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Clear cell sarcoma

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https://www.readbyqxmd.com/read/28969561/pharmaceutical-production-of-anti-tumor-and-immune-potentiating-enterococcus-faecalis-2001-%C3%AE-glucans-enhanced-activity-of-macrophage-and-lymphocytes-in-tumor-implanted-mice
#1
Yeun-Hwa Gu, Hyunju Choi, Takenori Yamashita, Ki-Mun Kang, Masahiro Iwasa, Moon-Jo Lee, Kyoung Hae Lee, Cheorl-Ho Kim
BACKGROUND: Enterococcus faecalis 2001 is a probiotic lactic acid bacterium and has been used as a biological response modifier (BRM). From physiological limitation of bacterial preservation in storage and safety, the live E. faecalis 2001 has been heat-treated and the BRM components containing high level of β-glucan, named EF-2001, has been prepared. METHOD: The heat-treated EF-2001 has been examined for the antioxidative potential for radical scavenging and anti-tumor activities as well as immune-enhancing response in mice...
October 2, 2017: Current Pharmaceutical Biotechnology
https://www.readbyqxmd.com/read/28950372/activity-and-safety-of-crizotinib-in-patients-with-advanced-clear-cell-sarcoma-with-met-alterations-european-organization-for-research-and-treatment-of-cancer-phase-2-trial-90101-create
#2
P Schöffski, A Wozniak, P G Casali, 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
Background: Clear cell sarcoma (CCSA) is an orphan malignancy, characterised by a specific t(12;22) translocation, leading to rearrangement of the EWSR1 gene and overexpression of MET. We prospectively investigated the efficacy and safety of the tyrosine kinase inhibitor (TKI) crizotinib in patients with advanced or metastatic CCSA. Patients and methods: Patients with CCSA received oral crizotinib 250 mg twice daily. Primary endpoint was objective response rate (ORR), secondary endpoints included duration of response, disease control rate (DCR), progression-free survival (PFS), progression-free rate (PFR), overall survival (OS), overall survival rate (OSR) and safety...
September 18, 2017: Annals of Oncology: Official Journal of the European Society for Medical Oncology
https://www.readbyqxmd.com/read/28942160/gold-nanoconjugates-reinforce-the-potency-of-conjugated-cisplatin-and-doxorubicin
#3
Sana Iram, Manaal Zahera, Salman Khan, Imran Khan, Asad Syed, Abu Ayoobul Ansary, Fuad Ameen, Omar H M Shair, Mohd Sajid Khan
Osteosarcoma or osteogenic sarcoma is the most common and prevalent cancerous tumor of bone and occurs especially in children and teens. Recent treatment strategy includes a combination of both chemotherapy and surgeries. Although, the use of single drug-based chemotherapy treatment remains unsatisfactory. Therefore, combinatorial therapy has emerged as a potential strategy for treatment with limited side- effects. Here, we evaluated the combinatorial anticancerous effect of cisplatin (CIS) and doxorubicin (DOX) bioconjugated bromelain encapsulated gold nanoparticles (B-AuNPs conjugated CIS and DOX) in the treatment of osteosarcoma...
September 7, 2017: Colloids and Surfaces. B, Biointerfaces
https://www.readbyqxmd.com/read/28927521/when-the-guardian-sleeps-reactivation-of-the-p53-pathway-in-cancer
#4
REVIEW
Olaf Merkel, Ninon Taylor, Nicole Prutsch, Philipp B Staber, Richard Moriggl, Suzanne D Turner, Lukas Kenner
The p53 tumor suppressor is inactivated in most cancers, thus suggesting that loss of p53 is a prerequisite for tumor growth. Therefore, its reintroduction through different means bears great clinical potential. After a brief introduction to current knowledge of p53 and its regulation by the ubiquitin-ligases MDM2/MDMX and post-translational modifications, we will discuss small molecules that are able to reactivate specific, frequently observed mutant forms of p53 and their applicability for clinical purposes...
