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Soft tissue sarcomas

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https://www.readbyqxmd.com/read/28819402/pd-l1-expression-is-associated-with-foxp3-regulatory-t-cell-infiltration-of-soft-tissue-sarcoma-and-poor-patient-prognosis
#1
Yi Que, Wei Xiao, Yuan-Xiang Guan, Yao Liang, Shu-Mei Yan, Huo-Ying Chen, Qiao-Qiao Li, Bu-Shu Xu, Zhi-Wei Zhou, Xing Zhang
Background: Programmed death ligand-1(PD-L1) functions as a negative mediator of immune response through different pathways in anti-tumor immunity. Recent studies have reported that PD-L1 plays a pivotal role in the function of regulatory T-cells (Tregs). Although increases in FOXP3+ Tregs infiltration and PD-L1 expression have been revealed in several cancers, their correlation with soft tissue sarcoma remains unknown. Methods: We included 163 cases of soft tissue sarcoma who were diagnosed and underwent extensive and radical resection at the Sun Yat-sen University Cancer Center, Guangzhou, China, from 2000-2010...
2017: Journal of Cancer
https://www.readbyqxmd.com/read/28818704/orphan-drug-regulation-a-missed-opportunity-for-children-and-adolescents-with-cancer
#2
Gilles Vassal, Pam Kearns, Patricia Blanc, Nicole Scobie, Delphine Heenen, Andy Pearson
BACKGROUND: Oncology represents a major sector in the field of orphan drug development in Europe. The objective was to evaluate whether children and adolescents with cancer benefited from the Orphan Drug Regulation. METHODS: Data on orphan drug designations (ODDs) and registered orphan drugs from 8th August 2000 to 10th September 2016 were collected from the Community Register of medicinal products for human use. Assessment history, product information and existence of paediatric investigation plans were searched and retrieved from the European Medicine Agency website...
August 14, 2017: European Journal of Cancer
https://www.readbyqxmd.com/read/28817827/role-of-next-generation-sequencing-as-a-diagnostic-tool-for-the-evaluation-of-bone-and-soft-tissue-tumors
#3
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/28817624/ephb4-ephrinb2-therapeutics-in-rhabdomyosarcoma
#4
Matthew E Randolph, Megan M Cleary, Zia Bajwa, Matthew N Svalina, Michael C Young, Atiya Mansoor, Pali Kaur, Carol J Bult, Martin W Goros, Joel E Michalek, Sunny Xiang, James Keck, Valery Krasnoperov, Parkash Gill, Charles Keller
Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma affecting children and is often diagnosed with concurrent metastases. Unfortunately, few effective therapies have been discovered that improve the long-term survival rate for children with metastatic disease. Here we determined effectiveness of targeting the receptor tyrosine kinase, EphB4, in both alveolar and embryonal RMS either directly through the inhibitory antibody, VasG3, or indirectly by blocking both forward and reverse signaling of EphB4 binding to EphrinB2, cognate ligand of EphB4...
2017: PloS One
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
#5
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
#6
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/28811330/epithelial-to-mesenchymal-and-mesenchymal-to-epithelial-transition-in-mesenchymal-tumors-a-paradox-in-sarcomas
#7
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
#8
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/28808411/preoperative-factors-associated-with-infiltrative-histologic-growth-patterns-in-extremity-soft-tissue-sarcoma
#9
Jong Woong Park, Han-Soo Kim, Cheol Lee, Hye Jin Yoo, Ji Yeon Yun, Ilkyu Han
Soft tissue sarcoma (STS) with an infiltrative histologic growth pattern, when compared to STS with an expansile pattern, may pose difficulties in local control. Preoperative assessment of the presence of infiltrative histologic growth pattern would be helpful in deciding treatment strategies. A review of 144 patients who underwent surgery for extremity STS was performed. Microscopically, the histologic growth pattern was defined as infiltrative if the penetration of the tumor cells into the surrounding tissue was observed...
