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Pediatric sarcoma

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https://www.readbyqxmd.com/read/28735378/a-phase-i-ii-trial-and-pharmacokinetic-study-of-mithramycin-in-children-and-adults-with-refractory-ewing-sarcoma-and-ews-fli1-fusion-transcript
#1
Patrick J Grohar, John Glod, Cody J Peer, Tristan M Sissung, Fernanda I Arnaldez, Lauren Long, William D Figg, Patricia Whitcomb, Lee J Helman, Brigitte C Widemann
PURPOSE: In a preclinical drug screen, mithramycin was identified as a potent inhibitor of the Ewing sarcoma EWS-FLI1 transcription factor. We conducted a phase I/II trial to determine the dose-limiting toxicities (DLT), maximum tolerated dose (MTD), and pharmacokinetics (PK) of mithramycin in children with refractory solid tumors, and the activity in children and adults with refractory Ewing sarcoma. PATIENTS AND METHODS: Mithramycin was administered intravenously over 6 h once daily for 7 days for 28 day cycles...
July 22, 2017: Cancer Chemotherapy and Pharmacology
https://www.readbyqxmd.com/read/28724045/spinal-tumors-in-children
#2
Andrei Fernandes Joaquim, Enrico Ghizoni, Marcelo Gomes Cordeiro Valadares, Simone Appenzeller, Simone Dos Santos Aguiar, Helder Tedeschi
Introduction: Spinal tumors are rare in the pediatric population, presenting many specific peculiarities when compared to adults. We have performed a broad narrative review to describe the most common spinal tumors in children, discussing their main characteristics and management options. Method: The authors have performed an extensive review of the peer-reviewed literature addressing the aforementioned objectives. Results: Multimodality radiological studies (plain films, 3D computed tomography scan and magnetic resonance imaging) are necessary for proper evaluation and differential diagnosis of spinal tumors in children...
May 2017: Revista da Associação Médica Brasileira
https://www.readbyqxmd.com/read/28710342/vincristine-ifosfamide-and-doxorubicin-for-initial-treatment-of-ewing-sarcoma-in-adults
#3
Michael J Wagner, Vancheswaran Gopalakrishnan, Vinod Ravi, J Andrew Livingston, Anthony P Conley, Dejka Araujo, Neeta Somaiah, Maria A Zarzour, Ravin Ratan, Wei-Lien Wang, Shreyaskumar R Patel, Alexander Lazar, Joseph A Ludwig, Robert S Benjamin
BACKGROUND: There are no clinical trials specifically addressing chemotherapy for adults with Ewing sarcoma (ES). Five-year event-free survival (EFS) of adults on pediatric studies of ES (44%-47%) is worse than that of children treated with the same therapy (69%). The object of this study was to review the results of therapy with vincristine, ifosfamide, and doxorubicin (VID) in the multidisciplinary treatment of adults with ES at our institution. MATERIALS AND METHODS: Charts for adults treated for ES from 1995 to 2011 were retrospectively reviewed...
July 14, 2017: Oncologist
https://www.readbyqxmd.com/read/28699149/is-the-clavicula-pro-humero-technique-of-value-for-reconstruction-after-resection-of-the-proximal-humerus-in-children
#4
Dominique Barbier, Benoît De Billy, Philippe Gicquel, Sophie Bourelle, Pierre Journeau
BACKGROUND: There are several options for reconstruction of proximal humerus resections after wide resection for malignant tumors in children. The clavicula pro humero technique is a biologic option that has been used in the past, but there are only scant case reports and small series that comment on the results of the procedure. Because the longevity of children mandates a reconstruction with potential longevity not likely to be achieved by other techniques, the clavicula pro humero technique may be a potential option in selected patients...
July 11, 2017: Clinical Orthopaedics and related Research
https://www.readbyqxmd.com/read/28697168/pretreatment-neutrophil-to-lymphocyte-ratio-and-lymphocyte-recovery-independent-prognostic-factors-for-survival-in-pediatric-sarcomas
#5
Liliana Vasquez, Esmeralda León, Brady Beltran, Ivan Maza, Monica Oscanoa, Jenny Geronimo
BACKGROUND: Pretreatment neutrophil-to-lymphocyte ratio (NLR) and absolute lymphocyte count (ALC) recovery have been shown to be associated with prognosis in several types of cancer in adults. However, evidence in pediatric cancer is scarce. The aim of our study was to evaluate whether pretreatment NLR and lymphocyte recovery are prognostic factors in pediatric sarcomas. MATERIALS AND METHODS: Study participants were identified from a retrospective cohort of 100 children with osteosarcoma (n=55), rhabdomyosarcoma (n=22), and Ewing sarcoma (n=23)...
