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Bone sarcomas

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https://www.readbyqxmd.com/read/28817827/role-of-next-generation-sequencing-as-a-diagnostic-tool-for-the-evaluation-of-bone-and-soft-tissue-tumors
#1
Kinga Szurian, Karl Kashofer, Bernadette Liegl-Atzwanger
Bone and soft-tissue tumors are in general rare. Diagnosing these tumors is challenging based on the significant number of different tumor entities, the rareness of these tumors, and the considerable morphological heterogeneity which can be found within a single tumor entity. Considering that more than half of the described soft-tissue tumors and approximately 25% of the bone tumors harbor recurrent genetic alterations, the use of auxiliary molecular examinations should be strongly considered. Molecular analyses are important to confirm the diagnosis, to guide treatment, to provide information about prognosis, and to allow patient recruitment for basket trials based on the molecular signature of a tumor...
August 18, 2017: Pathobiology: Journal of Immunopathology, Molecular and Cellular Biology
https://www.readbyqxmd.com/read/28817404/primary-renal-sarcomas-with-bcor-ccnb3-gene-fusion-a-report-of-2-cases-showing-histologic-overlap-with-clear-cell-sarcoma-of-kidney-suggesting-further-link-between-bcor-related-sarcomas-of-the-kidney-and-soft-tissues
#2
Pedram Argani, Yu-Chien Kao, Lei Zhang, Carlos Bacchi, Andres Matoso, Rita Alaggio, Jonathan I Epstein, Cristina R Antonescu
We report 2 primary renal sarcomas demonstrating BCOR-CCNB3 gene fusions that have recently been identified in undifferentiated round cell sarcomas of bone and soft tissue. These neoplasms occurred in male children aged 11 and 12 years, and both were cystic as a result of entrapment and dilatation of native renal tubules. Both cases were composed of variably cellular bland spindle cells with fine chromatin set in myxoid stroma and separated by a branching capillary vasculature. Both neoplasms demonstrated immunoreactivity for BCOR, cyclin D1, TLE1, and SATB2 in the spindle neoplastic cells and negativity in the prominent capillary vasculature...
August 16, 2017: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28816792/development-of-secondary-acute-myeloid-leukemia-in-a-pediatric-patient-concurrently-receiving-primary-therapy-for-ewing-sarcoma
#3
Brandon R McNew, Benjamin W Darbro, Deqin Ma, David J Gordon
Ewing sarcoma is a pediatric bone and soft tissue sarcoma that requires intensive therapy, which can cause secondary malignancies. We present a rare case of early, treatment-related AML in a pediatric patient concurrently receiving primary therapy for Ewing sarcoma. Despite AML-directed therapy, our patient died secondary to complications of hyperleukocytosis. Cytogenetic and mutation profiling of the leukemia cells revealed the DNA-topoisomerase-II-inhibitor-associated t(9;11)(p22;q23) translocation and clonal KRAS and BRAF mutations...
August 14, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28814152/distant-metastasis-in-patients-with-myxofibrosarcoma
#4
Hiroyuki Tsuchie, Mitsunori Kaya, Hiroyuki Nagasawa, Makoto Emori, Yasutaka Murahashi, Emi Mizushima, Naohisa Miyakoshi, Toshihiko Yamashita, Yoichi Shimada
PURPOSE: A clinical feature of myxofibrosarcoma is local recurrence, but knowledge about distant metastasis is sparse. We evaluated the tendency of clinical and histological features of metastasis in myxofibrosarcoma patients. METHODS: Fifty-eight patients with myxofibrosarcoma were treated in our hospitals, and a total of 16 consecutive patients with distant metastases were included in this retrospective study (9 males and 7 females, with a mean age of 77 years)...
August 17, 2017: Upsala Journal of Medical Sciences
https://www.readbyqxmd.com/read/28811330/epithelial-to-mesenchymal-and-mesenchymal-to-epithelial-transition-in-mesenchymal-tumors-a-paradox-in-sarcomas
#5
REVIEW
Giuseppina Sannino, Aruna Marchetto, Thomas Kirchner, Thomas G P Grünewald
The epithelial-to-mesenchymal transition (EMT) is a reversible process comprised of various subprograms via which epithelial cells reduce their intercellular adhesions and proliferative capacity while gaining a mesenchymal phenotype with increased migratory and invasive properties. This process has been well described in several carcinomas, which are cancers of epithelial origin, and is crucial to metastatic tumor cell dissemination and drug resistance. In contrast, the precise role of EMT-related processes in tumors originating from mesenchymal tissues, such as bone and soft-tissues sarcomas, is still largely unclear...
