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Pediatric sarcoma

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https://www.readbyqxmd.com/read/28069802/genomic-profiling-of-a-large-set-of-diverse-pediatric-cancers-identifies-known-and-novel-mutations-across-tumor-spectra
#1
Juliann Chmielecki, Mark Bailey, Jie He, Julia Elvin, Jo-Anne Vergilio, Shakti Ramkissoon, James Suh, Garrett M Frampton, James X Sun, Samantha Morley, Daniel Spritz, Siraj Ali, Laurie Gay, Rachel L Erlich, Jeffrey S Ross, Joana Buxhaku, Hilary Davies, Vinny Faso, Alexis Germain, Blair Glanville, Vincent A Miller, Philip J Stephens, Katherine A Janeway, John M Maris, Soheil Meshinchi, Trevor J Pugh, Jack F Shern, Doron Lipson
Pediatric cancers are generally characterized by low mutational burden and few recurrently mutated genes. Recent studies suggest that genomic alterations may help guide treatment decisions and clinical trial selection. Here, we describe genomic profiles from 1,215 pediatric tumors representing sarcomas, extracranial embryonal tumors, brain tumors, hematologic malignancies, carcinomas, and gonadal tumors. Comparable published datasets identified similar frequencies of clinically relevant alterations, validating this dataset as biologically relevant...
January 9, 2017: Cancer Research
https://www.readbyqxmd.com/read/28068876/tissue-engineered-model-of-human-osteolytic-bone-tumor
#2
Aranzazu Villasante, Alessandro Marturano, Samuel T Robinson, Zen Liu, X Edward Guo, Gordana Vunjak-Novakovic
Ewing's sarcoma (ES) is a poorly differentiated pediatric tumor of aggressive behavior characterized by propensity to metastasize to bone. Interactions between the tumor and bone cells orchestrate a vicious cycle in which tumor cells induce osteoclast differentiation and activation to cause osteolytic lesions, broken bones, pain and hypercalcemia. The lack of controllable models that can recapitulate osteolysis in ES impedes the development of new therapies and limits our understanding of how tumor cells invade bone...
January 9, 2017: Tissue Engineering. Part C, Methods
https://www.readbyqxmd.com/read/28062706/combinatorial-drug-screening-identifies-ewing-sarcoma-specific-sensitivities
#3
Branka Radic-Sarikas, Kalliopi P Tsafou, Kristina B Emdal, Theodore Papamarkou, Kilian V M Huber, Cornelia Mutz, Jeffrey A Toretsky, Keiryn L Bennett, Jesper V Olsen, Søren Brunak, Heinrich Kovar, Giulio Superti-Furga
Improvements in survival for Ewing sarcoma pediatric and adolescent patients have been modest over the past 20 years. Combinations of anticancer agents endure as an option to overcome resistance to single treatments caused by compensatory pathways. Moreover, combinations are thought to lessen any associated adverse side effects through reduced dosing, which is particularly important in childhood tumors. Using a parallel phenotypic combinatorial screening approach of cells derived from three pediatric tumor types, we identified Ewing sarcoma-specific interactions of a diverse set of targeted agents including approved drugs...
January 2017: Molecular Cancer Therapeutics
https://www.readbyqxmd.com/read/28062084/a-t-4-19-pediatric-undifferentiated-sarcoma-with-a-novel-variant-of-the-cic-dux4-fusion-transcript
#4
Lenka Krskova, Eva Stejskalova, Edita Kabickova, Marcela Mrhalova, Roman Kodet
We report cytogenetic and molecular genetic analysis of a pediatric tumor positive for the CIC-DUX4 fusion. The tumor belongs to a rare, diagnostically challenging subgroup of undifferentiated small round cell sarcomas. A balanced t(4;19)(q35;q13.1-2) was identified by G-banding, as a sole cytogenetic finding. The translocation was also identified by the M-FISH technique. After RT-PCR, the tumor sample was positive for the CIC-DUX4 fusion. The PCR product contains a novel, so far unreported variant of the CIC-DUX4 fusion transcript, with a fusion of the exon 20 from the CIC gene and the exon 1 from the DUX4 gene...
