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Ewing sarcoma origination

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https://www.readbyqxmd.com/read/29616903/sinonasal-ewing-sarcoma-a-case-report-and-literature-review
#1
Jonathan K Lin, Jonathan Liang
INTRODUCTION: Ewing sarcoma (ES) of the sinonasal tract and associated primitive neuroectodermal tumors are rare. To our knowledge, only 10 case reports of sinonasal ES of the nose or paranasal sinuses have been reported. Furthermore, there has been only 1 case of sinonasal ES arising from the middle turbinate. Recommended management of sinonasal ES stems from the management of its osseous counterpart, ES, but treatment with surgery, radiotherapy, and chemotherapy is varied. Five-year survival rates vary from 21% to 70%, with the lower rates representing patients presenting with metastatic disease...
March 7, 2018: Permanente Journal
https://www.readbyqxmd.com/read/29544549/ewing-sarcoma-of-the-adrenal-gland-a-case-report-and-review-of-the-literature
#2
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
https://www.readbyqxmd.com/read/29529639/what-are-the-complications-of-allograft-reconstructions-for-sarcoma-resection-in-children-younger-than-10-years-at-long-term-followup
#3
Luis A Aponte-Tinao, Jose I Albergo, Miguel A Ayerza, D Luis Muscolo, Federico Milano Ing, German L Farfalli
BACKGROUND: Preservation of limb function after resection of malignant bone tumors in skeletally immature children is challenging. Resection of bone sarcomas and reconstruction with an allograft in patients younger than 10 years old is one reconstructive alternative. However, long-term studies analyzing late complications and limb length discrepancy at skeletal maturity are scarce; this information would be important, because growth potential is altered in these patients owing to the loss of one physis during tumor resection...
March 2018: Clinical Orthopaedics and related Research
https://www.readbyqxmd.com/read/29491357/ectomesenchymal-chondromyxoid-tumor-a-comprehensive-updated-review-of-the-literature-and-case-report
#4
Astrid Truschnegg, Stephan Acham, Lumnije Kqiku, Norbert Jakse, Alfred Beham
Prompted by a unique case of an ectomesenchymal chondromyxoid tumor (ECT) of the palate in a 54-year-old female, we reviewed the English and German literature on this entity until the end of 2016 using PubMed. The search produced 74 lingual cases with a nearly equal sex distribution and a mean age of 39.3 years, and two extra-lingual cases sharing histological and immunohistological features including nodular growth, round, fusiform or spindle-shaped cellular architecture, and chondromyxoid stroma. Immunophenotyping showed the majority of cases to be positive for glial fibrillary acidic protein (GFAP), S-100 protein, glycoprotein CD57, pancytokeratin (AE1/AE3), and smooth muscle actin (SMA); in isolated cases there was molecular-genetic rearrangement or gain of Ewing sarcoma breakpoint region 1 (EWSR1) but no rearrangement of pleomorphic adenoma gene 1 (PLAG1)...
February 28, 2018: International Journal of Oral Science
https://www.readbyqxmd.com/read/29484083/adrenal-mass-of-unusual-etiology-ewing-sarcoma-in-a-young-man
#5
Levent Soydan, Ali Aslan Demir, Elif Sayman, Burcu Onomay Celik, Bala Basak Oven Ustaalioglu
Ewing sarcoma and peripheral primitive neuroectodermal tumor belong to the Ewing sarcoma (ES) family of tumors originating from a primitive neural tube. We report a 31-year-old man who was admitted to the urology clinic with complaints of fever, nausea, and dysuria. A right-sided adrenal mass was detected during ultrasonography. The lesion was then evaluated with magnetic resonance imaging, which showed areas of necrosis amid heterogeneous solid areas. Whole body scan with 2-deoxy-2-[fluorine-18]fluoro-D-glucose integrated with computed tomography and bone scan studies showed pulmonary and osseous metastatic foci...
December 2017: Radiology Case Reports
https://www.readbyqxmd.com/read/29440819/intravenous-lignocaine-infusion-for-intractable-pain-in-ewing-s-sarcoma
#6
Nivedita Page
A 23-year-old female presented to our palliative care center with Ewing's sarcoma of the humerus with lung metastases. Pain in her arm was unrelieved by nonsteroidal anti-inflammatory drugs, neuropathic medication as well as morphine. She could not tolerate any further increase in opioid dose but was so distraught due to the pain that she wanted to die. An intravenous lignocaine infusion in a dose of 2 mg/kg was given over an hour for three successive days. This successfully relieved her pain after which she was settled with her original medication...
January 2018: Indian Journal of Palliative Care
https://www.readbyqxmd.com/read/29397557/targeting-histone-deacetylase-activity-to-arrest-cell-growth-and-promote-neural-differentiation-in-ewing-sarcoma
#7
Bárbara Kunzler Souza, Patrícia Luciana da Costa Lopez, Pâmela Rossi Menegotto, Igor Araujo Vieira, Nathalia Kersting, Ana Lúcia Abujamra, André T Brunetto, Algemir L Brunetto, Lauro Gregianin, Caroline Brunetto de Farias, Carol J Thiele, Rafael Roesler
There is an urgent need for advances in the treatment of Ewing sarcoma (EWS), an aggressive childhood tumor with possible neuroectodermal origin. Inhibition of histone deacetylases (HDAC) can revert aberrant epigenetic states and reduce growth in different experimental cancer types. Here, we investigated whether the potent HDAC inhibitor, sodium butyrate (NaB), has the ability to reprogram EWS cells towards a more differentiated state and affect their growth and survival. Exposure of two EWS cell lines to NaB resulted in rapid and potent inhibition of HDAC activity (1 h, IC50 1...
