell carcinoma (HCCC) is an uncommon low-grade minor salivary gland neoplasm that usually arises in the head and neck region. We report a 55-year-old man who presented with a 2.5 cm lung mass that was partially obstructing the right bronchus intermedius. The tumor consisted of cords and nests of clear and eosinophilic cells in a hyalinized stromal background. The neoplastic cells expressed cytokeratin (CK) 7, CK 5/6, high-molecular weight cytokeratin (34BE12), p63 and p40, while TTF-1, napsin A, CK20, S100, smooth muscle actin, synaptophysin and chromogranin were negative...
May 16, 2017: Head and Neck Pathology
https://www.readbyqxmd.com/read/28474974/primary-spinal-epidural-cic-dux4-undifferentiated-sarcoma-in-a-child
#11
John E Donahue, Evgeny Yakirevich, Shan Zhong, Diana O Treaba, Nelli S Lakis, Siraj M Ali, Suzanne M de la Monte, Shamlal Mangray
Primitive round- or spindle-cell EWSR1-negative undifferentiated sarcomas harboring CIC-DUX4 gene fusion are the most common form of Ewing-like sarcomas. These tumors primarily occur in peripheral soft tissues, but examples have been described within viscera and the brain. As far as we are aware, CIC-DUX4 positive primary epidural spinal sarcoma has not been reported. Herein, we describe a T5-T6 epidural tumor in a 15-year-old girl in which many neoplastic cells had moderate and focally abundant cytoplasm, including plasmacytoid or rhabdoid cells, rather than the more common Ewing-like morphology described in the majority of such tumors...
January 1, 2017: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/28472564/entering-the-double-digits-ewing-sarcoma-in-adult-maxilla
#12
Mala Kamboj, Anju Devi, Virendra Singh, Sunita Singh
Ewing sarcoma is a rare malignant pediatric sarcoma of bone and soft tissues, which is more unusual in head and neck region. Although the exact histogenesis is still unknown, various cells have been proposed as cells of origin namely, endothelial, hematopoietic, fibroblastic, mesenchymal stem cells or neural derived mesenchymal stem cells. ES is more common in younger age group and among the gnathic bones, mandible is affected more than maxilla. This paper presents the first case of Ewing Sarcoma in the maxilla being reported in the adult age group from India and among the very few reported worldwide...
May 2017: Journal of Experimental Therapeutics & Oncology
https://www.readbyqxmd.com/read/28293503/near-complete-humerus-reconstruction-in-the-pediatric-patient-with-vascularized-free-fibula-transfer
#13
John Shuck, Benjamin C Wood, Christopher Zarella, Albert K Oh, Robert M Henshaw, Gary F Rogers
Vascularized free fibula transfer remains the gold standard for reconstruction of large segmental diaphyseal defects of the upper extremity. In the pediatric patient, before skeletal maturity, free fibula transfer with the fibular head provides an active physis for growth and an articular interface for glenohumeral joint reconstruction. Clinical and cadaveric studies have demonstrated that the vascular supply to the fibular head originates, in most cases, from the anterior tibial system. However, anatomical variation exists, and we report a case in which a vascularized fibula autograft including the physis was transferred on the peroneal artery in a 5-year-old patient with Ewing's sarcoma...
December 2016: Plastic and Reconstructive Surgery. Global Open
https://www.readbyqxmd.com/read/28289810/non-odontogenic-tumors-of-the-facial-bones-in-children-and-adolescents-role-of-multiparametric-imaging
#14
REVIEW
Minerva Becker, Salvatore Stefanelli, Anne-Laure Rougemont, Pierre Alexandre Poletti, Laura Merlini
Tumors of the pediatric facial skeleton represent a major challenge in clinical practice because they can lead to functional impairment, facial deformation, and long-term disfigurement. Their treatment often requires a multidisciplinary approach, and radiologists play a pivotal role in the diagnosis and management of these lesions. Although rare, pediatric tumors arising in the facial bones comprise a wide spectrum of benign and malignant lesions of osteogenic, fibrogenic, hematopoietic, neurogenic, or epithelial origin...
