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Clear cell sarcoma

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https://www.readbyqxmd.com/read/29923360/morphology-quiz-cytology-of-a-palmar-swelling-with-axillary-lymphadenopathy
#1
Parikshaa Gupta, Arvind Rajwanshi
Clear cell sarcoma of soft tissue (CCS-ST), also known as malignant melanoma of soft tissue and clear cell sarcoma of tendons and aponeuroses, is a rare high-grade, soft tissue sarcoma, currently included in the WHO category of soft tissue tumors with uncertain differentiation. Establishing a cytodiagnosis is challenging as there are many morphological mimics. Knowledge about the characteristic cytomorphological and immunocytochemical features of this entity is important to establish an accurate cytodiagnosis...
June 19, 2018: Cytopathology: Official Journal of the British Society for Clinical Cytology
https://www.readbyqxmd.com/read/29915264/recurrent-intragenic-rearrangements-of-egfr-and-braf-in-soft-tissue-tumors-of-infants
#2
Jenny Wegert, Christian Vokuhl, Grace Collord, Martin Del Castillo Velasco-Herrera, Sarah J Farndon, Charlotte Guzzo, Mette Jorgensen, John Anderson, Olga Slater, Catriona Duncan, Sabrina Bausenwein, Heike Streitenberger, Barbara Ziegler, Rhoikos Furtwängler, Norbert Graf, Michael R Stratton, Peter J Campbell, David Tw Jones, Christian Koelsche, Stefan M Pfister, William Mifsud, Neil Sebire, Monika Sparber-Sauer, Ewa Koscielniak, Andreas Rosenwald, Manfred Gessler, Sam Behjati
Soft tissue tumors of infancy encompass an overlapping spectrum of diseases that pose unique diagnostic and clinical challenges. We studied genomes and transcriptomes of cryptogenic congenital mesoblastic nephroma (CMN), and extended our findings to five anatomically or histologically related soft tissue tumors: infantile fibrosarcoma (IFS), nephroblastomatosis, Wilms tumor, malignant rhabdoid tumor, and clear cell sarcoma of the kidney. A key finding is recurrent mutation of EGFR in CMN by internal tandem duplication of the kinase domain, thus delineating CMN from other childhood renal tumors...
June 18, 2018: Nature Communications
https://www.readbyqxmd.com/read/29914686/establishment-and-characterization-of-a-cell-line-from-a-feline-histiocytic-sarcoma
#3
Ryo Miyamoto, Sena Kurita, Hiroyuki Tani, Masato Kobayashi, Soudai Sugiura, Kae Shigihara, Yuka Sato, Yoshikazu Tanaka, Kyoichi Tamura, Makoto Bonkobara
Feline histiocytic sarcoma (HS) is an aggressive and uncommon tumor originating from dendritic cells/macrophages. Here, a feline HS cell line, FHS-1, was established from a case of feline HS and characterized. Immunohistochemically, FHS-1 cells were positive for vimentin and Iba-1, and negative for MHC class II and CD163. FHS-1 cells were positive for α-naphthyl butyrate esterase staining, which was clearly inhibited by sodium fluoride. FHS-1 cells had phagocytic and antigen uptake/processing activities. Moreover, FHS-1 cells were tested for susceptibility to feline infectious peritonitis virus (FIPV) strain 79-1146; however, this cell line was not susceptible to this viral strain...
July 2018: Veterinary Immunology and Immunopathology
https://www.readbyqxmd.com/read/29898995/ews-ets-driven-ewing-sarcoma-requires-bet-bromodomain-proteins
#4
Paradesi Naidu Gollavilli, Aishwarya Pawar, Kari Wilder-Romans, Natesan Ramakrishnan, Carl G Engelke, Vijaya L Dommeti, Pranathi M Krishnamurthy, Archana Nallasivam, Ingrid J Apel, Tianlei Xu, Zhaohui Qin, Felix Y Feng, Irfan A Asangani
The EWS/ETS fusion transcription factors drive Ewing sarcoma (EWS) by orchestrating an oncogenic transcription program. Therapeutic targeting of EWS/ETS has been unsuccessful; however, identifying mediators of the EWS/ETS function could offer new therapeutic options. Here we describe the dependency of EWS/ETS-driven transcription upon chromatin reader BET bromdomain proteins and investigate the potential of BET inhibitors in treating EWS. EWS/FLI1 and EWS/ERG were found in a transcriptional complex with BRD4, and knockdown of BRD2/3/4 significantly impaired the oncogenic phenotype of EWS cells...
