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Synovial sarcoma

Thomas Brodowicz, Olivier Mir, Jennifer Wallet, Antoine Italiano, Jean-Yves Blay, François Bertucci, Wolfgang Eisterer, Christine Chevreau, Sophie Piperno-Neumann, Emmanuelle Bompas, Thomas Ryckewaert, Bernadette Liegl-Antzwager, Julien Thery, Nicolas Penel, Axel Le Cesne, Marie-Cécile Le Deley
INTRODUCTION: The placebo-controlled phase-2 REGOSARC trial demonstrated the efficacy of regorafenib in patients with leiomyosarcoma, synovial sarcoma and other non-adipocytic sarcoma but not in liposarcoma. Patients initially allocated to placebo were allowed to receive regorafenib after progression. We report here an updated analysis of the trial including evaluation of regorafenib activity after cross-over. METHODS: From June 2013 to December 2014, 139 patients were enrolled in the non-adipocytic sarcoma cohorts...
June 11, 2018: European Journal of Cancer
Yihebali Chi, Zhiwei Fang, Xiao-Nan Hong, Yang Yao, Ping Sun, Guowen Wang, Feng Du, Yongkun Sun, Qiong Wu, Guofan Qu, Shusen Wang, Jianmin Song, Jianchun Yu, Yongkui Lu, Xia Zhu, Xiaohui Niu, Zhiyong He, Jinwan Wang, Hao Yu, Jianqiang Cai
PURPOSE: The prognosis for patients with refractory soft-tissue sarcoma (STS) is dismal. Anlotinib has previously shown antitumor activity on STS in preclinical and phase I studies. EXPERIMENTAL DESIGN: Patients 18 years and older, progressing after anthracycline-based chemotherapy, naïve from angiogenesis inhibitors, with at least one measurable lesion according to RECIST 1.1, were enrolled. The main subtypes eligible were: undifferentiated pleomorphic sarcoma (UPS), liposarcoma (LPS), leiomyosarcoma (LMS), synovial sarcoma (SS), fibrosarcoma (FS), alveolar soft part sarcoma (ASPS) and clear cell sarcoma (CCS)...
June 12, 2018: Clinical Cancer Research: An Official Journal of the American Association for Cancer Research
Marc A Morgan, Ali Shilatifard
In this issue of Cancer Cell, McBride and colleagues report that the synovial sarcoma SS18-SSX fusion drives BAF complex recruitment to bivalent domains repressed by PRC2 complex to orchestrate aberrant transcriptional activation. Redistribution of BAF localization is a major driver of synovial sarcoma proliferation and presents a promising therapeutic target.
June 11, 2018: Cancer Cell
Manoj Kumar Panigrahi, Gourahari Pradhan, Nibedita Sahoo, Pritinanda Mishra, Susama Patra, Prasanta Raghab Mohapatra
Synovial sarcoma (SS) is a malignant mesenchymal tumor with variable epithelial differentiation that affects mostly young adults and can arise at any anatomic site. Primary intrathoracic SS is very rare accounting for <0.5% of all lung tumors. Most commonly, it arises from the lung followed by pleura and mediastinum. Primary pulmonary SS (PPSS) affects both sexes equally with no preference for any hemithorax. The morphology, immunostaining properties, cytogenetic features, and management strategy of PPSS are similar to that of soft tissue SS...
April 2018: Journal of Cancer Research and Therapeutics
E N Singh, S Kumar, U S Agarwal, R Meena, S Purohit
No abstract text is available yet for this article.
June 11, 2018: Clinical and Experimental Dermatology
Sandra P D'Angelo, Luca Melchiori, Melinda S Merchant, Donna B Bernstein, John Glod, Rosandra N Kaplan, Stephan A Grupp, William D Tap, Karen Chagin, Gwendolyn K Binder, Samik Basu, Daniel E Lowther, Ruoxi Wang, Natalie Bath, Alex Tipping, Gareth Betts, Indu Ramachandran, Jean-Marc Navenot, Hua Zhang, Daniel K Wells, Erin Van Winkle, Gabor Kari, Trupti Trivedi, Tom Holdich, Lini N Pandite, Rafael Amado, Crystal L Mackall
We evaluated safety and activity of autologous T cells expressing NY-ESO-1c259, an affinity-enhanced T cell receptor (TCR) recognizing an HLA-A2-restricted NY-ESO-1/LAGE-1a-derived peptide, in patients with metastatic synovial sarcoma (NY-ESO-1c259T cells). Confirmed antitumor responses occurred in 50% of patients (6/12) and were characterized by tumor shrinkage over several months. Circulating NY-ESO-1c259T cells were present post-infusion in all patients and persisted for at least 6 months in all responders...
June 11, 2018: Cancer Discovery
Nicolas De Hous, Bernard Paelinck, Jan Van den Brande, Vasiliki Siozopoulou, Steven Laga
No abstract text is available yet for this article.