July 10, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28696047/a-randomized-trial-of-the-effectiveness-of-the-neutropenic-diet-versus-food-safety-guidelines-on-infection-rate-in-pediatric-oncology-patients
#6
Karen M Moody, Rebecca A Baker, Ruth O Santizo, Inan Olmez, Jeanie M Spies, Amanda Buthmann, Linda Granowetter, Robin Y Dulman, Kanyalakshmi Ayyanar, Jonathan B Gill, Aaron E Carroll
BACKGROUND: The neutropenic diet (ND) is prescribed to avoid introduction of bacteria into a host's gastrointestinal tract and reduce infection. Due to a lack of evidence to support the ND, there continues to be debate among pediatric oncologists regarding its usefulness. This prospective randomized controlled trial evaluated the difference in neutropenic infection rates in pediatric oncology patients randomized to Food and Drug Administration approved food safety guidelines (FSGs) versus the ND plus FSGs during one cycle of chemotherapy...
July 11, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28696044/practice-patterns-of-palliative-radiation-therapy-in-pediatric-oncology-patients-in-an-international-pediatric-research-consortium
#7
Avani Dholakia Rao, Qinyu Chen, Ralph P Ermoian, Sara R Alcorn, Maria Luisa S Figueiredo, Michael J Chen, Karin Dieckmann, Shannon M MacDonald, Matthew M Ladra, Daria Kobyzeva, Alexey V Nechesnyuk, Kristina Nilsson, Eric C Ford, Brian A Winey, Rosangela C Villar, Stephanie A Terezakis
BACKGROUND/OBJECTIVES: The practice of palliative radiation therapy (RT) is based on extrapolation from adult literature. We evaluated patterns of pediatric palliative RT to describe regimens used to identify opportunity for future pediatric-specific clinical trials. DESIGN/METHODS: Six international institutions with pediatric expertise completed a 122-item survey evaluating patterns of palliative RT for patients ≤21 years old from 2010 to 2015. Two institutions use proton RT...
July 11, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28690908/primary-occipital-ewing-s-sarcoma-with-subsequent-spinal-seeding
#8
Ali Alqahtani, Roaa Amer, Eman Bakhsh
Ewing's sarcoma is a primary bone cancer that mainly affects the long bones. This malignancy is particularly common in pediatric patients. Primary cranial involvement accounts for 1% of cases, with occipital involvement considered extremely rare. In this case study, primary occipital Ewing's sarcoma with a posterior fossa mass and subsequent relapse resulting in spinal seeding is reported. A 3-year-old patient presented with a 1-year history of left-sided headaches, localized over the occipital bone with progressive torticollis...
2017: Case Reports in Pediatrics
https://www.readbyqxmd.com/read/28676226/late-toxicity-and-outcomes-following-radiation-therapy-for-chest-wall-sarcomas-in-pediatric-patients
#9
John T Lucas, Israel Fernandez-Pineda, Christopher L Tinkle, Michael W Bishop, Sue C Kaste, Rajiv Heda, Andrew M Davidoff, Matthew J Krasin
PURPOSE: To investigate the contribution of radiation therapy to acute and late toxicity in pediatric chest wall sarcoma patients and evaluate dosimetric correlates of higher incidence toxicities such as scoliosis and pneumonitis. METHODS AND MATERIALS: The data from 23 consecutively treated pediatric patients with chest wall sarcomas of various histologies (desmoid, Ewing, rhabdomyosarcoma, nonrhabdomyosarcoma-soft tissue sarcomas) were reviewed to evaluate the relationship between end-organ radiation dose, clinical factors, and the risk of subsequent late effects (scoliosis, pneumonitis)...
April 26, 2017: Practical Radiation Oncology
https://www.readbyqxmd.com/read/28660405/xanthogranulomatous-osteomyelitis-of-the-humerus-in-a-pediatric-patient-with-alagille-syndrome-a-case-report-and-literature-review
#10
Adnan Cheema, Alexandre Arkader, Bruce Pawel
Xanthogranulomatous osteomyelitis (XO) is an exceedingly rare disease characterized by infiltration of histiocytes and foamy macrophages. Both on gross examination and on radiographs, XO can mimic malignancy. We describe the case of a 5-year-old female with Alagille syndrome who presented with a pathologic fracture of the right humerus. Initial radiographs revealed multiple osteolytic lesions in the distal humerus while MRI showed a large soft tissue mass. Biopsy confirmed the diagnosis of XO, which has hitherto not been described in a patient with Alagille syndrome...
