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Pediatric bone tumor

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https://www.readbyqxmd.com/read/27907820/preauricular-pilomatricoma-an-uncommon-entity-in-a-dental-pediatric-patient
#1
Poyzan Bozkurt, Mehmet Eray Kolsuz, Ömer Günhan, Erdal Erdem, Kaan Orhan
INTRODUCTION: Pilomatricomas are benign follicular skin appendage tumors, commonly occurring in children and young adults. Most patients admit to dermatologists to seek treatment and are well known by them; however, dental professionals, especially pediatric dentists are not familiar with these tumors. PRESENTATION OF CASE: This report presents a 16-year-old female with preauricular pilomatricoma, located beneath the overlying skin of the temporomandibular region...
November 15, 2016: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/27898606/facial-nerve-meningioma-a-cause-of-pediatric-facial-weakness
#2
Nicholas L Deep, Sharon H Gnagi, David F Carpentieri, P David Adelson, Peter A Weisskopf
OBJECTIVE: To present an unusual case of a temporal bone meningioma with intrafascicular spread throughout the temporal facial nerve from cerebellopontine angle (CPA) to stylomastoid foramen. PATIENT: Four-year-old female with progressive facial weakness and normal hearing. MAIN OUTCOME MEASURE: Clinical, radiological, and histopathological findings of temporal bone meningiomas. RESULTS: A patient presented with progressive facial weakness and normal hearing...
November 24, 2016: Otology & Neurotology
https://www.readbyqxmd.com/read/27875302/proteasomal-degradation-of-the-ews-fli1-fusion-protein-is-regulated-by-a-single-lysine-residue
#3
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...
November 8, 2016: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/27864829/pediatric-case-of-anaplastic-lymphoma-kinase-positive-anaplastic-large-cell-lymphoma-forming-a-solitary-skin-tumor-on-the-forearm
#4
Michio Tokuyama, Yuta Kurashige, Tami Ota, Yasuaki Manabe, Hanako Yamaoka, Norihiro Ikoma, Akiko Fukumura, Michie Miyashita, Keisuke Otsubo, Tsuyoshi Morimoto, Hiroyuki Mochizuki, Natsuko Nakano, Naoya Nakamura, Akira Ozawa, Tomotaka Mabuchi
A 5-year-old girl noticed a rapidly growing reddish nodule on her right forearm. Although oral antibiotics had been administrated for 2 weeks, the tumor enlarged. Skin biopsy revealed excessive infiltration of atypical neoplastic cells expressing CD4, CD30 and anaplastic lymphoma kinase (ALK). These histological and immunohistochemical findings were consistent with anaplastic large cell lymphoma (ALCL). Computed tomography showed multiple lymphadenopathy, but lymph node biopsy and bone marrow examination did not show any evidence of systemic dissemination...
November 18, 2016: Journal of Dermatology
https://www.readbyqxmd.com/read/27863409/integrating-mechanisms-of-response-and-resistance-against-the-tubulin-binding-agent-eribulin-in-preclinical-models-of-osteosarcoma
#5
Valerie B Sampson, Nancy S Vetter, Wendong Zhang, Pratima U Patil, Robert W Mason, Erika George, Richard Gorlick, E A Kolb
Osteosarcoma is the most frequently occurring bone cancer in children and adolescents. Unfortunately, treatment failures are common. Eribulin is a synthetic microtubule inhibitor that has demonstrated activity in preclinical osteosarcoma models. The effects of eribulin were evaluated in two human osteosarcoma cell lines as well as in eribulin-sensitive and -resistant osteosarcoma xenograft tumors of the Pediatric Preclinical Testing Program (PPTP) by characterizing cell viability, microtubule destabilization, mitotic arrest and mechanism of cell death...
