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https://www.readbyqxmd.com/read/28724045/spinal-tumors-in-children
#1
Andrei Fernandes Joaquim, Enrico Ghizoni, Marcelo Gomes Cordeiro Valadares, Simone Appenzeller, Simone Dos Santos Aguiar, Helder Tedeschi
Introduction: Spinal tumors are rare in the pediatric population, presenting many specific peculiarities when compared to adults. We have performed a broad narrative review to describe the most common spinal tumors in children, discussing their main characteristics and management options. Method: The authors have performed an extensive review of the peer-reviewed literature addressing the aforementioned objectives. Results: Multimodality radiological studies (plain films, 3D computed tomography scan and magnetic resonance imaging) are necessary for proper evaluation and differential diagnosis of spinal tumors in children...
May 2017: Revista da Associação Médica Brasileira
https://www.readbyqxmd.com/read/28723616/prognostic-value-of-programmed-death-ligand-1-in-sarcoma-a-meta-analysis
#2
Zhenhua Zhu, Zheng Jin, Mei Zhang, Yajun Tang, Guang Yang, Xiaowei Yuan, Jihang Yao, Dahui Sun
BACKGROUND: The prognostic role of programmed death-ligand 1 (PD-L1) in sarcoma remains controversial. We performed a meta-analysis so as to investigate the impact of PD-L1 on clinicopathlogical findings and survival outcomes in sarcoma. MATERIALS AND METHODS: A comprehensive search in PubMed, Embase and the Cochrane Library was conducted for relevant studies. The odds ratios or hazard ratios, at 95% confidence intervals were used as measures for investigation of the correlation between PD-L1 expression and clinicopathlogical features or survival outcomes...
July 11, 2017: Oncotarget
https://www.readbyqxmd.com/read/28720588/ews-fli-is-a-master-regulator-of-metabolic-reprogramming-in-ewing-sarcoma
#3
Jason M Tanner, Claire Bensard, Peng Wei, Nathan M Krah, John C Schell, Jamie D Gardiner, Joshua D Schiffman, Stephen L Lessnick, Jared Rutter
Ewing sarcoma is a bone malignancy driven by a translocation event resulting in the fusion protein EWS/FLI1 (EF). EF functions as an aberrant and oncogenic transcription factor that misregulates the expression of thousands of genes. Previous work has focused principally on determining important transcriptional targets of EF, as well as characterizing important regulatory partnerships in EF-dependent transcriptional programs. Less is known, however, about EF-dependent metabolic changes or their role in Ewing sarcoma biology...
July 18, 2017: Molecular Cancer Research: MCR
https://www.readbyqxmd.com/read/28716733/ml327-induces-apoptosis-and-sensitizes-ewing-sarcoma-cells-to-tnf-related-apoptosis-inducing-ligand
#4
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...
July 14, 2017: Biochemical and Biophysical Research Communications
https://www.readbyqxmd.com/read/28713607/primary-osteosarcoma-of-the-breast-arising-in-an-intraductal-papilloma
#5
Khalefa Ali Alghofaily, Musab Hamoud Almushayqih, Muhannad Faleh Alanazi, Abdullah Abdulrahman Bin Salamah, Halldor Benediktsson
INTRODUCTION: Primary osteosarcoma of the breast is extremely rare, and an osteosarcoma arising from an intraductal papilloma is exceptional. CASE PRESENTATION: A 72-year-old Saudi Arabian woman presented with a solid, bone-containing breast mass that was diagnosed as primary osteosarcoma of the breast on biopsy. She had a history of untreated intraductal papilloma. Treatment was completed with a modified mastectomy after excluding extramammary metastases. However, she subsequently developed multiple recurrent lesions at the same site...
2017: Case Reports in Radiology
https://www.readbyqxmd.com/read/28699149/is-the-clavicula-pro-humero-technique-of-value-for-reconstruction-after-resection-of-the-proximal-humerus-in-children
#6
Dominique Barbier, Benoît De Billy, Philippe Gicquel, Sophie Bourelle, Pierre Journeau
BACKGROUND: There are several options for reconstruction of proximal humerus resections after wide resection for malignant tumors in children. The clavicula pro humero technique is a biologic option that has been used in the past, but there are only scant case reports and small series that comment on the results of the procedure. Because the longevity of children mandates a reconstruction with potential longevity not likely to be achieved by other techniques, the clavicula pro humero technique may be a potential option in selected patients...