December 16, 2016: Pathology, Research and Practice
https://www.readbyqxmd.com/read/28058544/breast-malignancies-in-children-presentation-management-and-survival
#5
Morgan K Richards, Adam B Goldin, Elizabeth A Beierle, John J Doski, Melanie Goldfarb, Monica Langer, Jed G Nuchtern, Sanjeev Vasudevan, Kenneth W Gow, Sara H Javid
PURPOSE: Pediatric breast malignancies are rare, and descriptions in the literature are limited. The purpose of our study was to compare pediatric and adult breast malignancy. METHODS: We performed a retrospective cohort study using the National Cancer Data Base comparing patients ≤21 years to those >21 years at diagnosis (1998-2012). Generalized linear models estimated differences in demographic, tumor, and treatment characteristics. Cox regression was used to compare overall survival...
January 5, 2017: Annals of Surgical Oncology
https://www.readbyqxmd.com/read/28056288/-clinical-application-of-125-i-radioactive-seeds-brachytherapy-in-the-treatment-of-the-pediatric-soft-tissue-sarcoma-in-head-and-neck
#6
D Zhao, L Zheng, X M Lü, M W Huang, Y Shi, X L Ma, J Yan, J G Zhang
Objective: To investigate the clinical application and preliminary results of (125)I radioactive seeds brachytherapy in the comprehensive treatment of the pediatric soft tissue sarcoma in head and neck. Methods: A total of 24 pediatric patients with soft tissue sarcoma in head and neck were treated at Peking University School of Stomatology from April 2012 to July 2015. The data was collected and analyzed through statistical methods, which included the pathological type, gender, age, tumor location, volume, treatment and the clinical results after the application of (125)I radioactive seeds brachytherapy...
January 3, 2017: Zhonghua Yi Xue za Zhi [Chinese medical journal]
https://www.readbyqxmd.com/read/28044936/nano-delivery-in-pediatric-tumors-looking-back-moving-forward
#7
Marta Colletti, Virginia Di Paolo, Angela Galardi, Giuseppe Maria Milano, Angela Mastronuzzi, Franco Locatelli, Angela Di Giannatale
Recent advances in the treatment of pediatric tumors led to an improvement of survival in this population. As a result, many pediatric survivors experience long-term effects that impact their quality of life. Therefore, it is extremely important to identify new treatment approaches that may target the tumor minimizing the drug-related side effects. Over the past 10 years, remarkable advances in nanomedicine have provided several potential tools for cancer treatment. Recently, there has been a growing interest towards therapeutic nanocarriers in the pediatric field, since they represent a new strategy to enhance the drug efficacy and reduce the toxicity...
January 2, 2017: Anti-cancer Agents in Medicinal Chemistry
https://www.readbyqxmd.com/read/28004263/transoral-robotic-surgery-for-the-pediatric-head-and-neck-surgeries
#8
Evren Erkul, Umamaheswar Duvvuri, Deepak Mehta, Utku Aydil
Pediatric robotic surgery is a relatively new technology that has been shown to be safe and feasible for a number of pediatric procedures. Our literature analysis was performed using Pubmed database between January 2005 and December 2015, using key words: "robotic," "robotic surgery," "TORS," "pediatric," "children," "head and neck," and "da Vinci". We selected only publications in English. Eight published reports met the selection criteria. We totally found 41 patients, and the age range was between 2 months and 19 years...
December 21, 2016: European Archives of Oto-rhino-laryngology
https://www.readbyqxmd.com/read/28001289/use-of-precision-medicine-molecular-profiling-of-baseline-tumor-specimen-may-not-benefit-outcomes-in-children-with-relapsed-refractory-or-rare-pediatric-sarcomas
#9
Jacquelyn Carter, Lijun Cheng, Jacob Zucker, Mark Marshall, Karen Pollok, Mary Murray, Lang Li, Jamie Renbarger
Due to the poor prognosis of pediatric patients with relapsed or refractory sarcomas, discovery and implementation of innovative approaches and tools to guide therapy is an urgent need.(1-3) This retrospective pilot study evaluated the impact of relapse and refractory therapies aligned with molecular characterization of biopsies collected at the time of primary diagnosis. This article is protected by copyright. All rights reserved.
December 21, 2016: Clinical Pharmacology and Therapeutics
https://www.readbyqxmd.com/read/27977030/myeloid-sarcoma-in-the-orbit
#10
Xiaoxiao Qian, James W Gigantelli, Minnie Abromowitch, Linda A Morgan, Donny W Suh
The authors describe a case of myeloid sarcoma of the orbit in a pediatric patient. An 8-month-old male infant presented to the ophthalmology clinic with a left orbital mass, which had been increasing in size over the previous 2 months. The mass was initially diagnosed at another clinic as an infantile hemangioma, and had been treated with a topical formulation of timolol. In the ophthalmology clinic, orbital magnetic resonance imaging showed a solid enhancing mass. A biopsy was performed, and histopathology revealed myeloid sarcoma...
