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https://www.readbyqxmd.com/read/29220303/locally-aggressive-connective-tissue-tumors
#1
Mrinal M Gounder, David M Thomas, William D Tap
In this review, we highlight the complexities of the natural history, biology, and clinical management of three intermediate connective tissue tumors: desmoid tumor (DT) or aggressive fibromatosis, tenosynovial giant cell tumor (TGCT) or diffuse-type pigmented villonodular synovitis (dtPVNS), and giant cell tumor of bone (GCTB). Intermediate histologies include tumors of both soft tissue and bone origin and are locally aggressive and rarely metastatic. Some common aspects to these tumors are that they can be locally infiltrative and/or impinge on critical organs, which leads to disfigurement, pain, loss of function and mobility, neurovascular compromise, and occasionally life-threatening consequences, such as mesenteric, bowel, ureteral, and/or bladder obstruction...
December 8, 2017: Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology
https://www.readbyqxmd.com/read/29071003/successful-treatment-of-cardiac-angiosarcoma-associated-with-disseminated-intravascular-coagulation-with-nab-paclitaxel-a-case-report-and-review-of-the-literature
#2
Kazunori Honda, Masashi Ando, Keiji Sugiyama, Seiichiro Mitani, Toshiki Masuishi, Yukiya Narita, Hiroya Taniguchi, Shigenori Kadowaki, Takashi Ura, Kei Muro
Angiosarcoma of the heart is an uncommon soft tissue sarcoma. A few cases of disseminated intravascular coagulation (DIC) associated with angiosarcoma occurring in various organs, but not the heart, have been reported. Although taxane is commonly used in the treatment of metastatic angiosarcoma, data on the efficacy of nab-paclitaxel for angiosarcoma are limited. Here, we report probably the first case of a patient with primary cardiac angiosarcoma with coexisting DIC who was successfully treated with nab-paclitaxel...
September 2017: Case Reports in Oncology
https://www.readbyqxmd.com/read/28988646/pembrolizumab-in-advanced-soft-tissue-sarcoma-and-bone-sarcoma-sarc028-a-multicentre-two-cohort-single-arm-open-label-phase-2-trial
#3
Hussein A Tawbi, Melissa Burgess, Vanessa Bolejack, Brian A Van Tine, Scott M Schuetze, James Hu, Sandra D'Angelo, Steven Attia, Richard F Riedel, Dennis A Priebat, Sujana Movva, Lara E Davis, Scott H Okuno, Damon R Reed, John Crowley, Lisa H Butterfield, Ruth Salazar, Jaime Rodriguez-Canales, Alexander J Lazar, Ignacio I Wistuba, Laurence H Baker, Robert G Maki, Denise Reinke, Shreyaskumar Patel
BACKGROUND: Patients with advanced sarcomas have a poor prognosis and few treatment options that improve overall survival. Chemotherapy and targeted therapies offer short-lived disease control. We assessed pembrolizumab, an anti-PD-1 antibody, for safety and activity in patients with advanced soft-tissue sarcoma or bone sarcoma. METHODS: In this two-cohort, single-arm, open-label, phase 2 study, we enrolled patients with soft-tissue sarcoma or bone sarcoma from 12 academic centres in the USA that were members of the Sarcoma Alliance for Research through Collaboration (SARC)...
October 4, 2017: Lancet Oncology
https://www.readbyqxmd.com/read/28952794/management-of-a-tibial-plafond-lung-cancer-metastasis-using-an-intramedullary-hindfoot-fusion-nail
#4
Andrew R Hsu, Adam D Lindsay
Metastatic disease to the tibial plafond is rare with few reports in the literature. No consensus exists regarding surgical reconstruction of large structural defects of the ankle due to these lesions, as each treatment must be tailored to the individual patient's goals and prognosis. Cancer metastases pose a unique challenge to limb salvage as there is often bone loss and poor soft tissue quality combined with the need for postoperative adjuvant therapy. The goal of surgery is to obtain early weightbearing, pain relief, and a durable reconstruction that will outlive the patient...
September 1, 2017: Foot & Ankle Specialist
https://www.readbyqxmd.com/read/28873262/reactivation-of-twist1-contributes-to-ewing-sarcoma-metastasis
#5
Sun Choo, Ping Wang, Robert Newbury, William Roberts, Jing Yang
BACKGROUND: Ewing sarcoma is a cancer of bone and soft tissue. Despite aggressive treatment, survival remains poor, particularly in patients with metastatic disease. Failure to treat Ewing sarcoma is due to the lack of understanding of the molecular pathways that regulate metastasis. In addition, no molecular prognostic markers have been identified for Ewing sarcoma to risk stratify patients. PROCEDURE: Ewing sarcoma patients were divided into high or low Twist1 gene expression and survival curves were generated using the R2 microarray-based Genomic Analysis platform (http://r2...