July 11, 2017: Clinical Orthopaedics and related Research
https://www.readbyqxmd.com/read/28697683/metastatic-sclerosing-epithelioid-fibrosarcoma-in-bone-marrow
#7
Rana Asakra, Shane Zaidi, Khin Thway
Sclerosing epithelioid fibrosarcoma (SEF) is an aggressive neoplasm thought to be related to low-grade fibromyxoid sarcoma, which typically occurs in middle-aged adults in the deep soft tissues of the lower extremities and trunk. It comprises nests and cords of relatively uniform epithelioid polygonal cells with clear or eosinophilic cytoplasm in densely sclerotic stroma, and it is typically associated with EWSR1 gene rearrangements, and most commonly EWSR1-CREB3L1 fusions. As primary SEF can arise in bone, and bone is also a common metastatic site for SEF, its recognition at this site is important...
July 1, 2017: International Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28696044/practice-patterns-of-palliative-radiation-therapy-in-pediatric-oncology-patients-in-an-international-pediatric-research-consortium
#8
Avani Dholakia Rao, Qinyu Chen, Ralph P Ermoian, Sara R Alcorn, Maria Luisa S Figueiredo, Michael J Chen, Karin Dieckmann, Shannon M MacDonald, Matthew M Ladra, Daria Kobyzeva, Alexey V Nechesnyuk, Kristina Nilsson, Eric C Ford, Brian A Winey, Rosangela C Villar, Stephanie A Terezakis
BACKGROUND/OBJECTIVES: The practice of palliative radiation therapy (RT) is based on extrapolation from adult literature. We evaluated patterns of pediatric palliative RT to describe regimens used to identify opportunity for future pediatric-specific clinical trials. DESIGN/METHODS: Six international institutions with pediatric expertise completed a 122-item survey evaluating patterns of palliative RT for patients ≤21 years old from 2010 to 2015. Two institutions use proton RT...
July 11, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28694637/isolated-intracranial-myeloid-sarcoma-occurring-as-relapse-in-acute-myeloid-leukemia
#9
Geetha Narayanan, Lali V Soman, Lakshmi Haridas, Harish Sugathan
Myeloid sarcoma (MS) or chloroma is a rare extramedullary tumor composed of extramedullary proliferation of blasts of granulocytic, monocytic, erythroid, or megakaryocytic lineage occurring at sites outside the bone marrow. MS occurs in 2%-8% of patients with acute myeloid leukemia (AML), sometimes it occurs as the presenting manifestation of relapse in a patient in remission. We describe the case of a young male with AML in remission for 6 years presenting with central nervous system symptoms. Magnetic resonance imaging showed an extra-axial altered intensity lesion in the parasagittal parietal region, infiltrating anterosuperiorly into anterior falx, and posterosuperior aspect of the superior sagittal sinus...
July 2017: Journal of Neurosciences in Rural Practice
https://www.readbyqxmd.com/read/28690908/primary-occipital-ewing-s-sarcoma-with-subsequent-spinal-seeding
#10
Ali Alqahtani, Roaa Amer, Eman Bakhsh
Ewing's sarcoma is a primary bone cancer that mainly affects the long bones. This malignancy is particularly common in pediatric patients. Primary cranial involvement accounts for 1% of cases, with occipital involvement considered extremely rare. In this case study, primary occipital Ewing's sarcoma with a posterior fossa mass and subsequent relapse resulting in spinal seeding is reported. A 3-year-old patient presented with a 1-year history of left-sided headaches, localized over the occipital bone with progressive torticollis...
2017: Case Reports in Pediatrics
https://www.readbyqxmd.com/read/28688724/pd-1-and-pd-l1-expression-in-bone-and-soft-tissue-sarcomas
#11
Alireza Torabi, Clarissa N Amaya, Frank H Wians, Brad A Bryan
PD-1 and its ligands have been shown to play a significant role in evasion of malignant tumour cells from the immune system. Last year, the Unites States Food and Drug Administration (FDA) approved anti-PD-1 inhibitors for treatment of non-small cell lung carcinoma and recently expanded the use of immunotherapy for metastatic urothelial cell carcinoma and Hodgkin lymphoma. However, studies on expression of PD-1 and its ligand in malignant bone and soft tissue sarcoma are sparse. In this study, we evaluated PD-1 and PD-L1 expression on variants of liposarcomas and rhabdomyosarcomas, osteosarcomas and chondrosarcomas...
