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Sarcoma, high grade

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https://www.readbyqxmd.com/read/29679497/ewsr1-patz1-gene-fusion-may-define-a-new-glioneuronal-tumor-entity
#1
Aurore Siegfried, Audrey Rousseau, Claude-Alain Maurage, Sarah Pericart, Yvan Nicaise, Frederic Escudie, David Grand, Alix Delrieu, Anne Gomez-Brouchet, Sophie Le Guellec, Camille Franchet, Sergio Boetto, Matthieu Vinchon, Jean-Christophe Sol, Franck-Emmanuel Roux, Valerie Rigau, Anne-Isabelle Bertozzi, David Tw Jones, Dominique Figarella-Branger, Emmanuelle Uro-Coste
We investigated the challenging diagnostic case of a ventricular cystic glioneuronal tumor with papillary features, by RNA sequencing using the Illumina TruSight RNA Fusion panel. We did not retrieve the SLC44A1-PRKCA fusion gene specific for papillary glioneuronal tumor, but an EWSR1-PATZ1 fusion transcript. RT-PCR followed by Sanger sequencing confirmed the EWSR1-PATZ1 fusion. It matched with canonic EWSR1 fusion oncogene, juxtaposing the entire N terminal transcriptional activation domain of EWSR1 gene and the C terminal DNA binding domain of a transcription factor gene, PATZ1...
April 21, 2018: Brain Pathology
https://www.readbyqxmd.com/read/29671892/frequent-loss-of-claudin-4-expression-in-dedifferentiated-and-undifferentiated-endometrial-carcinomas
#2
Basile Tessier-Cloutier, Robert A Soslow, Colin J R Stewart, Martin Köbel, Cheng-Han Lee
INTRO: Dedifferentiated/undifferentiated endometrial carcinomas (DDEC/UEC) 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 SWI/SNF-deficient sarcomas. The aim of this study is to examine claudin-4 expression in UEC/DDEC and other high-grade uterine carcinomas...
April 19, 2018: Histopathology
https://www.readbyqxmd.com/read/29670090/inhibition-of-cyclin-dependent-kinase-4-as-a-potential-therapeutic-strategy-for-treatment-of-synovial-sarcoma
#3
Xiaoyang Li, Nicole A Seebacher, Cassandra Garbutt, Hangzhan Ma, Peng Gao, Tao Xiao, Francis J Hornicek, Zhenfeng Duan
Synovial sarcoma is a highly aggressive but rare form of soft tissue malignancy that primarily affects the extremities of the arms or legs, for which current chemotherapeutic agents have not been proven to be very effective. The cyclin-dependent kinase 4/6-retinoblastoma protein (CDK4/6-Rb) pathway of cell cycle control is known to be aberrant in a large proportion of cancers. Recently, CDK4 inhibitors have successfully been used pre-clinically for the treatment of many human cancers, and in 2015, following the success of clinical trials, the FDA approved the first selective CDK4/6 inhibitor, palbociclib, for the treatment of endocrine therapy resistant breast cancers...
April 18, 2018: Cell Death & Disease
https://www.readbyqxmd.com/read/29663170/racial-ethnic-disparities-and-incidence-of-malignant-peripheral-nerve-sheath-tumors-results-from-the-surveillance-epidemiology-and-end-results-program-2000-2014
#4
Erin C Peckham-Gregory, Roberto E Montenegro, David A Stevenson, David H Viskochil, Michael E Scheurer, Philip J Lupo, Joshua D Schiffman
BACKGROUND: Malignant peripheral nerve sheath tumors (MPNSTs) are rare tumors, generally high-grade, and comprise ~ 5-10% of soft tissue sarcomas. Over two-thirds of MPNSTs metastasize, and upwards of 40% clinically recur. Etiologic risk factors for MPNSTs are historically understudied. There is evidence to suggest MPNST incidence differs across racial/ethnic groups in pediatric populations. Therefore, we sought to estimate differences in MPNST incidence by race/ethnicity among all ages in the United States...
April 16, 2018: Journal of Neuro-oncology
https://www.readbyqxmd.com/read/29660202/molecular-biomarkers-for-uterine-leiomyosarcoma-and-endometrial-stromal-sarcoma
#5
REVIEW
Hideaki Tsuyoshi, Yoshio Yoshida
Uterine leiomyosarcoma (u{\hyphen}LMS) and endometrial stromal sarcoma (ESS) are among the most frequent soft tissue sarcomas, which, in adults, lead to fatal lung metastases and have an extremely poor prognosis. Due to their rarity and heterogeneity, there are no suitable biomarkers for diagnosis and prognosis, though some biomarker candidates have appeared. Recently, The Cancer Genome Atlas (TCGA) projects dealing with u{\hyphen}LMS confirmed mutations and deletions in RB1, TP53, and PTEN. In addition, whole{\hyphen}exome sequencing of u{\hyphen}LMS has confirmed and demonstrated frequent alterations in TP53, RB1, α-thalassemia/mental retardation syndrome X-linked (ATRX), and mediator complex subunit 12 (MED12)...
