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Pediatric sarcoma

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https://www.readbyqxmd.com/read/29046803/antitumor-activity-of-yulangsan-polysacchrides-in-mice-bearing-s180-sarcoma-tumors
#1
Wen'E Cai, Xiaoyu Chen, Qingdong Pan, Shijun Zhang, Luojiao Tan, Xuyong Sun, Renbin Huang, Aijun Xia
Sarcoma is one of the most prevalent pediatric tumors and the therapeutic role of chemotherapy has yet to be elucidated. It has been reported that extracts of Longyanshen (Yulangsan) may enhance the sensitivity of drug-resistant cancer cell lines, and improve the immune dysfunction induced by cyclophosphamide (CTX) in mice. The present in vivo study investigated the antitumor effects of Yulangsan polysaccharides (YLSPS) and their interaction with CTX in murine sarcoma 180 (S180)-bearing mice. Immunohistochemistry was used to detect the expression of apoptosis-related proteins...
October 2017: Molecular and Clinical Oncology
https://www.readbyqxmd.com/read/29040902/primary-ewing-s-sarcoma-of-the-kidney-a-case-report
#2
Faris Alasmari, Hani Albadawe, Sultan Alkhateeb, Fahd Alsufiani, Samirah Ghandurah
This is a 15-year-old female who presented with sudden onset left flank pain associated with nausea and vomiting and a history of weight loss. Radiological investigation revealed a large non-obstructive tumor involving the lower pole of the left kidney which was primarily thought to be a renal cell carcinoma. She underwent left open radical nephrectomy with adrenalectomy. Histopathology of the resected specimen showed features of Ewing's sarcoma of the kidney which was confirmed by cytogenetic analysis. This is a rare disease especially in the pediatric group and in reporting such a rare case we hope it helps in identifying a potential course of the disease and its response to the involved treatment...
October 13, 2017: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/29040054/pediatric-nut-midline-carcinoma-therapeutic-success-employing-a-sarcoma-based-multimodal-approach
#3
Simone Storck, Alyssa L Kennedy, Karen J Marcus, Lisa Teot, Jennifer Vaughn, Astrid K Gnekow, Bruno Märkl, Ivo Leuschner, Steven G DuBois, Christopher A French, Michael C Frühwald
A subset of poorly differentiated squamous cell carcinomas, NUT midline carcinomas (NMC) are characterized by a translocation t(15;19)(q13;p13) [ 1 ]. The prognosis is generally dismal [ 2 ] and therapeutic success has been limited to exceptional cases [ 3 ]. We present two cases of pediatric NMC from two different institutions treated according to a multimodal sarcoma approach involving surgery, chemotherapy, and focal radiotherapy. One patient has remained in complete continuous remission for over 6 years, while the other is in CR in early follow-up off therapy...
October 17, 2017: Pediatric Hematology and Oncology
https://www.readbyqxmd.com/read/29039470/effects-of-baicalein-on-proliferation-apoptosis-migration-and-invasion-of-ewing-s-sarcoma-cells
#4
Conglin Ye, Xiaolong Yu, Jin Zeng, Min Dai, Bin Zhang
Ewing's sarcoma (ES) is a rare tumor that is more frequent in pediatric and adolescent age groups. In the past few decades, long-term survival in affected patients has improved due to the success of multimodal therapy. However, long-term survival is inevitably restricted by the late side-effects of chemotherapy. Besides, early metastasis also contributes to the poor prognosis of ES. Recently, traditional Chinese medicines (TCMs) have increasingly attracted interest due to the promising clinical results and fewer side-effects for the treatment of cancers...