September 5, 2017: Pediatric Blood & Cancer
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
#6
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
https://www.readbyqxmd.com/read/28549419/fluorescent-cxcr4-targeting-peptide-as-alternative-for-antibody-staining-in-ewing-sarcoma
#7
Laurens G L Sand, Tessa Buckle, Fijs W B van Leeuwen, Willem E Corver, Alwine B Kruisselbrink, Aart G Jochemsen, Pancras C W Hogendoorn, Károly Szuhai
BACKGROUND: Ewing sarcoma is an aggressive, highly metastatic primary bone and soft tissue tumor most frequently occurring in the bone of young adolescents. Patients, especially those diagnosed with a metastatic disease, have a poor overall survival. Chemokine receptor CXCR4 has a key pro-tumorigenic role in the tumor microenvironment of Ewing sarcoma and has been suggested to be involved in the increased metastatic propensity. Earlier studies on CXCR4 protein expression in Ewing sarcoma yielded contradictory results when compared to CXCR4 RNA expression studies...
May 26, 2017: BMC Cancer
https://www.readbyqxmd.com/read/28534008/a-comparison-of-pediatric-vs-adult-patients-with-the-ewing-sarcoma-family-of-tumors
#8
Vivek Verma, Kyle A Denniston, Christopher J Lin, Chi Lin
PURPOSE: This study sought to identify differences in clinical characteristics, outcomes, and treatments between adult and pediatric patients with the Ewing sarcoma family of tumors (ESFT). METHODS: By using the Surveillance, Epidemiology, and End Results database from 1983 to 2013, 1,870 patients were analyzed (n = 976 pediatric, n = 894 adult). Between the two groups, demographic, tumor, and treatment characteristics were collated and compared. The chi-square test determined differences in proportions of the variables between groups...
2017: Frontiers in Oncology
https://www.readbyqxmd.com/read/28488171/clinical-and-pathological-characteristics-of-gastrointestinal-stromal-tumor-gist-metastatic-to-bone
#9
Kemal Kosemehmetoglu, Gulsah Kaygusuz, Karen Fritchie, Ovgu Aydin, Ozlem Yapicier, Oznur Coskun, Ersin Karatayli, Senay Boyacigil, Gulnur Guler, Sergulen Dervisoglu, Isinsu Kuzu
Our aim in this study was to describe the clinical, morphological, and molecular profile of gastrointestinal stromal tumor (GIST) metastatic to bone. We analyzed the morphological, phenotypic, and molecular characteristics of seven cases, and in addition reviewed 17 cases from literature. Sequence analysis of KIT and PDGFRA genes was possible for six cases. For the GIST cases with bone metastasis, the most common primaries were small intestine (29%), stomach (25%), and rectum (21%). Sites of bone metastases were vertebrae (11), pelvis (8), femur (8), ribs (6), humerus (5), skull (3), scapula (1), and mandible (1)...
July 2017: Virchows Archiv: An International Journal of Pathology
https://www.readbyqxmd.com/read/28471719/revisions-to-the-international-neuroblastoma-response-criteria-a-consensus-statement-from-the-national-cancer-institute-clinical-trials-planning-meeting
#10
REVIEW
Julie R Park, Rochelle Bagatell, Susan L Cohn, Andrew D Pearson, Judith G Villablanca, Frank Berthold, Susan Burchill, Ariane Boubaker, Kieran McHugh, Jed G Nuchtern, Wendy B London, Nita L Seibel, O Wolf Lindwasser, John M Maris, Penelope Brock, Gudrun Schleiermacher, Ruth Ladenstein, Katherine K Matthay, Dominique Valteau-Couanet
Purpose More than two decades ago, an international working group established the International Neuroblastoma Response Criteria (INRC) to assess treatment response in children with neuroblastoma. However, this system requires modification to incorporate modern imaging techniques and new methods for quantifying bone marrow disease that were not previously widely available. The National Cancer Institute sponsored a clinical trials planning meeting in 2012 to update and refine response criteria for patients with neuroblastoma...
August 1, 2017: Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology
https://www.readbyqxmd.com/read/28436593/results-for-patients-with-sarcoma-not-otherwise-specified-and-other-diagnoses-than-ewing-sarcoma-treated-according-to-the-euro-ewing-99-trial
#11
MULTICENTER STUDY
Judith Amalie Frank, Andreas Ranft, Michael Paulussen, Heribert Juergens, Jarmila Kruseova, Sebastian Bauer, Felix Niggli, Peter Reichardt, Uta Dirksen
BACKGROUND: Euro-EWING 99 trial of the European Ewing tumor Working Initiative of National Groups (EE99) was an international phase III study in patients with Ewing sarcoma. The German Society of Pediatric Oncology and Hematology (GPOH) data center registered and followed patients with other diagnoses than Ewing sarcoma who were treated according to the EE99 protocol in an additional non-Ewing database. PROCEDURE: Data of 27 patients with other diagnoses than Ewing sarcoma treated according to the EE99 protocol were analyzed...