2017: Sarcoma
https://www.readbyqxmd.com/read/28802505/soft-tissue-tumors-of-uncertain-histogenesis-a-review-for-dermatopathologists
#10
REVIEW
Darya Buehler, Paul Weisman
The mesenchymal tumors discussed herein represent a heterogeneous group of neoplasms with distinctive morphologic, immunophenotypic, and molecular genetic features. These uncommon tumors often arise in the dermis and subcutis and can pose a major diagnostic challenge to dermatopathologists because they closely mimic melanoma, carcinoma, fibrous histiocytoma, schwannoma, or granulomatous inflammation. This article reviews the clinical presentation, histopathology, differential diagnosis, and diagnostic pitfalls of epithelioid sarcoma, clear cell sarcoma, perivascular epithelioid cell tumor, ossifying fibromyxoid tumor, pleomorphic hyalinizing angiectatic tumor, and hemosiderotic fibrolipomatous tumor...
September 2017: Clinics in Laboratory Medicine
https://www.readbyqxmd.com/read/28799063/clinical-outcomes-of-patients-with-epithelioid-sarcomas-impact-and-management-of-nodal-metastasis
#11
Hidetatsu Outani, Yoshinori Imura, Takaaki Tanaka, Satoshi Takenaka, Kazuya Oshima, Kenichiro Hamada, Shigeki Kakunaga, Susumu Joyama, Norifumi Naka, Ikuo Kudawara, Takafumi Ueda, Nobuhito Araki, Hideki Yoshikawa
PURPOSE: An epithelioid sarcoma is a rare histological subtype of a soft tissue sarcoma with a high local recurrence rate, which frequently shows lymph node metastasis. However, because of the rarity of this tumor, the impact of nodal metastasis and its appropriate management remain unclear. The present study investigated the clinical outcomes of patients with epithelioid sarcomas, with a focus on lymph node metastasis. METHODS: We retrospectively evaluated the clinical outcomes of 27 patients with epithelioid sarcomas treated between 1985 and 2015...
August 10, 2017: International Journal of Clinical Oncology
https://www.readbyqxmd.com/read/28797949/a-prediction-model-for-treatment-decisions-in-high-grade-extremity-soft-tissue-sarcomas-personalised-sarcoma-care-persarc
#12
Veroniek M van Praag, Anja J Rueten-Budde, Lee M Jeys, Minna Laitinen, Rob Pollock, Will Aston, Jos A van de Hage, P D Sander Dijkstra, Peter C Ferguson, Anthony M Griffin, Julie J Willeumier, Jay S Wunder, Michiel A J van de Sande, Marta Fiocco
BACKGROUND: To support shared decision-making, we developed the first prediction model for patients with primary soft-tissue sarcomas of the extremities (ESTS) which takes into account treatment modalities, including applied radiotherapy (RT) and achieved surgical margins. The PERsonalised SARcoma Care (PERSARC) model, predicts overall survival (OS) and the probability of local recurrence (LR) at 3, 5 and 10 years. AIM: Development and validation, by internal validation, of the PERSARC prediction model...
August 7, 2017: European Journal of Cancer
https://www.readbyqxmd.com/read/28797510/soft-tissue-tumors-rarely-presenting-primary-in-bone-diagnostic-pitfalls
#13
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/28794805/treatment-of-advanced-metastatic-soft-tissue-sarcoma-latest-evidence-and-clinical-considerations
#14
REVIEW
Gino K In, James S Hu, William W Tseng
Soft tissue sarcoma (STS) is a biologically heterogeneous malignancy with over 50 subtypes. Historically, there have been few systemic treatment options for this relatively rare disease. Traditional cytotoxic agents, such as anthracyclines, alkylating agents, and taxanes have limited clinical benefit beyond the first-line setting; across all high-grade STS subtypes, median overall survival remains approximately 12-18 months for advanced metastatic disease. The development of targeted therapies has led to recent US Food and Drug Administration approval of four new treatments for high-grade STS in the advanced metastatic setting...