August 15, 2017: Cancer Research
https://www.readbyqxmd.com/read/28810297/-clinical-and-pathologic-features-of-extrapleural-sarcomatoid-mesothelioma
#6
M C Wei, S J Yang
Objective: To investigate the morphological features, diagnosis and differential diagnosis of extrapleural sarcomatoid malignant mesothelioma (SMM). Methods: Six cases of extrapleural SMM were evaluated for their clinical, histological, immunohistochemical features, and prognosis. Results: Patients included 3 men and 3 women, with a median age of 60 years (range 41-75 years). All patients had no asbestos exposure in history and no pleural lesions. The tumors involved peritoneum (3 cases), bone (2 cases), and neck soft tissue (1 case)...
August 8, 2017: Zhonghua Bing Li Xue za Zhi Chinese Journal of Pathology
https://www.readbyqxmd.com/read/28806574/quantitative-diffusion-weighted-magnetic-resonance-imaging-assessment-of-chemotherapy-treatment-response-of-pediatric-osteosarcoma-and-ewing-sarcoma-malignant-bone-tumors
#7
Andrew J Degnan, Chul Y Chung, Amisha J Shah
OBJECTIVE: Assessment of tumor response to chemotherapy is essential in managing malignant pediatric bone tumors prior to resection. SUBJECTS AND METHODS: Pre-chemotherapy and post-chemotherapy osteosarcoma and Ewing sarcoma cases (n=18) were analyzed with apparent diffusion coefficient (ADC) values measured by two readers. RESULTS: Treated tumors demonstrated significantly greater ADC values compared to untreated tumors (p<0.001). Intraclass correlation coefficients ranged between 0...
August 5, 2017: Clinical Imaging
https://www.readbyqxmd.com/read/28803261/advantages-in-prognosis-of-adult-patients-with-ewing-sarcoma-11-years-experiences-and-current-treatment-management
#8
Dagmar Adamkova Krakorova, Katerina Kubackova, Ladislav Dusek, Tomas Tomas, Pavel Janicek, Stepan Tucek, Jana Prausova, Igor Kiss, Iva Zambo
Ewing sarcoma (ES) is an exceptionally rare tumor in adults. Data regarding outcomes of adult patients with ES and experiences with age-adapted therapeutic strategies are very limited. The aim of this study was to evaluate prognostic factors and clinical outcome in a cohort of adult patients treated according to pediatric protocols in the Czech Republic. The records of 58 adult ES patients diagnosed between 2002 and 2013 were reviewed and factors relevant to prognosis and survival were analyzed. The median age of study cohort was 29 years (range, 18-59)...
August 12, 2017: Pathology Oncology Research: POR
https://www.readbyqxmd.com/read/28802390/pediatric-head-and-neck-bone-sarcomas-an-analysis-of-204-cases
#9
Jacob S Brady, Sei Y Chung, Emily Marchiano, Jean Anderson Eloy, Soly Baredes, Richard Chan Woo Park
OBJECTIVES: To analyze the demographics, survival, and treatment efficacy of pediatric sarcomas of the facial skeleton and skull. METHODS: Retrospective study of cases from the US National Cancer Institute's Surveillance, Epidemiology, and End Results database. Pediatric patients between the ages of 0 and 18 diagnosed with a malignant sarcoma of either the mandible or the bones of skull, face, and associated joints from 1973 to 2013 were studied. RESULTS: In total, 204 patients were included in the analysis...
September 2017: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/28801374/myeloid-sarcoma-in-a-patient-with-myelodysplastic-syndrome-associated-with-del-5q-case-report-and-literature-review
#10
Josh A Showalter, Nidhi Tandon, Bihong Zhao, Guilin Tang, Nghia D Nguyen, L Jeffrey Medeiros
OBJECTIVE: Myeloid sarcoma (MS) is defined in the World Health Organization classification as a tumor mass consisting of myeloblasts with or without maturation and involving any anatomic site other than the bone marrow. We present a case of MS developing in a patient with 5q- myelodysplastic syndrome (MDS) and review the relevant literature. METHODS: A 77-year-old woman with recent diagnosis of MDS associated with del(5q) presented with symptoms and signs attributable to a mass involving the T8 vertebra...