October 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28362562/ten-year-progression-free-and-overall-survival-in-patients-with-unresectable-or-metastatic-gi-stromal-tumors-long-term-analysis-of-the-european-organisation-for-research-and-treatment-of-cancer-italian-sarcoma-group-and-australasian-gastrointestinal-trials
#12
RANDOMIZED CONTROLLED TRIAL
Paolo G Casali, John Zalcberg, Axel Le Cesne, Peter Reichardt, Jean-Yves Blay, Lars H Lindner, Ian R Judson, Patrick Schöffski, Serge Leyvraz, Antoine Italiano, Viktor Grünwald, Antonio Lopez Pousa, Dusan Kotasek, Stefan Sleijfer, Jan M Kerst, Piotr Rutkowski, Elena Fumagalli, Pancras Hogendoorn, Saskia Litière, Sandrine Marreaud, Winette van der Graaf, Alessandro Gronchi, Jaap Verweij
Purpose To report on the long-term results of a randomized trial comparing a standard dose (400 mg/d) versus a higher dose (800 mg/d) of imatinib in patients with metastatic or locally advanced GI stromal tumors (GISTs). Patients and Methods Eligible patients with advanced CD117-positive GIST from 56 institutions in 13 countries were randomly assigned to receive either imatinib 400 mg or 800 mg daily. Patients on the 400-mg arm were allowed to cross over to 800 mg upon progression. Results Between February 2001 and February 2002, 946 patients were accrued...
May 20, 2017: Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology
https://www.readbyqxmd.com/read/28319067/the-histone-demethylase-kdm3a-and-its-downstream-target-mcam-promote-ewing-sarcoma-cell-migration-and-metastasis
#13
M Sechler, J K Parrish, D K Birks, P Jedlicka
Ewing Sarcoma is the second most common solid pediatric malignant neoplasm of bone and soft tissue. Driven by EWS/Ets, or rarely variant, oncogenic fusions, Ewing Sarcoma is a biologically and clinically aggressive disease with a high propensity for metastasis. However, the mechanisms underpinning Ewing Sarcoma metastasis are currently not well understood. In the present study, we identify and characterize a novel metastasis-promotional pathway in Ewing Sarcoma, involving the histone demethylase KDM3A, previously identified by our laboratory as a new cancer-promoting gene in this disease...
July 20, 2017: Oncogene
https://www.readbyqxmd.com/read/28291905/long-term-results-of-therapy-with-sunitinib-in-metastatic-alveolar-soft-part-sarcoma
#14
Paulina Jagodzińska-Mucha, Tomasz Świtaj, Katarzyna Kozak, Hanna Koseła-Paterczyk, Anna Klimczak, Iwona Ługowska, Pawel Rogala, Michał Wągrodzki, Sławomir Falkowski, Piotr Rutkowski
BACKGROUND: Alveolar soft part sarcoma (ASPS) is a rare, highly vascularized soft tissue sarcoma characterized by a high frequency of metastatic disease and resistance to classical chemotherapy. The purpose of our analysis was to assess long-term sunitinib activity in the treatment of metastatic ASPS. PATIENTS AND METHODS: Between 2009 and 2015, 15 patients were diagnosed with metastatic ASPS and received therapy with sunitinib at initial continuous daily dosing of 37...
May 12, 2017: Tumori
https://www.readbyqxmd.com/read/28241093/phase-1-2-study-of-immunotherapy-with-dendritic-cells-pulsed-with-autologous-tumor-lysate-in-patients-with-refractory-bone-and-soft-tissue-sarcoma
#15
Shinji Miwa, Hideji Nishida, Yoshikazu Tanzawa, Akihiko Takeuchi, Katsuhiro Hayashi, Norio Yamamoto, Eishiro Mizukoshi, Yasunari Nakamoto, Shuichi Kaneko, Hiroyuki Tsuchiya
BACKGROUND: There are limited options for the curative treatment of refractory bone and soft tissue sarcomas. The purpose of this phase 1/2 study was to assess the immunological and clinical effects of dendritic cells (DCs) pulsed with autologous tumor lysate (TL) in patients with advanced bone and soft tissue sarcomas. METHODS: Thirty-seven patients with metastatic or recurrent sarcomas were enrolled in this study. Peripheral blood mononuclear cells obtained from the patients were suspended in media containing interleukin 4 (IL-4) and granulocyte-macrophage colony-stimulating factor...