October 10, 2017: International Journal of Oncology
https://www.readbyqxmd.com/read/29029988/mandibular-embryonal-rhabdomyosarcoma-with-cartilaginous-metaplasia-report-of-a-case-and-review-of-literature
#5
Scott M Peters, Tim Kunkle, Michael A Perrino, Elizabeth M Philipone, Angela J Yoon
Rhabdomyosarcoma (RMS) is a malignant tumor of skeletal muscle origin and frequently involves the head and neck region. It represents the second most common pediatric soft tissue sarcoma and accounts for 3% of all childhood cancers. Here, we report a case of embryonal RMS presenting as a right-sided facial swelling in a 7-year-old boy. Histologically, the tumor consisted of classic embryonal rhabdosarcomatous areas with metaplastic cartilage, in both initial biopsy and final resection specimens. Cartilaginous metaplasia arising in the background of RMS is a rare finding, thus raising a diagnostic challenge...
September 6, 2017: Oral Surgery, Oral Medicine, Oral Pathology and Oral Radiology
https://www.readbyqxmd.com/read/29029499/generation-and-characterization-of-erbb2-car-engineered-cytokine-induced-killer-cells-for-the-treatment-of-high-risk-soft-tissue-sarcoma-in-children
#6
Michael Merker, Verena Pfirrmann, Sarah Oelsner, Simone Fulda, Thomas Klingebiel, Winfried S Wels, Peter Bader, Eva Rettinger
Pediatric patients with recurrent, refractory or advanced soft tissue sarcoma (STS) who are simultaneously showing signs of cumulative treatment toxicity are in need of novel therapies. In this preclinical analysis, we identified ErbB2 as a targetable antigen on STS cells and used cytokine-induced killer (CIK) cells transduced with the lentiviral 2(nd)-generation chimeric antigen receptor (CAR) vector pS-5.28.z-IEW to target ErbB2-positive tumors. Solely CIK cell subsets with the CD3(+) T cell phenotype showed up to 85% cell surface expression of the respective CAR...
September 12, 2017: Oncotarget
https://www.readbyqxmd.com/read/29016412/false-lung-metastasis-concurrent-ewing-sarcoma-and-minimally-invasive-adenocarcinoma
#7
Allison S Bechtel, Bradley Cheek, Scott Bradfield
Pediatric lung cancer is a very rare occurrence, particularly as a primary lesion. A concurrent diagnosis is even more unusual and only reported a handful of times in Ewing sarcoma. Our patient is a 13-year-old boy who had concurrent diagnoses of Ewing sarcoma and minimally invasive adenocarcinoma of the lung, formerly bronchoalveolar carcinoma. To our knowledge this has also been found in at least 1 other case. There are some classic genetic mutations associated with Ewing sarcoma. None have been found to be linked with the concurrent diagnosis...
October 9, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28992614/serum-and-glucocorticoid-inducible-kinase-1-sensitive-survival-proliferation-and-migration-of-rhabdomyosarcoma-cells
#8
Evi Schmid, Matias Julian Stagno, Jing Yan, Sabine Schleicher, Willi Yu, Sabina Honisch, Florian Lang, Jörg Fuchs, Guido Seitz
BACKGROUND/AIMS: Rhabdomyosarcoma, the most common pediatric soft tissue sarcoma, may show an intrinsic refractoriness to standard chemotherapy in advanced tumor stages, which is associated with poor prognosis. Cellular mechanisms conferring tumor cell survival and therapy resistance in many tumor types include the serum & glucocorticoid inducible kinase (SGK) 1 pathway, a kinase expressed ubiquitously with particularly strong expression in skeletal muscle and some tumor types. The present study explored whether SGK1 is expressed in rhabdomyosarcoma and, if so, whether this kinase impacts on tumor cell survival, proliferation and migration...
October 9, 2017: Cellular Physiology and Biochemistry
https://www.readbyqxmd.com/read/28992567/conditional-survival-of-pediatric-adolescent-and-young-adult-soft-tissue-sarcoma-and-bone-tumor-patients
#9
Judy Y Ou, Holly Spraker-Perlman, Andrew C Dietz, Rochelle R Smits-Seemann, Sapna Kaul, Anne C Kirchhoff
BACKGROUND: Survival estimates for soft tissue sarcomas (STS) and malignant bone tumors (BT) diagnosed in pediatric, adolescent, and young adult patients are not easily available. We present survival estimates based on a patient having survived a defined period of time (conditional survival). Conditional survival estimates for the short-term were calculated for patients from diagnosis to the first five years after diagnosis and for patients surviving in the long-term (up to 20 years after diagnosis)...