February 3, 2018: Molecular Neurobiology
https://www.readbyqxmd.com/read/29387511/extraosseous-ewing-s-sarcoma-of-the-pancreas-an-uncommon-but-treatable-disease
#8
Muhammad W Saif, Kristin Kaley
Extraosseous Ewing's sarcoma/primitive neuroectodermal tumor (ES/PNET) is a rare but aggressive and malignant tumor, and has been reported in various sites such as the lungs, biliary tract, kidney, prostate, stomach, esophagus, oral cavity, salivary glands, urinary bladder, uterus, cervix, gonads, and vagina. However, the pancreas is considered to be an extremely uncommon site and only a handful of cases have been published to date. We present here another case of a pancreatic ES/PNET. Our case intensifies the importance to recognize this rare type of tumor in the pancreas as there is a broad spectrum of tumors with a similar morphology that includes sheets of small, round blue cells...
November 26, 2017: Curēus
https://www.readbyqxmd.com/read/29387307/bone-allograft-segment-covered-with-a-vascularized-fibular-periosteal-flap-a-new-technique-for-pediatric-mandibular-reconstruction
#9
Nicolas E Sierra, Paula Diaz-Gallardo, Jorge Knörr, Vasco Mascarenhas, Eloy García-Diez, Montserrat Munill-Ferrer, Maria S Bescós-Atín, Francisco Soldado
The free vascularized fibular graft is nowadays the preferred technique for pediatric mandibular reconstruction. Despite the versatility and proven efficacy for restoring the facial appearance and maxillomandibular function, those mandibular reconstructions with free vascularized fibula associate difficulties for a simultaneous restoration of the alveolar height and facial contour, which are derived from the height discrepancy between the fibula and the native mandible. In addition, the donor-site growth and morbidity are of special concern in the pediatric patient...
March 2018: Craniomaxillofacial Trauma & Reconstruction
https://www.readbyqxmd.com/read/29361725/chondrosarcoma-a-rare-misfortune-in-aging-human-cartilage-the-role-of-stem-and-progenitor-cells-in-proliferation-malignant-degeneration-and-therapeutic-resistance
#10
REVIEW
Karen A Boehme, Sabine B Schleicher, Frank Traub, Bernd Rolauffs
Unlike other malignant bone tumors including osteosarcomas and Ewing sarcomas with a peak incidence in adolescents and young adults, conventional and dedifferentiated chondrosarcomas mainly affect people in the 4th to 7th decade of life. To date, the cell type of chondrosarcoma origin is not clearly defined. However, it seems that mesenchymal stem and progenitor cells (MSPC) in the bone marrow facing a pro-proliferative as well as predominantly chondrogenic differentiation milieu, as is implicated in early stage osteoarthritis (OA) at that age, are the source of chondrosarcoma genesis...
January 21, 2018: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/29313488/replication-study-systematic-identification-of-genomic-markers-of-drug-sensitivity-in-cancer-cells
#11
John P Vanden Heuvel, Ewa Maddox, Samar W Maalouf, Elizabeth Iorns, Rachel Tsui, Alexandria Denis, Nicole Perfito, Timothy M Errington
In 2016, as part of the Reproducibility Project: Cancer Biology, we published a Registered Report (Vanden Heuvel et al., 2016), that described how we intended to replicate selected experiments from the paper 'Systematic identification of genomic markers of drug sensitivity in cancer cells' (Garnett et al., 2012). Here we report the results. We found Ewing's sarcoma cell lines, overall, were more sensitive to the PARP inhibitor olaparib than osteosarcoma cell lines; however, while the effect was in the same direction as the original study (Figure 4C; Garnett et al...
January 9, 2018: ELife
https://www.readbyqxmd.com/read/29285586/intracranial-ewing-sarcoma-four-pediatric-examples
#12
Michael J Yang, Ros Whelan, Jennifer Madden, Jean M Mulcahy Levy, B K Kleinschmidt-DeMasters, Todd C Hankinson, Nicholas K Foreman, Michael H Handler
BACKGROUND: Ewing sarcoma typically arises in bone and is unrelated to intraparenchymal small blue cell embryonal central nervous system (CNS) tumors previously designated primitive neuroectodermal tumors (PNETs). When the CNS is impacted, it is usually secondary to local extension from either the epidural space, skull, or intracranial or spinal metastases. Primary examples within the cranial vault are rare, usually dural-based, and are largely case reports in the literature. We detail four pediatric patients with solitary, primary intracranial Ewing sarcoma, all manifesting the archetypal EWRS1 gene rearrangement that confirms diagnosis...
March 2018: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/29227121/-what%C3%A2-s-new-in-ewing-like-sarcoma-family-soft-tissue-and-bone-sarcomas-with-cic-bcor-rearrangement-review-of-the-literature-and-first-personal-experience
#13
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
#14
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
#15
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
#16
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
#17
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...
January 2018: 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
#18
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
#19
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
#20
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
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