June 13, 2018: Cancer Research
https://www.readbyqxmd.com/read/29895706/safety-and-efficacy-of-anlotinib-a-multikinase-angiogenesis-inhibitor-in-patients-with-refractory-metastatic-soft-tissue-sarcoma
#5
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
https://www.readbyqxmd.com/read/29881993/primary-intracranial-spindle-cell-sarcoma-with-rhabdomyosarcoma-like-features-share-a-highly-distinct-methylation-profile-and-dicer1-mutations
#6
Christian Koelsche, Martin Mynarek, Daniel Schrimpf, Luca Bertero, Jonathan Serrano, Felix Sahm, David E Reuss, Yanghao Hou, Daniel Baumhoer, Christian Vokuhl, Uta Flucke, Iver Petersen, Wolfgang Brück, Stefan Rutkowski, Sandro Casavilca Zambrano, Juan Luis Garcia Leon, Rosdali Yesenia Diaz Coronado, Manfred Gessler, Oscar M Tirado, Jaume Mora, Javier Alonso, Xavier Garcia Del Muro, Manel Esteller, Dominik Sturm, Jonas Ecker, Till Milde, Stefan M Pfister, Andrey Korshunov, Matija Snuderl, Gunhild Mechtersheimer, Ulrich Schüller, David T W Jones, Andreas von Deimling
Patients with DICER1 predisposition syndrome have an increased risk to develop pleuropulmonary blastoma, cystic nephroma, embryonal rhabdomyosarcoma, and several other rare tumor entities. In this study, we identified 22 primary intracranial sarcomas, including 18 in pediatric patients, with a distinct methylation signature detected by array-based DNA-methylation profiling. In addition, two uterine rhabdomyosarcomas sharing identical features were identified. Gene panel sequencing of the 22 intracranial sarcomas revealed the almost unifying feature of DICER1 hotspot mutations (21/22; 95%) and a high frequency of co-occurring TP53 mutations (12/22; 55%)...
June 7, 2018: Acta Neuropathologica
https://www.readbyqxmd.com/read/29879220/therapeutic-potential-of-cpi-613-for-targeting-tumorous-mitochondrial-energy-metabolism-and-inhibiting-autophagy-in-clear-cell-sarcoma
#7
Yuki Egawa, Chiemi Saigo, Yusuke Kito, Toshiaki Moriki, Tamotsu Takeuchi
Clear cell sarcoma (CCS) is an aggressive type of soft tissue tumor that is associated with high rates of metastasis. In the present study, we found that CPI-613, which targets tumorous mitochondrial energy metabolism, induced autophagosome formation followed by lysosome fusion in HS-MM CCS cells in vitro. Interestingly, CPI-613 along with chloroquine, which inhibits the fusion of autophagosomes with lysosomes, significantly induced necrosis of HS-MM CCS cell growth in vitro. Subsequently, we established a murine orthotropic metastatic model of CCS and evaluated the putative suppressive effect of a combination of CPI-613 and chloroquine on CCS progression...
2018: PloS One
https://www.readbyqxmd.com/read/29851702/diffuse-strong-bcor-immunoreactivity-is-a-sensitive-and-specific-marker-for-clear-cell-sarcoma-of-the-kidney-ccsk-in-pediatric-renal-neoplasia
#8
Pedram Argani, Bruce Pawel, Sara Szabo, Miguel Reyes-Múgica, Charles Timmons, Cristina R Antonescu
No abstract text is available yet for this article.
May 30, 2018: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/29788832/feline-injection-site-sarcoma-immunohistochemical-characteristics
#9
Carolina S Carneiro, Genilson F de Queiroz, Ana Cbcf Pinto, Maria Lz Dagli, Julia M Matera
Objectives Feline injection site sarcoma (FISS) is a rapid growing locally aggressive tumor with a low metastatic rate. Its histologic features are clearly defined, but there are few studies regarding its immunohistochemical characteristics. The present study investigated the immunohistochemical characteristics of 21 cases of FISS. Methods FISSs from 12 male and nine female cats, 20 mixed-breed and one Siamese, were included in the study. After histopathological diagnosis, additional histologic sections were immunostained for vimentin, cytokeratin, desmin, S100 protein, viral feline leukemia virus (FeLV) particles, cyclooxygenase 2 (COX-2) and c-KIT...
May 1, 2018: Journal of Feline Medicine and Surgery
https://www.readbyqxmd.com/read/29768052/options-for-treating-different-soft-tissue-sarcoma-subtypes
#10
Isabelle Ray-Coquard, Delphine Serre, Peter Reichardt, Javier Martín-Broto, Sebastian Bauer
Management of soft tissue sarcoma is increasingly subtype-dependent. Surgery is recommended for uterine leiomyosarcoma, with trabectedin being the preferred option for advanced disease when the treatment goal is long-term tumor stabilization. Liposarcoma subgroups are characterized by distinctive morphologies and genetics, different patterns of disease progression and clinical behavior, and variable responses to treatment. Genetic analysis of sarcomas has provided insights into pathogenesis with potential for developing new molecular targets...