October 6, 2017: Cancer Epidemiology
https://www.readbyqxmd.com/read/28983376/radiation-induced-malignancies-our-experiences-with-five-cases
#10
Gunjesh Kumar Singh, Vikas Yadav, Pragya Singh, K T Bhowmik
Radiotherapy is one of the modalities of treatment of malignancies. Radiation-induced malignancies (RIMs) are late complications of radiotherapy, seen among the survivors of both adult and pediatric cancers. Mutagenesis of normal tissues is the likely basis for RIMs. Till date, RIM cannot be differentiated from primary cancers. We present a series of five patients who were treated at our institute between 2002 and 2016 and were subsequently diagnosed with RIM. Out of five patients, there were two cases of sarcomas, two of carcinomas and one neuroendocrine carcinoma of tongue (rare entity)...
December 2016: World Journal of Oncology
https://www.readbyqxmd.com/read/28965982/vaginoscopic-resection-for-vaginal-rhabdomyosarcoma-during-early-infancy-a-case-report
#11
Karolina Pańczak, Dawid Gawron, Patrycja Sosnowska, Karina Kapczuk, Przemysław Mańkowski
Embryonal rhabdomyosarcoma, a malignant soft-tissue neoplasm, is one of the most common pediatric tumors of the vagina. Although radical surgery has been replaced with radiation therapy, chemotherapy, and conservative surgery, the use of vaginoscopy is still uncommon. We present the case of an infant who underwent vaginoscopic resection owing to botryoid sarcoma at 9 months of age.
September 28, 2017: Journal of Minimally Invasive Gynecology
https://www.readbyqxmd.com/read/28951009/a-retrospective-cohort-study-to-assess-adjuvant-concurrent-chemoradiation-ccrt-compared-to-adjuvant-radiation-therapy-rt-in-the-treatment-of-grade-2-and-3-extremity-soft-tissue-sarcomas
#12
Jean Philippe Nesseler, Julia Salleron, Maria Rios, Philippe Nickers, Frederic Marchal, Fabien Brocard, Didier Peiffert, Guillaume Vogin
PURPOSE: To evaluate the efficacy and tolerance of adjuvant concurrent chemoradiation (CCRT) as treatment of grade 2 and 3 (G2-3) localized extremity soft tissue sarcomas (STS) by comparing CCRT with standard adjuvant radiation therapy (RT). PATIENTS AND METHODS: This monocentric retrospective study included non-pediatric patients (>16years) treated by adjuvant RT with or without chemotherapy (CT) after conservative resection of non-recurrent G2-3 extremity STS...
September 23, 2017: Radiotherapy and Oncology: Journal of the European Society for Therapeutic Radiology and Oncology
https://www.readbyqxmd.com/read/28945250/smarcb1-is-required-for-widespread-baf-complex-mediated-activation-of-enhancers-and-bivalent-promoters
#13
Robert T Nakayama, John L Pulice, Alfredo M Valencia, Matthew J McBride, Zachary M McKenzie, Mark A Gillespie, Wai Lim Ku, Mingxiang Teng, Kairong Cui, Robert T Williams, Seth H Cassel, He Qing, Christian J Widmer, George D Demetri, Rafael A Irizarry, Keji Zhao, Jeffrey A Ranish, Cigall Kadoch
Perturbations to mammalian SWI/SNF (mSWI/SNF or BAF) complexes contribute to more than 20% of human cancers, with driving roles first identified in malignant rhabdoid tumor, an aggressive pediatric cancer characterized by biallelic inactivation of the core BAF complex subunit SMARCB1 (BAF47). However, the mechanism by which this alteration contributes to tumorigenesis remains poorly understood. We find that BAF47 loss destabilizes BAF complexes on chromatin, absent significant changes in complex assembly or integrity...
