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Pediatric sarcoma

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https://www.readbyqxmd.com/read/28323337/treatment-pathway-of-bone-sarcoma-in-children-adolescents-and-young-adults
#1
REVIEW
Damon R Reed, Masanori Hayashi, Lars Wagner, Odion Binitie, Diana A Steppan, Andrew S Brohl, Eric T Shinohara, Julia A Bridge, David M Loeb, Scott C Borinstein, Michael S Isakoff
When pediatric, adolescent, and young adult patients present with a bone sarcoma, treatment decisions, especially after relapse, are complex and require a multidisciplinary approach. This review presents scenarios commonly encountered in the therapy of bone sarcomas with the goal of objectively presenting a consensus, multidisciplinary management approach. Little variation was found in the authors' group with respect to local control or systemic therapy. Clinical trials were universally prioritized in all settings...
March 21, 2017: Cancer
https://www.readbyqxmd.com/read/28319067/the-histone-demethylase-kdm3a-and-its-downstream-target-mcam-promote-ewing-sarcoma-cell-migration-and-metastasis
#2
M Sechler, J K Parrish, D K Birks, P Jedlicka
Ewing Sarcoma is the second most common solid pediatric malignant neoplasm of bone and soft tissue. Driven by EWS/Ets, or rarely variant, oncogenic fusions, Ewing Sarcoma is a biologically and clinically aggressive disease with a high propensity for metastasis. However, the mechanisms underpinning Ewing Sarcoma metastasis are currently not well understood. In the present study, we identify and characterize a novel metastasis-promotional pathway in Ewing Sarcoma, involving the histone demethylase KDM3A, previously identified by our laboratory as a new cancer-promoting gene in this disease...
March 20, 2017: Oncogene
https://www.readbyqxmd.com/read/28294349/malignant-glomus-tumors-of-the-head-and-neck-in-children-and-adults-evaluation-and-management
#3
Nikolaus E Wolter, Eelam Adil, Alexandria L Irace, Annette Werger, Antonio R Perez-Atayde, Christopher Weldon, Darren B Orbach, Carlos Rodriguez-Galindo, Reza Rahbar
OBJECTIVES/HYPOTHESIS: To describe our current multidisciplinary approach to pediatric malignant glomus tumors of the head and neck and review the current literature. STUDY DESIGN: Retrospective chart review at a tertiary referral children's hospital and a comprehensive literature review. METHODS: A comprehensive literature search of PubMed, Embase, Web of Science, Google Scholar, and EBSCO with respect to malignant glomus tumors of the head and neck was conducted...
March 14, 2017: Laryngoscope
https://www.readbyqxmd.com/read/28293503/near-complete-humerus-reconstruction-in-the-pediatric-patient-with-vascularized-free-fibula-transfer
#4
John Shuck, Benjamin C Wood, Christopher Zarella, Albert K Oh, Robert M Henshaw, Gary F Rogers
Vascularized free fibula transfer remains the gold standard for reconstruction of large segmental diaphyseal defects of the upper extremity. In the pediatric patient, before skeletal maturity, free fibula transfer with the fibular head provides an active physis for growth and an articular interface for glenohumeral joint reconstruction. Clinical and cadaveric studies have demonstrated that the vascular supply to the fibular head originates, in most cases, from the anterior tibial system. However, anatomical variation exists, and we report a case in which a vascularized fibula autograft including the physis was transferred on the peroneal artery in a 5-year-old patient with Ewing's sarcoma...
December 2016: Plastic and Reconstructive Surgery. Global Open
https://www.readbyqxmd.com/read/28289810/non-odontogenic-tumors-of-the-facial-bones-in-children-and-adolescents-role-of-multiparametric-imaging
#5
REVIEW
Minerva Becker, Salvatore Stefanelli, Anne-Laure Rougemont, Pierre Alexandre Poletti, Laura Merlini
Tumors of the pediatric facial skeleton represent a major challenge in clinical practice because they can lead to functional impairment, facial deformation, and long-term disfigurement. Their treatment often requires a multidisciplinary approach, and radiologists play a pivotal role in the diagnosis and management of these lesions. Although rare, pediatric tumors arising in the facial bones comprise a wide spectrum of benign and malignant lesions of osteogenic, fibrogenic, hematopoietic, neurogenic, or epithelial origin...
March 13, 2017: Neuroradiology
https://www.readbyqxmd.com/read/28288525/acquired-immune-deficiency-syndrome-associated-kaposi-sarcoma-in-a-child-presenting-as-a-solitary-plantar-hyperkeratotic-plaque
#6
Pierre-Olivier Grenier, Isabelle Auger
BACKGROUND AND OBJECTIVE: Acquired immune deficiency syndrome (AIDS)-associated Kaposi sarcoma (KS) among the pediatric population is a rare entity in North America and Europe, and its cutaneous manifestations are not well defined in the literature. The investigators report the case of a boy with an AIDS-associated KS presenting as an infiltrated hyperkeratotic plaque of the plantar arch. METHODS AND RESULTS: An 11-year-old African boy with congenital human immunodeficiency virus (HIV) had a skin biopsy of the plantar lesion that was consistent with a KS...