April 16, 2018: Cancer Science
https://www.readbyqxmd.com/read/29658583/intraductal-papillary-mucinous-neoplasms-of-the-pancreas-clinical-association-with-kras
#6
Xiao Yan Chang, Yan Wu, Yuan Li, Jing Wang, Jie Chen
Intraductal papillary mucinous neoplasms of the pancreas (IPMN) are among the most important precancerous lesions in the pancreas. V‑Ki‑ras 2 Kirsten rat sarcoma viral oncogene homolog (KRAS) is one of the most important genes involved in pancreatic neoplasms, and exhibits a high mutation rate in pancreatic ductal adenocarcinomas and pancreatic intraepithelial neoplasia. The present study aimed to further elucidate the associations among IPMN subtypes (gastric, intestinal, pancreatobiliary and oncocytic), pathological classifications [low‑grade, intermediate‑grade, and high‑grade IPMN, and associated minimally invasive carcinoma (invasive depth ≤0...
April 12, 2018: Molecular Medicine Reports
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
#7
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/29625604/apatinib-for-advanced-sarcoma-results-from-multiple-institutions-off-label-use-in-china
#8
Lu Xie, Wei Guo, Ye Wang, Taiqiang Yan, Tao Ji, Jie Xu
BACKGROUND: Anti-angiogenesis Tyrosine kinase inhibitors (TKIs) have been proved to show promising effects on prolonging progression-free survival (PFS) for advanced sarcoma after failure of standard multimodal Therapy. Methylsulfonic apatinib is one of those TKIs which specifically inhibits VEGFR-2. This paper summarizes the experience of three Peking University affiliated hospitals in off-label use of apatinib in the treatment of extensively pre-treated sarcoma. METHODS: We retrospectively analysed files of patients with advanced sarcoma not amenable to curative treatment, who were receiving an apatinib-containing regimen between June 1, 2015 and December 1, 2016...
April 6, 2018: BMC Cancer
https://www.readbyqxmd.com/read/29623468/misses-and-near-misses-in-diagnosing-nodular-fasciitis-and-morphologically-related-reactive-myofibroblastic-proliferations-experience-of-a-referral-center-with-emphasis-on-frequency-of-usp6-gene-rearrangements
#9
Ramona Erber, Abbas Agaimy
Although harmless, reactive and benign neoplastic myo-/fibroblastic proliferations represent a diagnostic challenge in routine surgical pathology practice as reflected by their frequency among consultation cases. In addition to resembling each other, the prototypical nodular fasciitis (NF) and NF-like lesions can be mistaken for low-grade or aggressive sarcomas. USP6 translocation was reported recently as the molecular driver and potential diagnostic marker of NF. We reviewed 71 lesions with a diagnosis of NF (n = 48) or NF-like myofibroblastic proliferations (n = 23) and screened them for USP6 translocation by fluorescence in situ hybridization (FISH)...
April 5, 2018: Virchows Archiv: An International Journal of Pathology
https://www.readbyqxmd.com/read/29621612/laparoscopic-anterior-pelvic-exenteration-with-super-radical-parametrectomy-for-a-recurrent-low-grade-endometrial-sarcoma-that-is-resistant-to-hormone-therapy-and-chemotherapy
#10
Hiroyuki Kanao, Tsuyoshi Hisa, Makiko Omi, Minoru Nagashima, Shuhei Okamoto, Yoichi Aoki, Terumi Tanigawa, Maki Matoda, Sanshiro Okamoto, Hidetaka Nomura, Kohei Omatsu, Kazuyoshi Kato, Kuniko Utsugi, Nobuhiro Takeshima
STUDY OBJECTIVE: To show total laparoscopic complete resection of a recurrent low grade endometrial sarcoma. DESIGN: Step-by-step demonstration of the technique of laparoscopic anterior pelvic exenteration with super radical parametrectomy, including the explanation of a detail pelvic anatomy. (Canadian Task Force Classification III) SETTING: Low grade endometrial stromal sarcoma (LGESS) is a rare malignancy that makes around 0.2% of all uterine malignancies [1]...