July 5, 2017: Pathology
https://www.readbyqxmd.com/read/28687689/persistent-sacral-chloroma-in-refractory-acute-myelogenous-leukaemia
#12
Scott Mathew McCarty, Dennis John Kuo
Acute myelogenous leukaemia (AML) is a clonal process involving the myeloid subgroup of white blood cells. Chloromas, or myeloid sarcomas, are masses of myeloid leukaemic cells and are a unique aspect of AML. This case involves a 14-year-old boy with AML who presented with multiple chloromas at diagnosis. The patient's extra-calvarial masses and bone marrow involvement responded to chemotherapy; however, his sacral epidural chloromas persisted despite four courses of chemotherapy. The central nervous system, bone marrow and testes have been known to be sanctuary sites for AML...
July 6, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28687009/post-surgery-fluids-promote-transition-of-cancer-stem-cell-to-endothelial-and-akt-mtor-activity-contributing-to-relapse-of-giant-cell-tumors-of-bone
#13
Flavio Fazioli, Gianluca Colella, Roberta Miceli, Mariano Giuseppe Di Salvatore, Michele Gallo, Serena Boccella, Annarosaria De Chiara, Carlo Ruosi, Filomena de Nigris
Giant cell tumors of bone (GCTB) are rare sarcomas with a high rate of unpredictable local relapse. Studies suggest that surgical methods affect recurrence, supporting the idea that local disease develops from re-growth of residual cancer cells. To identify early prognostic markers of individual risk of recurrence, we evaluated the effect of post-surgery fluids from a cohort of GCTB patients on growth of primary and established sarcoma cell lines, and mice xenographts. Post-surgery fluids increased cell growth and enhanced expression of CD44++, the principal receptor for the extracellular matrix component hyaluronan and the mesenchymal stem marker CD117+...
June 28, 2017: Oncotarget
https://www.readbyqxmd.com/read/28687003/inhibition-of-chk1-sensitizes-ewing-sarcoma-cells-to-the-ribonucleotide-reductase-inhibitor-gemcitabine
#14
Kelli L Goss, Stacia L Koppenhafer, Kathryn M Harmoney, William W Terry, David J Gordon
Ewing sarcoma is a bone and soft tissue sarcoma that occurs in children and young adults. The EWS-FLI1 gene fusion is the driver mutation in most Ewing sarcoma tumors and functions, in part, as an aberrant transcription factor. We recently identified that Ewing sarcoma cells are sensitive to inhibition of ribonucleotide reductase (RNR), which catalyzes the formation of deoxyribonucleotides from ribonucleotides. In this report, we show that Ewing sarcoma cells are sensitive to treatment with clofarabine, which is a nucleoside analogue and allosteric inhibitor of RNR...
June 28, 2017: Oncotarget
https://www.readbyqxmd.com/read/28684728/a-rare-case-of-sarcomatoid-carcinoma-of-the-lung-with-spine-metastasis-including-a-literature-review
#15
Hafiza Sobia Arshad, Rizwan Ahmed Dudekula, Masooma Niazi, Sandeep Malik, Misbahuddin Khaja
BACKGROUND Sarcomatoid carcinoma is a rare, aggressive, malignant cancer composed of sarcoma and sarcoma-like components, and can occur in different organs such as the thyroid gland, bone, skin, breast, pancreas, liver, urinary tract, and lung. Pulmonary sarcomatoid carcinoma accounts for only a small percentage of lung cancers and has histological variants that include pleomorphic carcinoma, giant cell carcinoma, spindle cell carcinoma, carcinosarcoma, and pulmonary blastoma. CASE REPORT Here, we present a case of sarcomatoid carcinoma in a 63-year-old HIV-positive Hispanic male who presented with back pain, dry cough, and weight loss...
July 7, 2017: American Journal of Case Reports
https://www.readbyqxmd.com/read/28682861/right-femoral-pathological-fracture-caused-by-primary-bone-epithelioid-angiosarcoma-case-report
#16
Yatong Li, Xiongfei Zou, Xiaoyan Chang, Xiao Chang, Shengfang Sun, Baozhong Zhang
RATIONALE: Epithelioid angiosarcoma (EAS) is an extremely rare malignant disease, which accounts no more than 1% of all soft tissue sarcomas. In this article, we would report a new case of EAS with multiple bone destruction and right femoral pathological fracture, which was an even rarer manifestation of EAS. PATIENT CONCERNS: In this case, a 64-year-old man with right femoral fracture was reported. He had suffered from a progressive low back pain for about 8 months, and the imaging examinations prompted a multiple bone destruction in his vertebra and lower limbs...