November 15, 2016: Oncotarget
https://www.readbyqxmd.com/read/27860482/long-noncoding-rna-ewsat1-promotes-osteosarcoma-cell-growth-and-metastasis-through-suppression-of-meg3-expression
#6
Lishan Sun, Cheng Yang, Juan Xu, Yanhong Feng, Liguo Wang, Tao Cui
Osteosarcoma (OS) is the most common primary bone tumor in children and adolescents. Long noncoding RNAs (lncRNAs) are a class of transcriptional products of the genome without protein-coding potential. Recently, lncRNA Ewing sarcoma-associated transcript 1 (EWSAT1) was functionally identified in Ewing sarcoma, a highly aggressive primary pediatric bone tumor. However, whether EWSAT1 plays a role in OS remains unclear. In the present study, gain- and loss-of-function assays demonstrated that EWSAT1 enhanced OS cell proliferation, migration, and invasion...
December 2016: DNA and Cell Biology
https://www.readbyqxmd.com/read/27860191/perinatal-factors-associated-with-clinical-presentation-of-osteosarcoma-in-children-and-adolescents
#7
Alyson A Endicott, Libby M Morimoto, Cassie N Kline, Joseph L Wiemels, Catherine Metayer, Kyle M Walsh
BACKGROUND: Osteosarcoma typically develops during puberty with tumors arising at sites of rapid bone growth, suggesting a role for growth-regulating pathways in tumor etiology. Birthweight is one measure of perinatal growth that has been investigated as an osteosarcoma risk factor. Whether birthweight affects clinical features of osteosarcoma remains unexplored. METHOD: Six hundred seventy patients with osteosarcoma, aged 0-19 years, were identified through the California Cancer Registry...
November 10, 2016: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/27812743/glomerular-hyperfiltration-in-children-with-cancer-prevalence-and-a-hypothesis
#8
Neha S Kwatra, Holly J Meany, Sunil J Ghelani, David Zahavi, Nayan Pandya, Massoud Majd
BACKGROUND: Glomerular hyperfiltration has recently been reported in children with malignancies and has been attributed to increased solute from breakdown of tumor tissues. OBJECTIVE: To evaluate the prevalence of hyperfiltration in the pediatric oncology population and explore its pathophysiological mechanism. MATERIALS AND METHODS: Tc-99 m diethylenetriaminepentaacetic acid (DTPA) glomerular filtration rate (GFR) examinations (437 studies) and medical records of 177 patients <21 years of age diagnosed with a malignancy between January 2005 and October 2013 were retrospectively reviewed...
November 3, 2016: Pediatric Radiology
https://www.readbyqxmd.com/read/27795776/-interest-of-imaging-in-tumors-benign-bone-in-children
#9
Ousmane Traoré, Kamilia Chban, Alzavine Fleur Hode, Yaya Diarra, Siham Salam, Lachen Ouzidane
Benign bone tumors are more common than malignant tumors in pediatrics. The exostosis (ostéchondrome) is the most common. The different imaging techniques are pivotal in the study of tumors including its standard radiography. The aim of this work is to highlight the interest in imaging the diagnostic management of bone benign tumors in children through a retrospective study of 169 patients. All patients were investigated by plain radiography, scanner supplement with multiplanar reconstruction before and after injection of PDC and / or MRI 1...
2016: Pan African Medical Journal
https://www.readbyqxmd.com/read/27792997/cd99-polymorphisms-significantly-influence-the-probability-to-develop-ewing-sarcoma-in-earlier-age-and-patient-disease-progression
#10
Marcella Martinelli, Alessandro Parra, Luca Scapoli, Paola De Sanctis, Valentina Chiadini, Claudia Hattinger, Piero Picci, Cinzia Zucchini, Katia Scotlandi
Ewing sarcoma (EWS), the second most common primary bone tumor in pediatric age, is known for its paucity of recurrent somatic abnormalities. Apart from the chimeric oncoprotein that derives from the fusion of EWS and FLI genes, recent genome-wide association studies have identified susceptibility variants near the EGR2 gene that regulate DNA binding of EWS-FLI. However, to induce transformation, EWS-FLI requires the presence of additional molecular events, including the expression of CD99, a cell surface molecule with critical relevance for the pathogenesis of EWS...