June 28, 2017: Skeletal Radiology
https://www.readbyqxmd.com/read/28640941/phox2b-reliably-distinguishes-neuroblastoma-among-small-round-blue-cell-tumors
#11
Yin P Hung, John P Lee, Andrew M Bellizzi, Jason L Hornick
AIMS: Neuroblastoma shows considerable histologic overlap with other small round blue cell tumors. PHOX2B, a transcription factor essential for autonomic nervous system development, has been reported as an immunohistochemical marker for neuroblastoma. The purpose of this study was to validate the specificity and diagnostic utility of PHOX2B for peripheral neuroblastic tumors. METHODS AND RESULTS: We evaluated 240 cases (133 in whole-tissue sections; 107 in tissue microarrays), including 76 peripheral neuroblastic tumors [median age 2 years; including 4 adults] and 164 other tumors: 44 Wilms tumors; 20 Ewing sarcomas; 10 each CIC-rearranged round cell sarcomas, poorly differentiated synovial sarcomas, lymphoblastic lymphomas, alveolar rhabdomyosarcomas, embryonal rhabdomyosarcomas, mesenchymal chondrosarcomas, Merkel cell carcinomas, olfactory neuroblastomas, and melanomas; 5 each NUT midline carcinomas and desmoplastic small round cell tumors...
June 22, 2017: Histopathology
https://www.readbyqxmd.com/read/28619077/what-is-the-impact-of-local-control-in-ewing-sarcoma-analysis-of-the-first-brazilian-collaborative-study-group-ewing1
#12
Ricardo G Becker, Lauro J Gregianin, Carlos R Galia, Reynaldo Jesus-Garcia Filho, Eduardo A Toller, Gerardo Badell, Suely A Nakagawa, Alexandre David, André M Baptista, Eduardo S Yonamime, Osvaldo A Serafini, Valter Penna, Julie Francine C Santos, Algemir L Brunetto
BACKGROUND: Relapse in localized Ewing sarcoma patients has been a matter of concern regarding poor prognosis. Therefore, we investigated the impact of local control modality (surgery, surgery plus radiotherapy, and radiotherapy) on clinical outcomes such as survival and recurrence in patients with non-metastatic Ewing sarcoma treated on the first Brazilian Collaborative Group Trial of the Ewing Family of Tumors (EWING1). METHODS: Seventy-three patients with localized Ewing sarcoma of bone aged < 30 years were included...
June 15, 2017: BMC Cancer
https://www.readbyqxmd.com/read/28607872/vascularized-fibula-based-physis-transfer-a-follow-up-study-of-longitudinal-bone-growth-and-complications
#13
Ronnie L Shammas, Yash J Avashia, Alfredo E Farjat, Anthony A Catanzano, L Scott Levin, William C Eward, Brian E Brigman, Detlev Erdmann
BACKGROUND: The vascularized free fibula epiphyseal transfer provides an option for the preservation of limb lengthening after resection of the proximal humerus in pediatric sarcoma patients. The purpose of this study was to provide a long-term follow-up of longitudinal growth patterns and outcomes after free fibula epiphyseal transfer in upper extremity reconstruction. METHODS: A retrospective review of 4 patients who underwent free fibula epiphyseal transfer after oncologic resection of the proximal humerus for osteosarcoma was performed...
May 2017: Plastic and Reconstructive Surgery. Global Open
https://www.readbyqxmd.com/read/28601426/corrigendum-to-risk-stratification-and-pattern-of-cardiotoxicity-in-pediatric-ewing-sarcoma-j-egypt-natl-cancer-instit-29-2017-53-56
#14
Emad Moussa, Manal Zamzam, Ahmed Kamel, Zeinab Salah, Iman Attia, Lina Gaber, Ranin Soliman, Sameera Ezzat
No abstract text is available yet for this article.
June 7, 2017: Journal of the Egyptian National Cancer Institute
https://www.readbyqxmd.com/read/28598585/epithelioid-hemangioendotheliomas-of-the-liver-and-lung-in-children-and-adolescents
#15
REVIEW
Simone Hettmer, Geoffroy Andrieux, Jochen Hochrein, Philipp Kurz, Jochen Rössler, Silke Lassmann, Martin Werner, Nikolas von Bubnoff, Christoph Peters, Ewa Koscielniak, Monika Sparber-Sauer, Charlotte Niemeyer, Thomas Mentzel, Hauke Busch, Melanie Boerries
Epithelioid hemangioendothelioma (EHE) is a rare, vascular sarcoma. Visceral forms arise in the liver/ lungs. We review the clinical and molecular phenotype of pediatric visceral EHE based on the case of a 9-year-old male child with EHE of the liver/lungs. His tumor expressed the EHE-specific fusion oncogene WWTR1-CAMTA1. Molecular characterization revealed a low somatic mutation rate and activated interferon signaling, angiogenesis regulation, and blood vessel remodeling. After polychemotherapy and resection of lung tumors, residual disease remained stable on oral lenalidomide...