May 2018: Future Oncology
https://www.readbyqxmd.com/read/29743654/expression-of-the-transcription-factor-zbtb46-distinguishes-human-histiocytic-disorders-of-classical-dendritic-cell-origin
#11
Ansuman T Satpathy, Ryanne A Brown, Ellen Gomulia, Carlos G Briseño, Maxwell R Mumbach, Zenggang Pan, Kenneth M Murphy, Yasodha Natkunam, Howard Y Chang, Jinah Kim
Distinguishing classical dendritic cells from other myeloid cell types is complicated by the shared expression of cell surface markers. ZBTB46 is a zinc finger and BTB domain-containing transcription factor, which is expressed by dendritic cells and committed dendritic cell precursors, but not by plasmacytoid dendritic cells, monocytes, macrophages, or other immune cell populations. In this study, we demonstrate that expression of ZBTB46 identifies human dendritic cell neoplasms. We examined ZBTB46 expression in a range of benign and malignant histiocytic disorders and found that ZBTB46 is able to clearly define the dendritic cell identity of many previously unclassified histiocytic disease subtypes...
May 9, 2018: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
https://www.readbyqxmd.com/read/29741569/activity-and-safety-of-crizotinib-in-patients-with-advanced-clear-cell-sarcoma-with-met-alterations-european-organization-for-research-and-treatment-of-cancer-phase-ii-trial-90101-create
#12
P Schöffski, A Wozniak, S Stacchiotti, P Rutkowski, J-Y Blay, L H Lindner, S J Strauss, A Anthoney, F Duffaud, S Richter, V Grünwald, M G Leahy, P Reichardt, J Sufliarsky, W T van der Graaf, R Sciot, M Debiec-Rychter, T van Cann, S Marréaud, M Lia, T Raveloarivahy, L Collette, S Bauer
No abstract text is available yet for this article.
May 5, 2018: Annals of Oncology: Official Journal of the European Society for Medical Oncology
https://www.readbyqxmd.com/read/29727806/neoplasms-associated-deaths-in-hiv-1-infected-and-non-infected-patients-in-bahia-brazil
#13
Marinho Marques, Estela Luz, Mateus Leal, João Vitor Oliveira, Rejane Patrício, Eduardo Martins Netto, Carlos Brites
BACKGROUND: HIV-infected patients are at a higher risk to develop malignancies than general population. Although AIDS-related malignancies are a common feature of late-stage disease, patients under successful antiretroviral therapy also have an increased risk for development of non-AIDS malignancies. OBJECTIVE: To compare the frequency and characteristics of adults HIV-infected patients and general population who died of malignancies in Bahia, Brazil from January 2000 to December 2010...
June 2018: Cancer Epidemiology
https://www.readbyqxmd.com/read/29693796/a-phase-1-study-of-cabozantinib-in-children-and-adolescents-with-recurrent-or-refractory-solid-tumors-including-cns-tumors-trial-advl1211-a-report-from-the-children-s-oncology-group
#14
Meredith K Chuk, Brigitte C Widemann, Charles G Minard, Xiaowei Liu, AeRang Kim, Melanie Brooke Bernhardt, Rachel A Kudgus, Joel M Reid, Stephan D Voss, Susan Blaney, Elizabeth Fox, Brenda J Weigel
BACKGROUND: We conducted a phase 1 trial to determine the maximum tolerated dose (MTD), toxicity profile, pharmacokinetics (PK), pharmacodynamics (PD), and preliminary activity of cabozantinib in children with refractory or relapsed solid tumors. METHODS: Patients received cabozantinib tablets on a continuous dosing schedule in a rolling-six escalating phase 1 trial design. PK and PD studies were performed. RESULTS: Forty-one patients, median (range) age 13 (4-18) years, received cabozantinib to achieve a weekly cumulative dose equivalent to 30 (n = 6), 40 (n = 23)...
April 25, 2018: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/29676368/clear-cell-sarcoma-arising-from-paraspinal-ligament-of-thoracic-spine-report-of-an-unusual-case
#15
Ranjan Agrawal, Prabal Deb, Jagdamba Sharan, Parbodh Kumar
Clear cell sarcoma (CCS) is an unusual but aggressive soft-tissue tumor with an incidence of <1% of all soft-tissue sarcomas. It was previously termed "malignant melanoma of soft parts" due to its resemblance clinically and morphologically. Normally CCS is seen in patients aged 20-40 years. A rare case of CCS of paraspinal ligament in a 5-year-old boy is being reported. Histopathology and immunohistochemistry markers confirmed the diagnosis. The present case is unique since the entity itself is rare and also due to its occurrence in a child...