September 25, 2017: Nature Genetics
https://www.readbyqxmd.com/read/28926683/salvage-rates-and-prognostic-factors-after-relapse-in-children-and-adolescents-with-malignant-peripheral-nerve-sheath-tumors
#14
Luca Bergamaschi, Gianni Bisogno, Carla Manzitti, Paolo D'Angelo, Giuseppe Maria Milano, Angela Scagnellato, Mirko Cappelletti, Stefano Chiaravalli, Patrizia Dall'Igna, Rita Alaggio, Antonio Ruggiero, Martina Di Martino, Maria Carmen Affinita, Marta Pierobon, Alberto Garaventa, Michela Casanova, Andrea Ferrari
BACKGROUND: Malignant peripheral nerve sheath tumor (MPNST) is one of the most common nonrhabdomyosarcoma soft tissue sarcomas encountered in pediatric age, and it is generally characterized by poor outcome, particularly for relapsing patients. MATERIALS AND METHODS: This study considered 73 patients <21 years of age with relapsing MPNST observed among 120 patients enrolled in Italian pediatric protocols from 1979 to 2004. With the aim of possibly establishing a risk-adapted stratification, patients' outcome was examined using univariate and multivariate analysis based on clinical features at onset, first-line treatments, clinical findings at the time of first relapse, and second-line treatments...
September 19, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28905489/consensus-and-controversies-regarding-the-treatment-of-rhabdomyosarcoma
#15
REVIEW
Scott C Borinstein, Diana Steppan, Masanori Hayashi, David M Loeb, Michael S Isakoff, Odion Binitie, Andrew S Brohl, Julia A Bridge, Mark Stavas, Eric T Shinohara, William H Meyer, Damon R Reed, Lars M Wagner
Optimal treatment of rhabdomyosarcoma (RMS) requires multidisciplinary approach, incorporating chemotherapy with local control. Although current therapies are built on cooperative group trials, a comprehensive standard of care to guide clinical decision making has been lacking, especially for relapsed patients. Therefore, we assembled a panel of pediatric and adolescent and young adult sarcoma experts to develop treatment guidelines for managing RMS and to identify areas in which further research is needed...
September 14, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28903844/bone-marrow-involvement-in-metastatic-pediatric-ewing-sarcoma
#16
Saadiya Javed Khan, Irfana Ishaq, Haleema Saeed, Muhammad Bilal Fayyaz, Syed Ali Shazif Baqari, Rabia Mohammad Wali
OBJECTIVE: To evaluate the frequency of bone marrow involvement with metastatic lung and bone sites in newly-diagnosed pediatric patients with Ewing sarcoma (ES). STUDY DESIGN: An observational study. PLACE AND DURATION OF STUDY: Shaukat Khanum Memorial Cancer Hospital, Lahore, Pakistan, from January 2010 to October 2015. METHODOLOGY: Newly-diagnosed pediatric-age patients with ES were inducted. Ten patients were excluded because bone marrow aspiration/biopsy (BMAB) was not done...
August 2017: Journal of the College of Physicians and Surgeons—Pakistan: JCPSP
https://www.readbyqxmd.com/read/28899971/matrix-screen-identifies-synergistic-combination-of-parp-inhibitors-and-nicotinamide-phosphoribosyltransferase-nampt-inhibitors-in-ewing-sarcoma
#17
Christine M Heske, Mindy I Davis, Joshua T Baumgart, Kelli M Wilson, Michael V Gormally, Lu Chen, Xiaohu Zhang, Michele Ceribelli, Damien Duveau, Rajarshi Guha, Marc Ferrer, Fernanda I Arnaldez, Jiuping Jay Ji, Huong-Lan Tran, Yiping Zhang, Arnulfo Mendoza, Lee J Helman, Craig J Thomas
PURPOSE: While many cancers are showing remarkable responses to targeted therapies, pediatric sarcomas, including Ewing sarcoma, remain recalcitrant. To broaden the therapeutic landscape, we explored the in vitro response of Ewing sarcoma cell lines against a large collection of investigational and approved drugs to identify candidate combinations. EXPERIMENTAL DESIGN: Drugs displaying activity as single agents were evaluated in combinatorial (matrix) format to identify highly active, synergistic drug combinations, and combinations were subsequently validated in multiple cell lines using various agents from each class...