June 9, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28597093/-pediatric-salivary-gland-tumors-and-tumor-like-lesions
#16
REVIEW
A Agaimy, H Iro, J Zenk
Salivary gland tumors and tumor-like lesions in the pediatric population are uncommon. They comprise a heterogeneous group of infectious/inflammatory and neoplastic conditions. Pediatric salivary neoplasms include benign tumors of mesenchymal or epithelial origin as well as malignancies of epithelial (carcinomas), mesenchymal (sarcoma) or hematolymphoid (lymphoma) derivation. Infectious/inflammatory conditions and hematolymphoid malignancies may represent either genuine parenchymal pathology or conditions involving intraglandular lymph nodes of the parotid glands (intraglandular lymphadenopathy and intraglandular nodal lymphomas)...
July 2017: Der Pathologe
https://www.readbyqxmd.com/read/28589056/vaginal-ewing-sarcoma-an-uncommon-clinical-entity-in-pediatric-patients
#17
Nathan M Cross, A Luana Stanescu, Erin R Rudzinski, Douglas S Hawkins, Marguerite T Parisi
Ewing sarcoma, including classical Ewing sarcoma of the bone and primitive neuroectodermal tumors arising in bone or extraosseous primary sites, is a highly aggressive childhood neoplasm. We present two cases of Ewing sarcoma arising from the vagina in young girls. Previously reported cases in literature focused on their pathologic rather than radiographic features. We describe the spectrum of multimodality imaging appearances of Ewing sarcoma at this unusual primary site. Awareness of vaginal Ewing tumors may facilitate prompt diagnosis and lead to a different surgical approach than the more commonly encountered vaginal rhabdomyosarcoma...
2017: Journal of Clinical Imaging Science
https://www.readbyqxmd.com/read/28582410/a-phase-i-study-of-single-agent-perifosine-for-recurrent-or-refractory-pediatric-cns-and-solid-tumors
#18
Oren J Becher, Nathan E Millard, Shakeel Modak, Brian H Kushner, Sofia Haque, Ivan Spasojevic, Tanya M Trippett, Stephen W Gilheeney, Yasmin Khakoo, David C Lyden, Kevin C De Braganca, Jill M Kolesar, Jason T Huse, Kim Kramer, Nai-Kong V Cheung, Ira J Dunkel
The PI3K/Akt/mTOR signaling pathway is aberrantly activated in various pediatric tumors. We conducted a phase I study of the Akt inhibitor perifosine in patients with recurrent/refractory pediatric CNS and solid tumors. This was a standard 3+3 open-label dose-escalation study to assess pharmacokinetics, describe toxicities, and identify the MTD for single-agent perifosine. Five dose levels were investigated, ranging from 25 to 125 mg/m2/day for 28 days per cycle. Twenty-three patients (median age 10 years, range 4-18 years) with CNS tumors (DIPG [n = 3], high-grade glioma [n = 5], medulloblastoma [n = 2], ependymoma [n = 3]), neuroblastoma (n = 8), Wilms tumor (n = 1), and Ewing sarcoma (n = 1) were treated...
2017: PloS One
https://www.readbyqxmd.com/read/28571929/trans-oral-robotic-surgery-for-a-ewing-s-sarcoma-of-tongue-in-a-pediatric-patient-a-case-report
#19
Frank Rikki Canevari, Filippo Montevecchi, Stefania Galla, Raffaele Sorrentino, Claudio Vicini, Federico Sireci
No abstract text is available yet for this article.
May 2, 2017: Brazilian Journal of Otorhinolaryngology
https://www.readbyqxmd.com/read/28561686/advances-in-the-treatment-of-pediatric-bone-sarcomas
#20
Patrick J Grohar, Katherine A Janeway, Luke D Mase, Joshua D Schiffman
Bone tumors make up a significant portion of noncentral nervous system solid tumor diagnoses in pediatric oncology patients. Ewing sarcoma and osteosarcoma, both with distinct clinical and pathologic features, are the two most commonly encountered bone cancers in pediatrics. Although mutations in the germline have classically been more associated with osteosarcoma, there is recent evidence germline alterations in patients with Ewing sarcoma also play a significant role in pathogenesis. Treatment advances in this patient population have lagged behind that of other pediatric malignancies, particularly targeted interventions directed at the biologic underpinnings of disease...
2017: American Society of Clinical Oncology Educational Book
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