May 1, 2017: Cancer
https://www.readbyqxmd.com/read/28203089/impact-of-tumor-volume-doubling-time-on-post-metastatic-survival-in-bone-or-soft-tissue-sarcoma-patients-treated-with-metastasectomy-and-or-radiofrequency-ablation-of-the-lung
#16
Tomoki Nakamura, Akihiko Matsumine, Motoshi Takao, Atsuhiro Nakatsuka, Takao Matsubara, Kunihiro Asanuma, Akihiro Sudo
Metastasectomy represents the standard treatment for improving survival in patients with lung metastases (LMs) from bone (BS) or soft-tissue sarcoma (STS). Recently, radiofrequency ablation (RFA) of the LMs has been proved to be a useful option which can promise the similar effect to metastasectomy. The aim of this study was to determine prognostic factors, including tumor volume doubling time (TVDT), for post-metastatic survival in BS and STS patients treated with metastasectomy and/or RFA of the lung. Forty-eight patients with LMs were retrospectively reviewed...
2017: OncoTargets and Therapy
https://www.readbyqxmd.com/read/28110186/numb-chin-syndrome-as-a-sign-of-mandibular-metastasis-a-case-report
#17
Thomas Aerden, Koenraad Grisar, Patrick Neven, Esther Hauben, Constantinus Politis
INTRODUCTION: Metastasis to the oral cavity can be located in both the soft and bony tissues and comprise only 1% of all oral malignancies; however, it is clinically significant because it indicates widespread metastatic disease and an unfavorable prognosis. A numb chin is an important presentation of oral metastasis, but other dental and systemic pathology may be involved. PRESENTATION OF CASE: We present the case of a 54-year-old woman who presented with numb chin syndrome 8 years after a diagnosis of primary breast carcinoma...
2017: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/28077014/factors-associated-with-reduced-functional-outcome-and-quality-of-life-in-patients-having-limb-sparing-surgery-for-soft-tissue-sarcomas-a-national-multicenter-study-of-128-patients
#18
MULTICENTER STUDY
Casper Saebye, Hanna M Fugloe, Tine Nymark, Akmal Safwat, Michael M Petersen, Thomas Baad-Hansen, Anders Krarup-Hansen, Johnny Keller
BACKGROUND: Limb-sparing surgery for sarcomas has become possible in most cases. However, the impact of the procedure on the functional outcome has only been investigated in a few studies. The aim of this study has been to identify tumor- and patient-related factors associated with reduced functional outcome and quality of life after limb-sparing surgery in soft tissue sarcoma patients. MATERIAL AND METHODS: In total, 128 patients (mean age = 58, female/male = 54/74) who were treated with limb-sparing surgery without bone resection for soft tissue sarcomas in Denmark during the period 1 January 2009 to 31 December 2011 were included...
February 2017: Acta Oncologica
https://www.readbyqxmd.com/read/28029320/pulmonary-infiltrates-in-a-patient-with-advanced-melanoma
#19
REVIEW
Charles A Powell
The Oncology Grand Rounds series is designed to place original reports published in the Journal into clinical context. A case presentation is followed by a description of diagnostic and management challenges, a review of the relevant literature, and a summary of the authors' suggested management approaches. The goal of this series is to help readers better understand how to apply the results of key studies, including those published in Journal of Clinical Oncology, to patients seen in their own clinical practice...
March 2017: Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology
https://www.readbyqxmd.com/read/27785071/current-classification-treatment-options-and-new-perspectives-in-the-management-of-adipocytic-sarcomas
#20
REVIEW
Alessandro De Vita, Laura Mercatali, Federica Recine, Federica Pieri, Nada Riva, Alberto Bongiovanni, Chiara Liverani, Chiara Spadazzi, Giacomo Miserocchi, Dino Amadori, Toni Ibrahim
Sarcomas are a heterogeneous group of mesenchymal tumors arising from soft tissue or bone, with an uncertain etiology and difficult classification. Soft tissue sarcomas (STSs) account for around 1% of all adult cancers. Till date, more than 50 histologic subtypes have been identified. Adipocyte sarcoma or liposarcoma (LPS) is one of the most common STS subtypes, accounting for 15% of all sarcomas, with an incidence of 24% of all extremity STSs and 45% of all retroperitoneal STSs. The new World Health Organization classification system has divided LPS into four different subgroups: atypical lipomatous tumor/well-differentiated LPS, dedifferentiated LPS, myxoid LPS, and pleomorphic LPS...
2016: OncoTargets and Therapy
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