October 25, 2016: Oncotarget
https://www.readbyqxmd.com/read/27782927/patterns-of-diagnosis-and-misdiagnosis-in-pediatric-cancer-and-relationship-to-survival
#11
Jing Chen, Craig A Mullen
BACKGROUND: Pediatric cancer is rare and its symptoms are often ambiguous. The aims of this study were to investigate the time needed to make a diagnosis, assess the frequency of misdiagnosis, and to determine whether these factors affected survival. METHODS: A review of records of 364 pediatric patients diagnosed with cancer at the University of Rochester Golisano Children's Hospital between 2004 and 2012 was conducted. Data were extracted on patient and health care system-related factors and clinical outcomes...
October 25, 2016: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/27778155/current-management-of-aneurysmal-bone-cysts
#12
REVIEW
Howard Y Park, Sara K Yang, William L Sheppard, Vishal Hegde, Stephen D Zoller, Scott D Nelson, Noah Federman, Nicholas M Bernthal
Aneurysmal bone cysts (ABCs) are benign bone lesions arising predominantly in the pediatric population that can cause local pain, swelling, and pathologic fracture. Primary lesions, which constitute roughly two thirds of all ABCs, are thought to be neoplastic in nature, with one third of ABCs arising secondary to other tumors. Diagnosis is made with various imaging modalities, which exhibit characteristic features such as "fluid-fluid levels," although biopsy is critical, as telangiectatic osteosarcoma cannot be excluded based on imaging alone...
December 2016: Current Reviews in Musculoskeletal Medicine
https://www.readbyqxmd.com/read/27761737/middle-ear-adenoma-with-neuroendocrine-differentiation-a%C3%A2-pediatric-case-report
#13
Elisabeth Sterrer, Franz Windisch, Kurt Frey, Klaus Rettensteiner, Benjamin Loader
BACKGROUND: Middle ear adenoma with neuroendocrine differentiation (MEA-ND), also known as carcinoid tumor of the middle ear, is an extremely rare neoplasm, especially when located within the tympanic cavity. To the best of our knowledge, this represents the first pediatric case of a MEA-ND described in Europe. Excluding the present case, only three other pediatric cases of this disease have been published globally. CASE REPORT: We report on a 15-year-old female patient who presented with conductive hearing loss of the right ear...
October 19, 2016: Wiener Klinische Wochenschrift
https://www.readbyqxmd.com/read/27756397/primary-alveolar-rhabdomyosarcoma-of-the-bone-two-cases-and-review-of-the-literature
#14
Petra Balogh, Rita Bánusz, Monika Csóka, Zsófia Váradi, Edit Varga, Zoltán Sápi
BACKGROUND: Rhabdomyosarcoma (RMS) is a malignant tumor of mesenchymal origin and comprises the largest category of soft-tissue sarcomas both in children and adolescents. From a pediatric oncology point of view, RMS has traditionally been classified into alveolar (ARMS) and embryonal (ERMS) subtypes. The anatomical localization of the tumor may vary, but commonly involve the head/neck regions, male and female urogenital tract or the trunk and extremities. CASE PRESENTATION: Here, we report two challenging cases involving 17- and 9-years-olds males where diffuse and multiplex bone lesions suggested either a hematological disease or a primary bone tumor (mesenchymal chondrosarcoma)...
October 18, 2016: Diagnostic Pathology
https://www.readbyqxmd.com/read/27729085/chordoma-dedifferentiation-after-proton-beam-therapy-a-case-report-and-review-of-the-literature
#15
Joseph Frankl, Cassi Grotepas, Baldassare Stea, G Michael Lemole, Alexander Chiu, Rihan Khan
BACKGROUND: Chordoma is a rare invasive bone tumor that may occur anywhere along the neuraxis. A total of three primary histological varieties have been identified: conventional, chondroid, and dedifferentiated. CASE PRESENTATION: We report a case of an 8-year-old white girl who presented with conventional chordoma, was treated with surgical resection and mixed proton and photon beam therapy, and had a recurrence in the resection cavity 2.5 years later with dedifferentiated morphology...