July 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28679368/non-vascularised-fibula-grafts-for-reconstruction-of-segmental-and-hemicortical-bone-defects-following-meta-diaphyseal-tumour-resection-at-the-extremities
#17
Ulrich Lenze, Stefanie Kasal, Fritz Hefti, Andreas Heinrich Krieg
BACKGROUND: The reconstruction of meta-/diaphyseal bone defects following bone tumour resection is challenging, and biological treatment options should be applied whenever possible, especially in benign lesions and early stage sarcomas. We aimed to evaluate the results of segmental (SR) and hemicortical reconstructions (HR) at the extremities using non-vascularised fibula grafts. METHODS: We retrospectively enrolled 36 patients who were treated with non-vascularised fibula reconstructions (15 SR, 21 HR) after bone tumour resection (15 malignant, 21 benign)...
July 5, 2017: BMC Musculoskeletal Disorders
https://www.readbyqxmd.com/read/28671673/ews-fli1-perturbs-mrtfb-yap-1-tead-target-gene-regulation-inhibiting-cytoskeletal-autoregulatory-feedback-in-ewing-sarcoma
#18
A M Katschnig, M O Kauer, R Schwentner, E M Tomazou, C N Mutz, M Linder, M Sibilia, J Alonso, D N T Aryee, H Kovar
Ewing sarcoma (EWS) is a paediatric bone cancer with high metastatic potential. Cellular plasticity resulting from dynamic cytoskeletal reorganization, typically regulated via the Rho pathway, is a prerequisite for metastasis initiation. Here, we interrogated the role of the Ewing sarcoma driver oncogene EWS-FLI1 in cytoskeletal reprogramming. We report that EWS-FLI1 strongly represses the activity of the Rho-F-actin signal pathway transcriptional effector MRTFB, affecting the expression of a large number of EWS-FLI1-anticorrelated genes including structural and regulatory cytoskeletal genes...
July 3, 2017: Oncogene
https://www.readbyqxmd.com/read/28671156/overview-of-extremity-musculoskeletal-neoplasms-at-the-ahmadu-bello-university-teaching-hospital-zaria-nigeria
#19
Maitama Mohammed Inuwa, Lawal Yau Zakariyau, Dahiru I Ismail, Ejagwulu S Friday, Aniko A Ibrahim, Abdulmalik A Mohammed
BACKGROUND: The burden of managing patients with musculoskeletal neoplasms in the West African sub-region is quite significant. This is largely due to late presentation, delay in making diagnosis, and failure of obtaining consent to certain surgical procedures. Improvements in diagnosis and treatment over the years have however increased life and limb survival for many patients. This study was aimed at evaluating the clinicopathologic pattern of neoplasm as it affects the upper and lower limbs with a view to determining the most common types, the most involved sites of the body and the age at presentation of these tumors...
July 2017: Annals of African Medicine
https://www.readbyqxmd.com/read/28670184/unusual-asymptomatic-fluorodeoxyglucose-avid-pheochromocytoma-in-a-case-of-myxoid-liposarcoma-of-the-extremity-on-18-f-fluorodeoxyglucose-positron-emission-tomography-computed-tomography
#20
Divya Shivdasani, Natasha Singh, Melvika Pereira, Anand Zade
Sarcomas are a heterogeneous group of rare tumors and arise either from soft tissue or from bone. Soft-tissue sarcomas (STSs) initially metastasize to the lungs. Metastases to extrapulmonary sites such as liver, brain, and soft tissue distant from primary tumor usually develop later. However, cases with isolated adrenal metastasis without disseminated disease have been reported in literature. We present a case of primary myxoid liposarcoma of the lower limb, in which staging (18)-F fluorodeoxyglucose positron emission tomography-computed tomography (FDG PET-CT) scan detected a suspicious FDG avid adrenal lesion which eventually on resection was diagnosed as asymptomatic pheochromocytoma...
July 2017: World Journal of Nuclear Medicine
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