April 2018: Indian Journal of Pathology & Microbiology
https://www.readbyqxmd.com/read/29671892/frequent-loss-of-claudin-4-expression-in-dedifferentiated-and-undifferentiated-endometrial-carcinomas
#16
Basile Tessier-Cloutier, Robert A Soslow, Colin J R Stewart, Martin Köbel, Cheng-Han Lee
AIMS: Dedifferentiated endometrial carcinomas (DDECs)/undifferentiated endometrial carcinomas (UECs) are aggressive endometrial cancers with frequent genomic inactivation of core components of switch/sucrose non-fermentable (SWI/SNF) complex proteins. Claudin-4, an epithelial intercellular tight junction protein, was recently found to be expressed in SWI/SNF-deficient undifferentiated carcinomas but not in SWI/SNF-deficient sarcomas. The aim of this study was to examine claudin-4 expression in UECs/DDECs and other high-grade uterine carcinomas...
April 19, 2018: Histopathology
https://www.readbyqxmd.com/read/29661713/review-with-novel-markers-facilitates-precise-categorization-of-41-cases-of-diagnostically-challenging-undifferentiated-small-round-cell-tumors-a-clinicopathologic-immunophenotypic-and-molecular-analysis
#17
Isidro Machado, Akihiko Yoshida, María Gema Nieto Morales, Lucas Faria Abrahão-Machado, Samuel Navarro, Julia Cruz, Javier Lavernia, Antonina Parafioriti, Piero Picci, Antonio Llombart-Bosch
BACKGROUND: Despite extensive immunohistochemical (IHC) and molecular studies combined with morphologic findings, a group of round/ovoid cell tumors histologically similar to Ewing sarcomas (ES) but lacking EWSR1-rearrangements may remain unclassifiable. DESIGN: We retrospectively analyzed 41 Ewing-like tumors (formalin-fixed, paraffin-embedded) previously determined as negative or non-informative for EWSR1-rearrangements by FISH and/or RT-PCR. A new histopathology revision and additional IHC and molecular analyses were carried out in order to investigate whether additional IHC and/or molecular testing in combination with the morphological findings may help in reaching a definitive diagnosis...
November 29, 2017: Annals of Diagnostic Pathology
https://www.readbyqxmd.com/read/29626598/ewsr1-nfatc2-gene-fusion-in-a-soft-tissue-tumor-with-epithelioid-round-cell-morphology-and-abundant-stroma-a-case-report-and-review-of-the-literature
#18
Jarish N Cohen, Amit J Sabnis, Gregor Krings, Soo-Jin Cho, Andrew E Horvai, Jessica L Davis
Mesenchymal round cell tumors are a diverse group of neoplasms defined by primitive, often high-grade cytomorphology. The most common molecular alterations detected in these tumors are gene rearrangements involving EWSR1 to one of many fusion partners. Rare EWSR1-NFATC2 gene rearrangements, corresponding to a t(20;22) gene translocation, have been described in mesenchymal tumors with clear round cell morphology and a predilection for the skeleton. We present a case of a tumor harboring the EWSR1-NFATC2 gene fusion arising in the subcutaneous tissue of a young woman...
April 4, 2018: Human Pathology
https://www.readbyqxmd.com/read/29623567/clear-cell-sarcoma-like-tumor-of-the-gastrointestinal-tract
#19
Gokce Askan, Faruk Erdem Kombak, Ipek Erbarut Seven, Olca Basturk
No abstract text is available yet for this article.
April 6, 2018: Journal of Gastrointestinal Cancer
https://www.readbyqxmd.com/read/29607435/renal-clear-cell-sarcoma-presenting-as-a-spontaneous-renal-hematoma-a-rare-presentation
#20
Zeynep Canan Ozdemir, Burcu Ayvaci, Yeter Duzenli Kar, Mehmet Oguzman, Mehmet Surhan Arda, Mustafa Fuat Acikalin, Ozcan Bor
Clear cell sarcoma of the kidney (CCSK) is an uncommon renal neoplasm of childhood. It represents between 2% and 9% of all pediatric renal tumors, and generally arises before the age of 5 years. It often mimics other pediatric renal tumors. Presently described is the case of a 7-year-old girl who presented with complaints of vomiting and abdominal pain. Abdominal ultrasonography revealed a right renal mass, and the patient developed a renal hematoma a few hours after admission. The patient underwent a nephroureterectomy with a provisional diagnosis of Wilms tumor; however, histopathological examination of a specimen revealed CCSK...
2018: Northern Clinics of Istanbul
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