October 12, 2016: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/27705929/a-5-day-cytoreductive-chemotherapy-followed-by-haplo-identical-hsct-fa5-bucy-as-a-tumor-ablative-regimen-improved-the-survival-of-patients-with-advanced-hematological-malignancies
#16
Ting Yang, Qiaoxian Lin, Jinhua Ren, Ping Chen, Xiaohong Yuan, Xiaofeng Luo, Tingbo Liu, Jing Zheng, Zhihong Zheng, Xiaoyun Zheng, Xinji Chen, Langhui Zhang, Hao Zheng, Zaisheng Chen, Xueling Hua, Shaohua Le, Jian Li, Zhizhe Chen, Jianda Hu
Haplo-HSCT has been used when HLA-matched siblings are not available. Conditioning regimens aim to reduce tumor burden prior to HSCT and provide sufficient immunoablation. We report the outcome of haplo-HSCT in 63 consecutive patients from 2/2013 to 12/2015 (19 females/44 males) with high-risk or relapsed/refractory hematological malignancies (n=29-AML; 8-sAML; 19-ALL; 5-advanced-MDS; 2-CML-BC). Median age was 20 years (range: 1.1-49). Twenty-one patients achieved remission prior to transplant, while 42 did not...
October 1, 2016: Oncotarget
https://www.readbyqxmd.com/read/27684080/acquired-upper-extremity-growth-arrest
#17
Erich M Gauger, Lauren L Casnovsky, Erica J Gauger, Deborah C Bohn, Ann E Van Heest
This study reviewed the clinical history and management of acquired growth arrest in the upper extremity in pediatric patients. The records of all patients presenting from 1996 to 2012 with radiographically proven acquired growth arrest were reviewed. Records were examined to determine the etiology and site of growth arrest, management, and complications. Patients with tumors or hereditary etiology were excluded. A total of 44 patients (24 boys and 20 girls) with 51 physeal arrests who presented at a mean age of 10...
September 29, 2016: Orthopedics
https://www.readbyqxmd.com/read/27668432/clinical-characteristics-of-benign-pediatric-cranial-vault-tumors-surgical-considerations-based-on-100-cases
#18
Tymon Skadorwa, Bogdan Ciszek
BACKGROUND/AIMS: Tumors of the cranial vault occur at every age of childhood. Although they are mostly benign lesions, their symptomatology is variable and requires extended diagnostics. The choice of therapeutic strategy strongly depends on histopathological diagnosis, and therefore surgical excision is the elective treatment in such cases. Despite several published papers, the literature still lacks reliable clinical characteristics regarding this heterogeneous group of lesions in pediatric patients...
2017: Pediatric Neurosurgery
https://www.readbyqxmd.com/read/27651036/novel-insights-and-therapeutic-interventions-for-pediatric-osteosarcoma
#19
Leo Kager, Gevorg Tamamyan, Stefan Bielack
High-grade osteosarcomas are the most common primary malignant tumors of bone. With complete surgical resection and multi-agent chemotherapy up to 70% of patients with high-grade osteosarcomas and localized extremity tumors can become long-term survivors. The prognosis, however, is poor for patients with nonresectable, primary metastatic or relapsed disease. Outcome is essentially unchanged for three decades. Herein, we describe selected novel insights into the genomics, biology and immunology of the disease and discuss selected strategies, which hold promise to overcome the current stagnation in the therapeutic success in childhood osteosarcoma...
September 21, 2016: Future Oncology
https://www.readbyqxmd.com/read/27638000/osteoarticular-allograft-reconstruction-of-post-traumatic-defect-of-distal-femur-in-a-pediatric-patient-a-case-report-and-literature-review
#20
Amrut Borade, Harish Kempegowda, Meagan Fernandez, Daniel S Horwitz
Successful results of osteoarticular allografts in reconstruction of periarticular bone defect after tumor resection encouraged its utilization in post-traumatic defects. Here we describe a case of post-traumatic skeletal defect in a 4 year-old girl treated with osteoarticular allograft reconstruction. Due to severity of the associated soft tissue injury and contamination at presentation staged treatment with antibiotic spacer followed by the reconstruction was carried out. At the end of one year the patient achieved 'Musculoskeletal tumor society' functional score of 27 points and radiographic score of 93%...
September 6, 2016: Injury
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