April 2, 2018: Journal of Minimally Invasive Gynecology
https://www.readbyqxmd.com/read/29619120/preoperative-ultrasonographic-evaluation-for-malignancy-of-soft-tissue-sarcoma-a-retrospective-study
#11
Takeshi Morii, Tomonori Kishino, Naoko Shimamori, Mitsue Motohashi, Hiroaki Ohnishi, Keita Honya, Takayuki Aoyagi, Takashi Tajima, Shoichi Ichimura
Background: Ultrasonography is useful for distinguishing between benign and malignant soft-tissue tumors. However, no study has focused on its usefulness in the differential diagnosis between low-grade and high-grade soft-tissue sarcomas. We conducted a retrospective study to determine the usefulness of the parameters of ultrasonograph and to develop a practical scoring system for distinguishing between high-grade and low-grade sarcomas. Methods: Twenty-two cases of low-grade and 43 cases of high-grade malignant soft-tissue sarcoma were enrolled...
2018: Open Orthopaedics Journal
https://www.readbyqxmd.com/read/29610117/cd163-is-required-for-protumoral-activation-of-macrophages-in-human-and-murine-sarcoma
#12
Daisuke Shiraishi, Yukio Fujiwara, Hasita Horlad, Yoichi Saito, Toyohisa Iriki, Junko Tsuboki, Pan Cheng, Naomi Nakagata, Hiroshi Mizuta, Hirofumi Bekki, Yasuharu Nakashima, Yoshinao Oda, Motohiro Takeya, Yoshihiro Komohara
Recent findings have shown the significance of CD163-positive macrophages in tumor progression, yet there have been few studies on the function of CD163 in macrophages. Here we uncover the role of CD163 in macrophage activation using CD163-deficient mice and human samples. We detected CD163 in 62 undifferentiated pleomorphic sarcoma samples, in which a high percentage of CD163-positive macrophages was associated with decreased overall survival and higher histological grade. We observed macrophage-induced tumor cell proliferation in co-cultures of human monocyte-derived macrophages and leiomyosarcoma (TYLMS-1) and myxofibrosarcoma (NMFH-1) cell lines, which was abrogated by silencing of CD163...
April 2, 2018: Cancer Research
https://www.readbyqxmd.com/read/29606586/larotrectinib-for-paediatric-solid-tumours-harbouring-ntrk-gene-fusions-phase-1-results-from-a-multicentre-open-label-phase-1-2-study
#13
Theodore W Laetsch, Steven G DuBois, Leo Mascarenhas, Brian Turpin, Noah Federman, Catherine M Albert, Ramamoorthy Nagasubramanian, Jessica L Davis, Erin Rudzinski, Angela M Feraco, Brian B Tuch, Kevin T Ebata, Mark Reynolds, Steven Smith, Scott Cruickshank, Michael C Cox, Alberto S Pappo, Douglas S Hawkins
BACKGROUND: Gene fusions involving NTRK1, NTRK2, or NTRK3 (TRK fusions) are found in a broad range of paediatric and adult malignancies. Larotrectinib, a highly selective small-molecule inhibitor of the TRK kinases, had shown activity in preclinical models and in adults with tumours harbouring TRK fusions. This study aimed to assess the safety of larotrectinib in paediatric patients. METHODS: This multicentre, open-label, phase 1/2 study was done at eight sites in the USA and enrolled infants, children, and adolescents aged 1 month to 21 years with locally advanced or metastatic solid tumours or CNS tumours that had relapsed, progressed, or were non-responsive to available therapies regardless of TRK fusion status; had a Karnofsky (≥16 years of age) or Lansky (<16 years of age) performance status score of 50 or more, adequate organ function, and full recovery from the acute toxic effects of all previous anticancer therapy...
March 29, 2018: Lancet Oncology
https://www.readbyqxmd.com/read/29596317/distribution-of-glutathione-stabilized-gold-nanoparticles-in-feline-fibrosarcomas-and-their-role-as-a-drug-delivery-system-for-doxorubicin-preclinical-studies-in-a-murine-model
#14
Katarzyna Zabielska-Koczywąs, Anna Wojtalewicz, Ewelina Użarowska, Agata Klejman, Agata Wojtkowska, Izabella Dolka, Marek Wojnicki, Katarzyna Sobczak, Michał Wójcik, Haifa Shen, Mauro Ferrari, Roman Lechowski
Feline injection site sarcomas (FISS) are malignant skin tumors with high recurrence rates despite the primary treatment of radical surgical resections. Adjunctive radiotherapy or chemotherapy with doxorubicin is mostly ineffective. Cellular and molecular causes of multidrug resistance, specific physio-chemical properties of solid tumors impairing drug transport, and the tumor microenvironment have been indicated for causing standard chemotherapy failure. Gold nanoparticles are promising imaging tools, nanotherapeutics, and drug delivery systems (DDS) for chemotherapeutics, improving drug transport within solid tumors...
March 29, 2018: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/29577178/recurrent-adult-intraabdominal-undifferentiated-high-grade-pleomorphic-sarcoma-infiltrated-the-descending-colon-a-case-report-and-review-of-the-literature
#15
Xu Ziwei, Sun Yueming
No abstract text is available yet for this article.