December 8, 2016: Journal of Pediatric Ophthalmology and Strabismus
https://www.readbyqxmd.com/read/27955731/non-rhabdomyosarcoma
#11
Roshni Dasgupta, David Rodeberg
Pediatric non-rhabdomyosarcoma soft tissue sarcomas (NRSTS) are a heterogeneous group encompassing more than 50 different histological diagnoses arising from primitive mesenchymal tissue. Together, they comprise about half the soft tissue sarcomas diagnosed in children and young adults. Despite each histologies relative rarity, their management schema is similar among the different NRSTS histologies. Surgical management is an important component of the multimodal treatment strategy of all these tumors. Resection with negative margins, while maintaining function, plays an important role as a primary treatment of these patients as well as diminishing the risks of local and distant recurrence...
October 2016: Seminars in Pediatric Surgery
https://www.readbyqxmd.com/read/27939125/radiologic-response-assessment-in-pediatric-soft-tissue-sarcoma-computed-assisted-volume-evaluation
#12
Giovanna Orsatti, Valeria Beltrame, Filippo Crimì, Anna Chiara Frigo, Gianni Bisogno, Roberto Stramare
OBJECTIVES: To compare 3 methods of dimensional assessment, with particular attention to a new software assisted method of volume calculation, in soft tissue sarcoma, and to investigate the interobserver agreement and the intermethod agreement in chemotherapy response classification and resultant clinical repercussions. STUDY DESIGN: We studied 34 pediatric patients with nonmetastatic soft tissue sarcoma who had undergone only diagnostic biopsy. Tumor size was measured both at diagnosis and after induction chemotherapy by 3 observers and using 3 measurement methods: maximum axis (1 diameter), estimated volume (3 diameters), and computed volume (software-assisted volume calculation)...
December 8, 2016: Journal of Pediatrics
https://www.readbyqxmd.com/read/27922973/incidence-and-outcomes-of-dermatofibrosarcoma-protuberans-in-the-us-pediatric-population
#13
Gustavo A Rubio, Andrea Alvarado, David J Gerth, Jun Tashiro, Seth R Thaller
BACKGROUND: Dermatofibrosarcoma protuberans (DFSP) is a low-grade soft tissue sarcoma. In the pediatric population, DFSP is exceedingly rare. Aim of this study was to describe the epidemiology and clinical outcomes in a large pediatric cohort. METHODS: Surveillance, Epidemiology, and End Results (SEER) database (1973-2010) was analyzed for all patients with dermatofibrosarcoma occurring in patients <20 years of age. Data were extracted based on age, gender, race, anatomic site, histology, stage, treatment modalities, and survival...
December 5, 2016: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/27914587/outcomes-of-chest-wall-resections-in-pediatric-sarcoma-patients
#14
Carmen Lopez, Arlene Correa, Ara Vaporciyan, Mary Austin, David Rice, Andrea Hayes-Jordan
PURPOSE: Chest wall tumors in pediatric patients are rare. This study evaluates outcomes in pediatric patients who have undergone chest wall resections secondary to sarcomas. METHODS: A retrospective review was performed for patients <19years old who underwent chest wall resections for sarcoma 1999-2014 at the University of Texas MD Anderson Cancer Center. RESULTS: Of 44 patients, Ewing's sarcoma (n=18) and osteosarcoma (n=16) were most common...
October 28, 2016: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/27900036/management-of-pediatric-head-and-neck-rhabdomyosarcoma-a-case-series-of-36-patients
#15
Joanna Radzikowska, Wojciech Kukwa, Andrzej Kukwa, Anna M Czarnecka, Maciej Kawecki, Fei Lian, Cezary Szczylik, Antoni Krzeski
Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in the pediatric population. In 35% of cases, RMS develops in the head and neck (H&N) region, and only combined therapy is recognized as a curative treatment. However, recent advances in skull base and reconstructive surgery, along with microsurgery and endoscopic surgery, have strengthened the role of surgery as an important part of RMS treatment. In the present study, 36 pediatric RMS cases (24 males and 12 females) were analyzed after surgical treatment...