July 2017: Mutation Research
https://www.readbyqxmd.com/read/28905842/malignant-perivascular-epithelioid-cell-tumor-of-the-orbit-report-of-a-case-and-review-of-literature
#5
Md Shahid Alam, Bipasha Mukherjee, S Krishnakumar, Jyotirmay Biswas
Perivascular epithelioid cell tumor (PEComa) is a rare neoplasm considered to arise from myomelanocytic cell lineage. The uterus is reportedly the most common site to be involved. Orbital PEComa is extremely rare with only two cases reported till date. A 5-year-old male presented with a right medial orbital mass for the last 6 months. The patient was diagnosed with alveolar soft part sarcoma elsewhere. Magnetic resonance imaging features were suggestive of lymphangioma with bleeding. The excision biopsy revealed multiple tumor cells comprising epithelioid cells with clear cytoplasm, along with nuclear atypia and mitosis...
September 2017: Indian Journal of Ophthalmology
https://www.readbyqxmd.com/read/28892972/renal-synovial-sarcoma-in-a-young-pregnant-lady-a-case-report-and-clinico-pathological-profile
#6
Gregory Pathrose, Nirmal Thampi John, Pradeep Hariharan
Synovial sarcoma is a soft tissue neoplasm with clearly defined histologic, immunohistochemical and molecular features. These tumours usually arise in the extremities of young adults. Their occurrence in the kidney is extremely rare. A 25-year-old pregnant lady in her first trimester was incidentally found to have a left renal mass on perinatal ultrasonography. MRI showed a well encapsulated, heterointense mass replacing the left kidney. Following medical termination of her pregnancy, a radical nephrectomy was performed...
July 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28852958/clinical-benefit-of-antiangiogenic-therapy-in-advanced-and-metastatic-chondrosarcoma
#7
Robin L Jones, Daniela Katz, Elizabeth T Loggers, Darin Davidson, Eve T Rodler, Seth M Pollack
Chondrosarcoma is the most common bone sarcoma in adults. Conventional chondrosarcoma, the commonest histological subtype, is largely resistant to anthracycline-based chemotherapy. There have been anecdotal reports of durable clinical benefit with antiangiogenic agents in this disease. A retrospective search of patients treated at three sarcoma referral centers was performed to identify patients with advanced chondrosarcoma treated with antiangiogenic agents. The aim of this study was to evaluate the efficacy and safety of antiangiogenic agents in advanced chondrosarcoma...
August 29, 2017: Medical Oncology
https://www.readbyqxmd.com/read/28833375/clear-cell-sarcomas-of-kidney-are-characterized-by-bcor-gene-abnormalities-including-exon-15-internal-tandem-duplications-and-bcor-ccnb3-gene-fusion
#8
Meng Kang Wong, Cedric Chuan Young Ng, Chik Hong Kuick, Sze Jet Aw, Vikneswari Rajasegaran, Jing Quan Lim, Jain Sudhanshi, Eva Loh, Minzhi Yin, Jing Ma, Zhongde Zhang, Prasad Iyer, Amos Hong Pheng Loh, Derrick Wen Quan Lian, Shi Wang, Shaun Giap Hean Goh, Tse Hui Lim, Alvin Soon Tiong Lim, Tony Ng, Angela Goytain, Alwin Hwai Liang Loh, Puay Hoon Tan, Bin Tean Teh, Kenneth Tou En Chang
AIMS: Clear cell sarcoma of kidney (CCSK) is a rare paediatric renal malignant tumour. The majority of CCSKs have internal tandem duplications (ITD) of the BCOR gene while a minority has the YWHAE-NUTM2 gene fusion. A third 'double-negative' (DN) category comprises CCSKs with neither BCOR ITD nor YWHAE-NUTM2 fusion. The aim of this study was to characterize 11 histologically diagnosed CCSKs immunohistochemically (with CCND1, BCOR and CCNB3 stains), and genetically. METHODS AND RESULTS: By next-generation sequencing, 10 cases (90...
August 18, 2017: Histopathology
https://www.readbyqxmd.com/read/28832074/programmed-death-ligand-1-pd-l1-expression-in-malignant-mesenchymal-tumors
#9
Kemal Kösemehmetoğlu, Ece Özoğul, Berrin Babaoğlu, Gaye Güler Tezel, Gökhan Gedikoğlu
OBJECTIVE: Programmed death ligand 1 (PD-L1) found on tumor cells has recently been reported to have a key role in the development and dissemination of many tumors, such as lung and breast carcinomas. In this study, we retrospectively analyzed PD-L1 expression among different types of sarcomas. MATERIAL AND METHOD: Tissue microarrays of 3-4 mm diameter were composed from paraffin blocks of 222 various sarcomas. Slides prepared from microarrays were stained for PD-L1 antibody (Cell Signaling, E1L3N®) using Leica Bond Autostainer...