March 26, 2018: Journal of Gastrointestinal Cancer
https://www.readbyqxmd.com/read/29571698/perfusion-mr-imaging-at-3-tesla-can-it-predict-tumor-grade-and-histologic-necrosis-rate-of-musculoskeletal-sarcoma
#16
P A Gondim Teixeira, A Renaud, S Aubert, M Ben Haj Amor, Y-M Robin, A Cotten, L Ceugnart
PURPOSE: To identify quantitative perfusion parameters that are best associated with tumor grade and tumor necrosis at magnetic resonance (MR) imaging at 3-Tesla. METHODS: MR perfusion studies of 31 patients with a musculoskeletal sarcoma were retrospectively evaluated by two readers. There were 18 men and 13 women with a mean age of 34.9±24.4 (standard deviation [SD] years) (range: 6-87 years). All patients underwent carcinologic tumor resection less than 3 months after MR imaging...
March 20, 2018: Diagnostic and Interventional Imaging
https://www.readbyqxmd.com/read/29562930/telomerase-promoter-mutations-in-human-immunodeficiency-virus-related-conjunctiva-neoplasia
#17
Noemy Starita, Luigi Buonaguro, Franco M Buonaguro, Maria Lina Tornesello
BACKGROUND: Squamous cell carcinoma (SCC) of the conjunctiva is a common cancer in Africa mainly associated with solar ultraviolet (UV) exposure and human immunodeficiency virus (HIV) infection. We analyzed the role of HIV on the occurrence of telomerase reverse transcriptase (TERT) promoter mutations among a cohort of conjunctiva neoplasia Ugandan patients. METHODS: Telomerase reverse transcriptase promoter mutations were searched in 72 conjunctiva neoplasia cases, comprising SCC and intraepithelial neoplasia grade 1-3 (CIN1-3), as well as in 53 conjunctiva normal tissues and in 24 HIV-related Kaposi sarcoma...
March 21, 2018: Journal of Translational Medicine
https://www.readbyqxmd.com/read/29560643/patients-with-primary-localized-high-grade-sarcomas-of-the-digestive-tract-excluding-gist-a-retrospective-study-from-the-french-sarcoma-group
#18
A de Nonneville, C Toullec, J Y Blay, D Ranchere, P E Stoeckle, A Italiano, S Bonvalot, A P Terrier, F Duffaud, F Bertucci, D Cupissol, N Isambert, S Piperno Neumann, J M Coindre, S Salas
INTRODUCTION : The natural history of localized high-grade sarcomas of the digestive tract (SDT) excluding GIST has been rarely considered owing to their low incidence and heterogeneity. We describe the histoclinical characteristics of SDT and correlate them with patients' outcomes. METHODS: We retrospectively collected medical files from a European database covering connective tissue tumors listed in Europe for about twenty years. Only untreated localized primary high-grade SDT were included...
October 2017: Acta Gastro-enterologica Belgica
https://www.readbyqxmd.com/read/29558901/myxoid-liposarcoma-local-relapse-and-metastatic-pattern-in-43-patients
#19
Hans Roland Dürr, Jessica Rauh, Andrea Baur-Melnyk, Thomas Knösel, Lars Lindner, Falk Roeder, Volkmar Jansson, Alexander Klein
BACKGROUND: Liposarcomas are the second most common type of soft tissue sarcomas, 30-50% of these are of myxoid subtype. The aim of this retrospective study was to analyze the local control rate, the metastatic pattern and survival of patients in a consecutive single-institution series. METHODS: From 1983 to 2015, 43 patients with myxoid liposarcoma of the extremities and trunk wall underwent resections. The margin was defined as R0 (wide) or R1 (marginal). Patients were followed for evidence of local recurrence or distant metastasis...
March 20, 2018: BMC Cancer
https://www.readbyqxmd.com/read/29556391/radiation-recall-pneumonitis-induced-by-ifosfamide-for-malignant-soft-tissue-tumor-arising-from-the-chest-wall-a-case-report
#20
Hiroaki Murata, Kenzou Koyama, Yasunobu Takezawa, Yasunori Nishigaki
The present study reports a rare case of radiation recall pneumonitis attributed to ifosfamide that was used as a single antitumor agent for a recurrent malignant soft tissue tumor, arising from the chest wall. A 74-year-old man, who had a mass in his right upper chest wall, underwent surgical excision under the diagnosis of chronic expanding hematoma. He was referred to the present hospital with a recurrent tumor as the excised tumor was diagnosed as undifferentiated high-grade sarcoma. The extension of the tumor following previous surgery was aggressive and the tumor margins were unclear on magnetic resonance imaging...
April 2018: Molecular and Clinical Oncology
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