June 10, 2018: Journal of Cardiac Surgery
Nivedita Sharma, Nebu Abraham George, Rajesh Singh, Elizabeth Mathew Iype, Bipin T Varghese, Shaji Thomas
Head and neck soft tissue sarcoma (HNSTS) is a rare neoplasm accounting for 1% of all head and neck tumours. Because of rarity and varied biological behaviour among various subtypes, knowledge about these tumours is limited. This study aimed at analysing clinicopathological, recurrence and survival pattern of surgically treated HNSTS. Case records of 28 patients of HNSTS who underwent surgery at the Regional Cancer Centre (RCC), Trivandrum (India) between 2002 and 2012 were analysed retrospectively for demographic profile, clinical features, treatment given, recurrence pattern and outcome...
June 2018: Indian Journal of Surgical Oncology
(no author information available yet)
SS18-SSX targets BAF complexes away from enhancers to broad domains where they activate bivalent genes.
June 8, 2018: Cancer Discovery
Anne-Laure Giraudet, Philippe Alexandre Cassier, Chicaco Iwao-Fukukawa, Gwenaelle Garin, Jean-Noël Badel, David Kryza, Sylvie Chabaud, Laurence Gilles-Afchain, Gilles Clapisson, Claude Desuzinges, David Sarrut, Adrien Halty, Antoine Italiano, Masaharu Mori, Takuya Tsunoda, Toyomasa Katagiri, Yusuke Nakamura, Laurent Alberti, Claire Cropet, Simon Baconnier, Sandrine Berge-Montamat, David Pérol, Jean-Yves Blay
BACKGROUND: Synovial Sarcomas (SS) are rare tumors occurring predominantly in adolescent and young adults with a dismal prognosis in advanced phases. We report a first-in-human phase I of monoclonal antibody (OTSA-101) targeting FZD10, overexpressed in most SS but not present in normal tissues, labelled with radioisotopes and used as a molecular vehicle to specifically deliver radiation to FZD10 expressing SS lesions. METHODS: Patients with progressive advanced SS were included...
June 8, 2018: BMC Cancer
Diksha Yadav, Shipra Agarwal, Arundhati Sharma, Ekta Malik, Devasenathipathy Kandasamy, Alok Thakar, Rajni Yadav, Adarsh Barward, Lata Kini
Synovial sarcoma (SS) is a high-grade soft tissue sarcoma, characterized by t(X;18)(p11;q11), resulting in fusion of genes SYT on chromosome 18 and SSX 1 or SSX2 or SSX 4 on chromosome X.1 SS commonly involves extremities (83%).1 Head and neck involvement is rare, constituting only 2.5-3.5% of all sarcomas in this region.2 This article is protected by copyright. All rights reserved.
June 6, 2018: Cytopathology: Official Journal of the British Society for Clinical Cytology
Yin P Hung, Christopher D M Fletcher, Jason L Hornick
AIMS: Infantile fibrosarcoma is characterized by intersecting fascicles of spindle cells and ETV6-NTRK3 gene fusion in most cases. Given histologic overlap with other spindle-cell tumors, the diagnosis can be challenging and often requires molecular confirmation. A recently developed pan-TRK antibody shows promise for identifying tumors with NTRK fusions. The purpose of this study was to evaluate the potential diagnostic utility of pan-TRK immunohistochemistry for infantile fibrosarcoma...
June 4, 2018: Histopathology
Vickie Y Jo, Adrián Mariño-Enríquez, Christopher D M Fletcher, Jason L Hornick
Biphenotypic sinonasal sarcoma (BSNS) is a distinctive, anatomically restricted, low-grade spindle cell sarcoma that shows considerable histologic overlap with other cellular spindle cell neoplasms. This tumor type shows both myogenic and neural differentiation, which can be demonstrated by immunohistochemistry; however, the available diagnostic markers are relatively nonspecific. BSNS is characterized by PAX3 rearrangements, with MAML3 as the most common fusion partner. Our aim was to determine whether immunohistochemistry using a monoclonal PAX3 antibody could distinguish BSNS from potential histologic mimics, as well as to evaluate a widely available polyclonal PAX8 antibody, which is known to cross-react with other paired box transcription factor family members...
May 31, 2018: American Journal of Surgical Pathology
Matthew J McBride, John L Pulice, Hannah C Beird, Davis R Ingram, Andrew R D'Avino, Jack F Shern, Gregory W Charville, Jason L Hornick, Robert T Nakayama, Enrique M Garcia-Rivera, Dejka M Araujo, Wei-Lien Wang, Jen-Wei Tsai, Michelle Yeagley, Andrew J Wagner, P Andrew Futreal, Javed Khan, Alexander J Lazar, Cigall Kadoch
Synovial sarcoma (SS) is defined by the hallmark SS18-SSX fusion oncoprotein, which renders BAF complexes aberrant in two manners: gain of SSX to the SS18 subunit and concomitant loss of BAF47 subunit assembly. Here we demonstrate that SS18-SSX globally hijacks BAF complexes on chromatin to activate an SS transcriptional signature that we define using primary tumors and cell lines. Specifically, SS18-SSX retargets BAF complexes from enhancers to broad polycomb domains to oppose PRC2-mediated repression and activate bivalent genes...