2017: Türk Patoloji Dergisi
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
#10
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/28816272/-radical-nephrectomy-and-thrombectomy-for-mayo-clinic-stage-iii-tumor-thrombus-a-surgical-technique-and-clinical-experience
#11
Z Liu, L L Ma, X J Tian, G L Wang, X F Hou, S D Zhang, S H Deng
OBJECTIVE: To evaluate the effectiveness and safety of radical nephrectomy and inferior vena cava thrombectomy in the treatment of patients with Mayo III tumor thrombus, and to introduce our experience and surgical technique. METHODS: The clinical data of 8 patients with Mayo III tumor thrombus from October 2014 to September 2016 were analyzed retrospectively. Of the 8 patients, 3 were male and 5 were female. The average age was (50.8±18.7) years (18 to 77 years)...
August 18, 2017: Beijing da Xue Xue Bao. Yi Xue Ban, Journal of Peking University. Health Sciences
https://www.readbyqxmd.com/read/28802505/soft-tissue-tumors-of-uncertain-histogenesis-a-review-for-dermatopathologists
#12
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/28763028/rous-sarcoma-virus-rna-stability-element-inhibits-deadenylation-of-mrnas-with-long-3-utrs
#13
Vidya Balagopal, Karen L Beemon
All retroviruses use their full-length primary transcript as the major mRNA for Group-specific antigen (Gag) capsid proteins. This results in a long 3' untranslated region (UTR) downstream of the termination codon. In the case of Rous sarcoma virus (RSV), there is a 7 kb 3'UTR downstream of the gag terminator, containing the pol, env, and src genes. mRNAs containing long 3'UTRs, like those with premature termination codons, are frequently recognized by the cellular nonsense-mediated mRNA decay (NMD) machinery and targeted for degradation...
August 1, 2017: Viruses
https://www.readbyqxmd.com/read/28753253/clear-cell-atypical-fibroxanthoma-an-unusual-case-in-an-unusual-place
#14
Zachary Goldstein, George Sarantopoulos, Chandra Smart
Atypical fibroxanthoma represents a low-grade sarcoma that usually presents in the elderly population on sun-damaged areas of the head and neck regions. This neoplasm is characterized on histology by atypical pleomorphic, epithelioid to spindled cells, arranged in a haphazard pattern, set within a background of solar elastosis. In this case report, we present a unique case of the rare variant of clear-cell atypical fibroxanthoma arising in an unusual place, specifically the lower extremities.
July 28, 2017: Journal of Cutaneous Pathology
https://www.readbyqxmd.com/read/28745431/trabectedin-is-a-promising-antitumor-agent-potentially-inducing-melanocytic-differentiation-for-clear-cell-sarcoma
#15
Takaaki Nakai, Yoshinori Imura, Hironari Tamiya, Shutaro Yamada, Sho Nakai, Naohiro Yasuda, Keiko Kaneko, Hidetatsu Outani, Satoshi Takenaka, Kenichiro Hamada, Akira Myoui, Nobuhito Araki, Takafumi Ueda, Kazuyuki Itoh, Hideki Yoshikawa, Norifumi Naka
Clear cell sarcoma is an aggressive soft tissue sarcoma and highly resistant to conventional chemotherapy and radiation therapy. This devastating disease is defined by EWSR1-ATF1 fusion gene resulting from chromosomal translocation t(12;22)(q13;q12) and characterized by melanocytic differentiation. A marine-derived antineoplastic agent, trabectedin, inhibits the growth of myxoid liposarcoma and Ewing sarcoma by causing adipogenic differentiation and neural differentiation, respectively. In this study, we examined the antitumor effects and mechanism of action of trabectedin on human clear cell sarcoma cell lines...