April 2017: Neuroradiology
https://www.readbyqxmd.com/read/28129857/discovering-novel-oncogenic-pathways-and-new-therapies-using-zebrafish-models-of-sarcoma
#15
REVIEW
M N Hayes, D M Langenau
Sarcoma is a type of cancer affecting connective, supportive, or soft tissue of mesenchymal origin. Despite rare incidence in adults (<1%), over 15% of pediatric cancers are sarcoma. Sadly, both adults and children with relapsed or metastatic disease have devastatingly high rates of mortality. Current treatment options for sarcoma include surgery, radiation, and/or chemotherapy; however, significant limitations exist with respect to the efficacy of these strategies. Strong impetus has been placed on the development of novel therapies and preclinical models for uncovering mechanisms involved in the development, progression, and therapy resistance of sarcoma...
2017: Methods in Cell Biology
https://www.readbyqxmd.com/read/28060373/small-round-blue-cell-tumors-of-the-sinonasal-tract-a-differential-diagnosis-approach
#16
Lester Dr Thompson
One of the most challenging diagnostic categories within tumors of the sinonasal tract is the small round blue cell tumors. Biopsies are usually small and limited, resulting in considerable diagnostic difficulty for practicing surgical pathologists. These tumors share several overlapping histologic and immunophenotypic findings while also showing considerable variation within and between cases. Specific tumor site of origin, imaging findings, and clinical findings must be combined with the histology and pertinent ancillary studies if the correct diagnosis is to be reached...
January 2017: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
https://www.readbyqxmd.com/read/27920700/primary-ewing-sarcoma-primitive-neuroectodermal-tumor-of-the-stomach
#17
Safi Khuri, Hayim Gilshtein, Sa'd Sayidaa, Bishara Bishara, Yoram Kluger
Ewing sarcoma/primitive neuroectodermal tumor (ES/PNET) is a tumor of small round cells arising in skeletal tissues. These tumors rarely arise in the stomach. We present a 31-year-old healthy female patient who was admitted to our surgical ward due to upper gastrointestinal hemorrhage. Upper endoscopy revealed a large ulcerated bleeding mass originating from the lesser curvature. Biopsy revealed tumor cell immunoreactivity positive for CD99, vimentin, and Ki67 (an index of proliferation). These findings were compatible with gastric ES/PNET...
September 2016: Case Reports in Oncology
https://www.readbyqxmd.com/read/27913051/erg-expression-in-multiple-myeloma-a-potential-diagnostic-pitfall
#18
Juliana Knief, Katharina Reddemann, Jan Gliemroth, Swantje Brede, Tobias Bartscht, Christoph Thorns
INTRODUCTION: ERG expression has been described as a frequent event in prostate cancer indicating poor prognosis and promoting oncogenesis. It has also been demonstrated in Ewing's sarcoma, acute myeloid leukemia and acute T-lymphoblastic leukemia but could not be found in other epithelial tumors, Hodgkin's or Non-Hodgkin's lymphoma. We aimed to analyze ERG expression in multiple myeloma, following an index case of a patient with metastases of unknown origin in the spine strongly expressing ERG, which were thought to be of prostatic origin but turned out to be plasmacytic lesions...
February 2017: Pathology, Research and Practice
https://www.readbyqxmd.com/read/27898190/primary-ewing-s-sarcoma-of-the-sinonasal-tract-in-adults-a-challenging-disease
#19
Davide Lombardi, Davide Mattavelli, Luca O Redaelli De Zinis, Remo Accorona, Maria L Morassi, Fabio Facchetti, Vittorio Ferrari, Davide Farina, Rossella Bertulli, Piero Nicolai
BACKGROUND: Sinonasal localization of Ewing's sarcoma in adults is an exceedingly rare event. METHODS: The clinical records of 5 patients with primary sinonasal Ewing's sarcoma treated from 1992 to 2012 were retrospectively analyzed. All pathologic slides were reviewed by 2 experienced pathologists. All patients underwent multimodality treatments. RESULTS: Median age was 36 years (range, 25-52 years). At referral, 2 patients had the original diagnosis changed by review of the histologic slides...
November 29, 2016: Head & Neck
https://www.readbyqxmd.com/read/27751600/pediatric-soft-tissue-tumor-pathology-a-happy-morpho-molecular-union
#20
REVIEW
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...
November 2016: Seminars in Diagnostic Pathology
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