August 2017: Annals of Clinical and Laboratory Science
https://www.readbyqxmd.com/read/28797510/soft-tissue-tumors-rarely-presenting-primary-in-bone-diagnostic-pitfalls
#11
REVIEW
Marta Sbaraglia, Alberto Righi, Marco Gambarotti, Daniel Vanel, Piero Picci, Angelo P Dei Tos
Primary bone sarcomas represent extremely rare entities. The use of now abolished labels, such as malignant fibrous histiocytoma and hemangiopericytoma, has significantly hampered the chance of identifying specific entities. It is now accepted that a broad variety of mesenchymal malignancies most often arising on the soft tissue may actually present as primary bone lesions. A more accurate morphologic partition is justified based on availability of distinct therapeutic options. An integrated diagnostic approach represents the only way to achieve a correct classification...
September 2017: Surgical Pathology Clinics
https://www.readbyqxmd.com/read/28790781/to-study-the-efficacy-of-thallium-201-as-tumor-seeking-agent-and-to-study-its-role-in-therapeutic-response
#12
A V S Anil Kumar, P G Kumar, Sangeetha Sampath, V Suhag
BACKGROUND: Tumour seeking characteristics of TL-201 have been underutilized. The study was undertaken to evaluate the role of TL-201 scintigraphy in tumour imaging. METHODS: A total of 50 cases were studied over a period of 18 months (13 lymphomas, 11 breast carcinoma, 10 lung cancer, 6 of soft tissue sarcoma, 2 bone tumours and 4 cases each of thyroid and brain tumours). Thallium-201 chloride was injected IV in the dose range of 3-5 mCi. Imaging was done using Siemen's ECAM dual headed gamma camera...
July 2017: Medical Journal, Armed Forces India
https://www.readbyqxmd.com/read/28790479/immunohistochemistry-in-primary-b-cell-lymphoma-of-tibia-masquerading-as-chronic-osteomyelitis
#13
Raju Karuppal, Samarth Manjunath, Rajendran V Raman, S Sandhya
The diagnosis between chronic osteomyelitis, Ewing sarcoma and lymphoma often is being confusing in many occasions. As the latter two conditions are malignant, early diagnosis and interventions are crucial. We present a 28 year old male with features of chronic osteomyelitis of right tibia 2 years back then changed through Ewing sarcoma to B-cell lymphoma in the histological diagnosis. This case report highlights the difficulties that arise in diagnosing primary bone lymphomas which may masquerade as chronic osteomyelitis and hence the need for immunohistochemistry in chronic recurrent osteomyelitis...
July 2017: Indian Journal of Orthopaedics
https://www.readbyqxmd.com/read/28780919/anthracycline-induced-cardiotoxicity-in-patients-with-paediatric-bone-sarcoma-and-soft-tissue-sarcoma
#14
Ilaria Bini, Sebastian D Asaftei, Chiara Riggi, Elisa Tirtei, Rosaria Manicone, Eleonora Biasin, Maria Eleonora Basso, Gabriella Agnoletti, Franca Fagioli
OBJECTIVES: Anthracycline cardiotoxicity is an important side-effect in long-term childhood cancer survivors. We evaluated the incidence of and factors associated with anthracycline cardiotoxicity in a population of patients diagnosed with bone or soft tissue sarcoma. Materials and methods We retrospectively enrolled patients diagnosed with bone or soft tissue sarcoma, from 1995 to 2011, treated with anthracycline chemotherapy at our Centre and with a follow-up echocardiography carried out ⩾3 years from cardiotoxic therapy completion...
August 7, 2017: Cardiology in the Young
https://www.readbyqxmd.com/read/28764951/reconstruction-of-the-bony-chin-using-sagittal-split-osteotomies-of-the-remaining-mandible-a-new-technique-for-a-special-indication-case-report
#15
Berthold H Hell
There are several indications for resecting the bony chin, such as squamous cell carcinoma, sarcoma, or benign tumors of the jaw such as ameloblastoma. Several techniques for reconstruction of the bony chin, such as the use of metal plates and also revascularized free bone grafts of the iliac crest, fibula, or scapula, are common. In the case of poor vascular supply, however, alternative techniques may be necessary. In this report, a new technique is described using pedicled bone flaps from both sagittally split mandibular stumps following chin resection...