November 2016: Oncology Letters
https://www.readbyqxmd.com/read/27896933/feasibility-and-clinical-integration-of-molecular-profiling-for-target-identification-in-pediatric-solid-tumors
#16
Thomas Pincez, Nathalie Clément, Eve Lapouble, Gaëlle Pierron, Maud Kamal, Ivan Bieche, Virginie Bernard, Paul Fréneaux, Jean Michon, Daniel Orbach, Isabelle Aerts, Hélène Pacquement, Franck Bourdeaut, Irene Jiménez, Estelle Thébaud, Caroline Oudot, Cécile Vérité, Sophie Taque, Cormac Owens, François Doz, Christophe Le Tourneau, Olivier Delattre, Gudrun Schleiermacher
BACKGROUND: The role of tumor molecular profiling in directing targeted therapy utilization remains to be defined for pediatric tumors. We aimed to evaluate the feasibility of a sequencing and molecular biology tumor board (MBB) program, and its clinical impact on children with solid tumors. PROCEDURE: We report on a single-center MBB experience of 60 pediatric patients with a poor prognosis or relapsed/refractory solid tumors screened between October 2014 and November 2015...
November 29, 2016: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/27882658/access-to-clinical-trials-for-adolescents-with-soft-tissue-sarcomas-enrollment-in-european-pediatric-soft-tissue-sarcoma-study-group-epssg-protocols
#17
Andrea Ferrari, Annalisa Trama, Angela De Paoli, Christophe Bergeron, Johannes H M Merks, Meriel Jenney, Daniel Orbach, Julia C Chisholm, Soledad Gallego, Heidi Glosli, Gian Luca De Salvo, Laura Botta, Gemma Gatta, Gianni Bisogno
BACKGROUND: Adolescents with cancer are enrolled in clinical trials at far lower rates than children. This report compares the number of adolescents (15-19-year-olds) and children (0-14-year-olds) enrolled in the protocols of the European pediatric Soft tissue sarcoma Study Group (EpSSG) with the number of cases expected to occur. METHODS: The observed-to-expected (O/E) ratio was detected in the EpSSG countries contributing most of the cases, that is, Italy, France, Spain, the Netherlands, United Kingdom, and Ireland...
November 24, 2016: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/27879517/dux4-immunohistochemistry-is-a-highly-sensitive-and-specific-marker-for-cic-dux4-fusion-positive-round-cell-tumor
#18
Bradford Siegele, Jon Roberts, Jennifer O Black, Erin Rudzinski, Sara O Vargas, Csaba Galambos
The histologic differential diagnosis of pediatric and adult round cell tumors is vast and includes the recently recognized entity CIC-DUX4 fusion-positive round cell tumor. The diagnosis of CIC-DUX4 tumor can be suggested by light microscopic and immunohistochemical features, but currently, definitive diagnosis requires ancillary genetic testing such as conventional karyotyping, fluorescence in situ hybridization, or molecular methods. We sought to determine whether DUX4 expression would serve as a fusion-specific immunohistochemical marker distinguishing CIC-DUX4 tumor from potential histologic mimics...
November 22, 2016: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/27875302/proteasomal-degradation-of-the-ews-fli1-fusion-protein-is-regulated-by-a-single-lysine-residue
#19
Maria E Gierisch, Franziska Pfistner, Laura A Lopez-Garcia, Lena Harder, Beat W Schäfer, Felix K Niggli
E-26 transformation-specific (ETS) proteins are transcription factors directing gene expression through their conserved DNA binding domain. They are implicated as truncated forms or interchromosomal rearrangements in a variety of tumors including Ewing sarcoma, a pediatric tumor of the bone. Tumor cells express the chimeric oncoprotein EWS-FLI1 from a specific t(22;11)(q24;12) translocation. EWS-FLI1 harbors a strong transactivation domain from EWSR1 and the DNA-binding ETS domain of FLI1 in the C-terminal part of the protein...
December 23, 2016: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/27870705/clinical-study-on-female-genital-tract-rhabdomyosarcoma-in-childhood-changes-during-20-years-in-one-center
#20
Jie Yang, Jiaxin Yang, Mei Yu, Zhen Yuan, Dongyan Cao, Shen Keng
OBJECTIVE: Rhabdomyosarcoma (RMS) is a rare tumor in girls. We intend to analyze the clinical feature, therapeutic effect, and prognosis of pediatric and adolescent RMS of the female genitourinary tract. METHODS: Pediatric or adolescent patients with female genital tract RMS in 1 tertiary hospital from 1995 to 2015 were retrospectively reviewed. The medical records were collected, including general information, tumor primary site, stage, histology, treatments, adverse effects, and survival outcomes...
November 17, 2016: International Journal of Gynecological Cancer
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