May 23, 2018: Cancer Cell
Fatima Zahra Mrabet, Hafsa El Ouazzani, Leila El Akkari, Sanaa Hammi, Jamal Eddine Bourkadi, Fouad Zouaidia
Primary pleuropulmonary synovial sarcoma is extremely rare. The diagnosis can only be made after having eliminated an extrapleuropulmonary localization in the past and at the time of diagnosis. Our presentation is about a 40-year-old woman having a cough and dyspnea since three weeks ago; imaging had showed a left pleurisy with pleuropulmonary process. Histological study of the biopsy confirmed the diagnosis of pleuropulmonary synovial sarcoma. PET-SCAN had not identified any extrathoracic localization. This tumor is known for its aggressive nature and high risk of metastasis...
2018: Case Reports in Pulmonology
Ana Cristina Vargas, Christina Selinger, Laveniya Satgunaseelan, Wendy A Cooper, Ruta Gupta, Paul Stalley, Wendy Brown, Judy Soper, Julie Schatz, Richard Boyle, David M Thomas, Martin H N Tattersall, Vivek Bhadri, Fiona Maclean, Sally Fiona Bonar, Richard A Scolyer, Rooshdiya Z Karim, Stanley W McCarthy, Annabelle Mahar, Sandra A O'Toole
AIM: Fluorescence in situ hybridization (FISH) is an important ancillary tool for the classification of bone/soft tissue (BST) tumors. The aim of this study was to evaluate the contribution of FISH to the final classification of common BST entities in the molecular pathology department of the Royal Prince Alfred Hospital (RPAH), which is one of the most important referral centers for the management of sarcomas in Australia. METHODS: All routine diagnostic FISH tests performed on BST formalin-fixed paraffin embedded (FFPE) tissue specimens at the RPAH in a 5-year period (February, 2010-November, 2015) were reviewed...
May 28, 2018: Asia-Pacific Journal of Clinical Oncology
Yuqiao Diao, Ping Zhang, Ruoheng Dai, Jianfa Xu, Helin Feng
PURPOSE: Previous studies have shown a correlation between the expression of H3K27me3 and pathological characteristics of malignant tumors. This study aimed to investigate the association of H3K27me3 and VEGF expression with clinical outcomes of synovial sarcoma patients. METHODS: This study included 48 patients with synovial sarcoma. H3K27me3 and VEGF levels were evaluated by immunohistochemical staining, and their correlation with clinical parameters was analyzed by Spearman's and Pearson's test...
May 20, 2018: Pathology, Research and Practice
Z Zhou, Y Q Li, D D Li, B Liu, G Y Miao, X P Xiao, X W Peng
We report a case which involves the maxillary sinus by original of synovial sarcoma of infratemporal fossa and pterygopalatine. A 40-years-old man presented with a history of tumor under the earlobe. It is derived from the synovial sarcoma under temporal fossa after tumor excision. The patient is limited to open his mouth and nasal obstruction. The nasal pathologic examination consider synovial sarcoma.We report the case to improve doctors' comprehension of its pathogenesis,clinical manifestations,teeatment and prognosis...
October 5, 2017: Journal of Clinical Otorhinolaryngology, Head, and Neck Surgery
Pei-Wen Fan, Li Huang, Xue-Mei Chang, Ya-Ning Feng, Xuan Yao, Yan-Chun Peng, Tao Dong, Ruo-Zheng Wang
Background: Development of innovative immunotherapy is imperative to improve the poor survival of the nasopharyngeal carcinoma (NPC) patients. In this study, we evaluated the T cell response to melanoma-associated antigen (MAGE)-A1, MAGE-A3, or synovial sarcoma X-2 (SSX-2) in the peripheral blood of treatment-naive NPC patients. The relationship of responses among the three proteins and the human leukocyte antigen (HLA)-A types were analyzed to provide evidence of designing novel therapy...
June 5, 2018: Chinese Medical Journal
Ngoc T Hoang, Luis A Acevedo, Michael J Mann, Bhairavi Tolani
Soft-tissue sarcomas are rare malignant tumors arising from connective tissues and have an overall incidence of about five per 100,000 per year. While this diverse family of malignancies comprises over 100 histological subtypes and many molecular aberrations are prevalent within specific sarcomas, very few are therapeutically targeted. Instead of utilizing molecular signatures, first-line sarcoma treatment options are still limited to traditional surgery and chemotherapy, and many of the latter remain largely ineffective and are plagued by disease resistance...
2018: Cancer Management and Research
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