August 2017: Therapeutic Advances in Medical Oncology
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
#15
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/28782739/laparoscopic-excision-of-leiomyosarcoma-of-inferior-vena-cava
#16
Pravin Rajendra Suryawanshi, Mohit Munesh Agrawal
Leiomyosarcoma of inferior vena cava (IVC) is a rare soft tissue tumour. Only 0.5% of all soft tissue sarcomas of adults and only 300 cases are reported till date. We describe our encounter with such a case where we were successful in radical excision of tumour with the use of vascular staplers on the IVC without compromising IVC lumen. Leiomyosarcoma of IVC is a rare entity, and laparoscopic excision of the tumour is possible in exophytic tumour.
August 1, 2017: Journal of Minimal Access Surgery
https://www.readbyqxmd.com/read/28780919/anthracycline-induced-cardiotoxicity-in-patients-with-paediatric-bone-sarcoma-and-soft-tissue-sarcoma
#17
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/28776571/expanded-molecular-profiling-of-myxofibrosarcoma-reveals-potentially-actionable-targets
#18
Ellen Heitzer, Sandra Sunitsch, Magdalena M Gilg, Birgit Lohberger, Beate Rinner, Karl Kashofer, Nicole Stündl, Peter Ulz, Joanna Szkandera, Andreas Leithner, Bernadette Liegl-Atzwanger
Myxofibrosarcomas are morphologically heterogeneous soft tissue sarcomas lacking a specific immunohistochemical expression profile and recurrent genetic changes. The study was designed to gain further insights into the molecular landscape of myxofibrosarcomas by targeted re-sequencing of known cancer driver hotspot mutations and the analysis of genomewide somatic copy number alterations. A well-defined group of myxofibrosarcomas, including myxofibrosarcomas G1 (n=6), myxofibrosarcomas G3 (n=7), myxofibrosarcomas with morphologically heterogeneous and independently selectable G1 and G3 areas within a tumor (n=8), and myxofibrosarcomas G3 with subsequent tumor recurrence (n=1) or metastatic disease (n=3) were evaluated...
August 4, 2017: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
https://www.readbyqxmd.com/read/28774857/alveolar-soft-part-sarcoma-metastatic-to-the-mandible-a-report-and-review-of-literature
#19
S M Peters, M A Perrino, A J Yoon, E M Philipone
Alveolar soft part sarcoma (ASPS) is a rare neoplasm constituting less than 1% of all soft tissue sarcomas. It tends to occur in the deep soft tissues of the lower extremities, however approximately 5-12% of cases are primary to the head and neck region. ASPS metastatic to the oral cavity is rare, with only four documented cases in the literature. Here, we present the case of a 29-year-old woman with ASPS metastatic to the mandible. To the best of our knowledge, this represents only the 5th documented case of ASPS metastatic to the oral cavity, and more specifically, the 3rd documented case of mandibular metastasis...
July 31, 2017: Journal of Stomatology, Oral and Maxillofacial Surgery
https://www.readbyqxmd.com/read/28768365/importance-of-preoperative-diagnosis-for-management-of-patients-with-suspected-retroperitoneal-sarcoma
#20
REVIEW
David E Gyorki, Peter F M Choong, John Slavin, Michael A Henderson
Soft tissue sarcoma is an umbrella term which encompasses over 60 histological tumour types. Approximately 15% of soft tissue sarcomas arise in the retroperitoneum. This complex group of tumours poses unique management challenges due to their often large size, histological heterogeneity and complexity of anatomical relationships. This review discusses the management of retroperitoneal tumours including the need for preoperative diagnosis, the evidence for neoadjuvant radiotherapy, the role of multivisceral resection and the importance of a multidisciplinary team approach...
August 2, 2017: ANZ Journal of Surgery
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