June 16, 2017: Journal of Cranio-maxillo-facial Surgery
https://www.readbyqxmd.com/read/28762959/mechanical-properties-and-cell-culture-characteristics-of-polycaprolactone-kagome-structure-scaffold-fabricated-by-precision-extruding-deposition-system
#16
Se-Hwan Lee, Yong Sang Cho, Myoung Wha Hong, Bu Kyu Lee, Yong-Doo Park, Sang-Hyug Park, Young-Yul Kim, Young-Sam Cho
To date, to enhance the mechanical properties of three-dimensional scaffolds used for bone regeneration in tissue engineering, many researchers have tried in the viewpoint of structure and chemistry. Meanwhile, in the structural engineering field, the kagome structure has been known as an excellent relative strength. In this study, to enhance the mechanical properties of a synthetic polymer scaffold used for tissue engineering, we applied the three-dimensional kagome structure to a porous scaffold for bone regeneration...
August 1, 2017: Biomedical Materials
https://www.readbyqxmd.com/read/28761532/skull-base-bony-lesions-management-nuances-a-retrospective-analysis-from-a-tertiary-care-centre
#17
Amit Kumar Singh, Arun Kumar Srivastava, Jayesh Sardhara, Kamlesh Singh Bhaisora, Kuntal Kanti Das, Anant Mehrotra, Rabi Narayan Sahu, Awadhesh Kumar Jaiswal, Sanjay Behari
BACKGROUND: Skull base lesions are not uncommon, but their management has been challenging for surgeons. There is large no of bony tumors at the skull base which has not been studied in detail as a group. These tumors are difficult not only because of their location but also due to their variability in the involvement of important local structure. Through this retrospective analysis from a Tertiary Care Centre, we are summarizing the details of skull base bony lesions and its management nuances...
July 2017: Asian Journal of Neurosurgery
https://www.readbyqxmd.com/read/28761507/primary-intracranial-dural-based-ewing-sarcoma-peripheral-primitive-neuroectodermal-tumor-mimicking-a-meningioma-a-rare-tumor-with-review-of-literature
#18
REVIEW
Vikul Kumar, Anshu Singh, Vivek Sharma, Mohan Kumar
Ewing sarcoma/peripheral primitive neuroectodermal tumor (ES/pPNET) is a malignant small, round cell tumor arising from bone and soft tissue in children and young adults. It can occur at osseous and extraosseous sites. Its usual locations are diaphysis of long bones followed by pelvis, ribs, vertebrae, and rarely skull. We reviewed the literature and PubMed advanced search on ES/pPNET occurring at extraosseous sites, mainly involving the central nervous system (CNS). We reported a case of a 22-year-old male presenting with seizure finally diagnosed as a case of ES/pPNET...
July 2017: Asian Journal of Neurosurgery
https://www.readbyqxmd.com/read/28756981/congenital-undifferentiated-sarcoma-associated-to-bcor-ccnb3-gene-fusion
#19
Clara Alfaro-Cervello, Verónica Andrade-Gamarra, Gema Nieto, Lara Navarro, Susana Martín-Vañó, Juan Pablo García de la Torre, María Bengoa Caamaño, Mª Luisa García Mauriño, Rosa Noguera, Samuel Navarro
Small round cell sarcomas are aggressive bone and soft tissue tumors that predominantly affect children and young adults. A new group of sarcomas with a recurrent BCOR-CCNB3 gene fusion has been recently identified in previously unclassifiable small round cell sarcomas. BCOR-CCNB3 sarcomas share clinical and pathologic similarities with Ewing sarcoma, but show a stronger male predilection and less aggressiveness, as well as distinct gene expression profiling and pangenomic SNP array analyses. We report the unusual case of a congenital BCOR-CCNB3 retroperitoneal sarcoma in a female born at 34th gestational week, which was diagnosed in necropsy after 21hours of life...
July 14, 2017: Pathology, Research and Practice
https://www.readbyqxmd.com/read/28755281/radiation-induced-focal-cortical-necrosis-of-the-femur-presenting-as-a-lytic-lesion
#20
Hakan Ilaslan, Jean Schils, Michael Joyce, Chirag Shah, Yaxia Zhang
Management of soft tissue sarcomas is often complicated, requiring radiation before and in some cases after limb-sparing surgery. Radiation necrosis is a severe complication after radiation treatment and is typically dose related and involves medullary bone. We report on two cases of hitherto unreported focal circumscribed intra-cortical lytic lesions within the radiation portal, which appeared 19 months and 31 months, respectively, after the conclusion of radiation treatment. Both patients had a history of soft tissue sarcoma treated with radiation (66 Gy) and surgical resection...
July 28, 2017: Skeletal Radiology
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