September 12, 2017: Clinical Cancer Research: An Official Journal of the American Association for Cancer Research
https://www.readbyqxmd.com/read/28892044/a-novel-mouse-model-of-rhabdomyosarcoma-underscores-the-dichotomy-of-mdm2-alt1-function-in-vivo
#18
D F Comiskey, A G Jacob, B L Sanford, M Montes, A K Goodwin, H Steiner, E Matsa, A S Tapia-Santos, T W Bebee, J Grieves, K La Perle, P Boyaka, D S Chandler
Alternative splicing of the oncogene murine double minute 2 (MDM2) is induced in response to genotoxic stress. MDM2-ALT1, the major splice variant generated, is known to activate the p53 pathway and impede full-length MDM2's negative regulation of p53. Despite this perceptible tumor-suppressive role, MDM2-ALT1 is also associated with several cancers. Furthermore, expression of MDM2-ALT1 has been observed in aggressive metastatic disease in pediatric rhabdomyosarcoma (RMS), irrespective of histological subtype...
September 11, 2017: Oncogene
https://www.readbyqxmd.com/read/28883017/nf%C3%AE%C2%BAb-signaling-in-alveolar-rhabdomyosarcoma
#19
Megan M Cleary, Atiya Mansoor, Teagan Settelmeyer, Yuichi Ijiri, Katherine J Ladner, Matthew N Svalina, Brian P Rubin, Denis C Guttridge, Charles Keller
Alveolar rhabdomyosarcoma (aRMS) is a pediatric soft tissue cancer commonly associated with a chromosomal translocation that leads to the expression of a Pax3:Foxo1 or Pax7:Foxo1 fusion protein, the developmental underpinnings of which may give clues to its therapeutic approaches. In aRMS, the NFκB-YY1-miR-29 regulatory circuit is dysregulated, resulting in repression of miR-29 and loss of the associated tumor suppressor activity. To further elucidate the role of NFκB in aRMS, we first tested 55 unique sarcoma cell lines and primary cell cultures in a large-scale chemical screen targeting diverse molecular pathways...
September 1, 2017: Disease Models & Mechanisms
https://www.readbyqxmd.com/read/28877062/recurrent-braf-gene-fusions-in-a-subset-of-pediatric-spindle-cell-sarcomas-expanding-the-genetic-spectrum-of-tumors-with-overlapping-features-with-infantile-fibrosarcoma
#20
Yu-Chien Kao, Christopher D M Fletcher, Rita Alaggio, Leonard Wexler, Lei Zhang, Yun-Shao Sung, Dicle Orhan, Wei-Chin Chang, David Swanson, Brendan C Dickson, Cristina R Antonescu
Infantile fibrosarcomas (IFS) represent a distinct group of soft tissue tumors occurring in patients under 2 years of age and most commonly involving the extremities. Most IFS show recurrent ETV6-NTRK3 gene fusions, sensitivity to chemotherapy, and an overall favorable clinical outcome. However, outside these well-defined pathologic features, no studies have investigated IFS lacking ETV6-NTRK3 fusions, or tumors with the morphology resembling IFS in older children. This study was triggered by the identification of a novel SEPT7-BRAF fusion in an unclassified retroperitoneal spindle cell sarcoma in a 16-year-old female by targeted RNA sequencing...
September 4, 2017: American Journal of Surgical Pathology
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