September 2017: Cancer Medicine
https://www.readbyqxmd.com/read/28728220/-diagnostic-utility-of-tyrosine-hydroxylase-in-peripheral-neuroblastic-tumors
#16
N Zhang, N Liu, L J He
Objective: To investigation the diagnostic utility of tyrosine hydroxylase (TH) immunohistochemically as a marker of peripheral neuroblastic tumors(pNT). Methods: The study included 1 024 cases, 643 primary and metastatic pNT cases, 381 non-pNT cases, including small round cell tumors such as primitive neuroectodermal tumor (PNET), rhabdomyosarcoma, lymphoma, nephroblastoma, as well as other more common tumors (medulloblastoma, hepatoblastoma, pleuropulmonary blastoma, renal clear cell sarcoma, Langerhans cell histiocytosis, lipoblatoma etc)...
July 8, 2017: Zhonghua Bing Li Xue za Zhi Chinese Journal of Pathology
https://www.readbyqxmd.com/read/28725344/complete-response-of-mediastinal-clear-cell-sarcoma-to-pembrolizumab-with-radiotherapy
#17
Samuel Marcrom, Jennifer F De Los Santos, Robert M Conry
BACKGROUND: Clear cell sarcoma (CCS) is a rare, aggressive soft tissue sarcoma thought to derive from neural crest and characterized by a 12;22 translocation. The resulting fusion protein directly activates expression of the melanocyte master transcription factor and drives the same down-stream pathways in CCS and melanoma leading to significant clinical parallels between these malignancies. Striking success of immune checkpoint blockade in melanoma has promoted interest in immunotherapy of CCS...
2017: Clinical Sarcoma Research
https://www.readbyqxmd.com/read/28704208/hgnet-bcor-tumors-of-the-cerebellum-clinicopathologic-and-molecular-characterization-of-3-cases
#18
Romain Appay, Nicolas Macagno, Laetitia Padovani, Andrey Korshunov, Marcel Kool, Nicolas André, Didier Scavarda, Torsten Pietsch, Dominique Figarella-Branger
The central nervous system (CNS) high-grade neuroepithelial tumor with BCOR alteration (CNS HGNET-BCOR) is a recently described molecular entity. We report 3 new CNS HGNET-BCOR cases sharing common clinical presentation and pathologic features. The 3 cases concerned children aged 3 to 7 years who presented with a voluminous mass of the cerebellum. Pathologic features included proliferation of uniform spindle to ovoid cells with fine chromatin associated with a rich arborizing capillary network. Methylation profiling classified these cases as CNS HGNET-BCOR tumors...
September 2017: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28698435/-histology-specific-chemotherapy-in-soft-tissue-sarcomas
#19
Eisuke Kobayashi, Akira Kawai
Soft-tissue sarcomas(STSs)are rare mesenchymal tumors, accounting for less than 1%of all adult malignancies. STSs also have diversity, with more than 50 different histological subtypes. While surgical complete resection is a definitive treatment for localized STS, chemotherapy is the treatment option for managing locally advanced and metastatic STS. Although doxorubicin ±ifosfamide is still the first-line therapy for most STS subtypes, some STSs(alveolar soft part sarcoma, clear cell sarcoma, epithelioid sarcoma and extraskeletal myxoid chondrosarcoma)have been reported to have little response to these cytotoxic chemotherapies...
June 2017: Gan to Kagaku Ryoho. Cancer & Chemotherapy
https://www.readbyqxmd.com/read/28697683/metastatic-sclerosing-epithelioid-fibrosarcoma-in-bone-marrow
#20
Rana Asakra, Shane Zaidi, Khin Thway
Sclerosing epithelioid fibrosarcoma (SEF) is an aggressive neoplasm thought to be related to low-grade fibromyxoid sarcoma, which typically occurs in middle-aged adults in the deep soft tissues of the lower extremities and trunk. It comprises nests and cords of relatively uniform epithelioid polygonal cells with clear or eosinophilic cytoplasm in densely sclerotic stroma, and it is typically associated with EWSR1 gene rearrangements, and most commonly EWSR1-CREB3L1 fusions. As primary SEF can arise in bone, and bone is also a common metastatic site for SEF, its recognition at this site is important...
July 1, 2017: International Journal of Surgical Pathology
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