March 1, 2017: Journal of Cutaneous Medicine and Surgery
https://www.readbyqxmd.com/read/28272685/therapy-induced-neural-differentiation-in-ewing-s-sarcoma-a-case-report-and-review-of-the-literature
#7
Kıvılcım Eren Erdoğan, Mehmet Ali Deveci, Zeynep Ruken Hakkoymaz, Gülfiliz Gönlüşen
Ewing's sarcoma (ES) is a small round cell tumor of adolescents or young adults that usually arises in the deep soft tissues of the extremities. The tumor cells have uniform round nuclei, fine powdery chromatin and indistinct nucleoli. CD99 (O13) is a product of the MIC 2 gene that is highly sensitive to ES but not specific. A panel of markers should be used for the differential diagnosis of small round cell tumors because nearly all others, on occasion, show membranous staining for CD99. One of the defining feature of ES is the presence of 22q12 gene rearrangement...
February 4, 2017: Türk Patoloji Dergisi
https://www.readbyqxmd.com/read/28258912/risk-stratification-and-pattern-of-cardiotoxicity-in-pediatric-ewing-sarcoma
#8
Emad Moussa, Manal Zamzam, Ahmed Kamel, Zeinab Salah, Iman Attia, Lina Gaber, Ranin Soliman, Sameera Ezzat
INTRODUCTION: Anthracycline chemotherapy contributes to improved outcomes in Ewing sarcoma; however, the most feared complication is cardiotoxicity. Echocardiograms were routinely used to monitor cardiac function after anthracycline treatment. Nevertheless, indices chosen to assess cardiac toxicity vary significantly among different centers, and no uniform protocol has been accepted as ideal. METHODS: This retrospective study included children with Ewing sarcoma treated at Children's Cancer Hospital Egypt over 4years...
February 28, 2017: Journal of the Egyptian National Cancer Institute
https://www.readbyqxmd.com/read/28249647/a-clinicopathologic-study-on-ss18-fusion-positive-head-and-neck-synovial-sarcomas
#9
Adepitan A Owosho, Cherry L Estilo, Evan B Rosen, SaeHee K Yom, Joseph M Huryn, Cristina R Antonescu
OBJECTIVE: To determine clinicopathologic factors on survival in patients with head and neck synovial sarcoma. PATIENTS AND METHODS: We retrospectively identified patients with molecularly confirmed synovial sarcomas of the head and neck (SS-HN), either by the presence of SS18-SSX fusion transcript by RT-PCR or SS18 gene rearrangement by FISH, who were managed at our institution over a 20-year period (1996-2015). Kaplan-Meier survival analysis and log-rank test were performed to evaluate variables related to disease specific survival (DSS)...
March 2017: Oral Oncology
https://www.readbyqxmd.com/read/28248841/ewings-sarcoma-a-case-of-respiratory-distress-and-opacification-that-was-not-pneumonia
#10
Jordee M Wells, Sandra P Spencer
The Ewing sarcoma family of tumors typically appears in the second decade of life with regional pain and swelling of a long bone. The following case presents a pediatric patient, aged 4 years, given a diagnosis of Ewing sarcoma of the rib with the initial presentation of respiratory distress, hypoxia, and pleural effusion. Respiratory distress accounts for a large majority of emergency department visits annually, so it is the distinct responsibility of the emergency department physician to avoid premature closure in attributing the most common diagnoses to account for the presenting symptoms...
February 28, 2017: Pediatric Emergency Care
https://www.readbyqxmd.com/read/28248691/prospective-evaluation-of-postural-control-and-gait-in-pediatric-patients-with-cancer-after-a-4-week-inpatient-rehabilitation-program
#11
Carsten Müller, Dieter Rosenbaum, Konstantin A Krauth
OBJECTIVE: The aim of the study was to investigate the effects of a 4-wk inpatient rehabilitation program on postural control and gait in pediatric patients with cancer. DESIGN: Eighty-eight patients with brain tumors (n = 59) and bone/soft tissue sarcomas (n = 29) were evaluated. Postural control was assessed examining the velocity of the center of pressure and single-leg stance time on a pressure distribution platform. Walk ratio, a measure of neuromotor control, was used to evaluate intervention effects on gait...
February 28, 2017: American Journal of Physical Medicine & Rehabilitation
https://www.readbyqxmd.com/read/28197724/histological-and-immunohistochemical-characteristics-of-undifferentiated-small-round-cell-sarcomas-associated-with-cic-dux4-and-bcor-ccnb3-fusion-genes
#12
Yuichi Yamada, Masaaki Kuda, Kenichi Kohashi, Hidetaka Yamamoto, Junkichi Takemoto, Takeaki Ishii, Kunio Iura, Akira Maekawa, Hirofumi Bekki, Takamichi Ito, Hiroshi Otsuka, Makoto Kuroda, Yumi Honda, Shinji Sumiyoshi, Takeshi Inoue, Naoe Kinoshita, Atsushi Nishida, Kyoko Yamashita, Ichiro Ito, Shizuo Komune, Tomoaki Taguchi, Yukihide Iwamoto, Yoshinao Oda
CIC-DUX4 and BCOR-CCNB3 fusion-gene-associated small round cell sarcomas account for a proportion of pediatric small round cell sarcomas, but their pathological features have not been sufficiently clarified. We reviewed a large number of soft tissue tumors registered at our institution, retrieved the cases of unclassified tumors with a small round cell component, and subjected them to histopathological, immunohistochemical, and gene profile analysis. We reviewed 164 cases of unclassified tumors with a small round cell component and analyzed them by RT-PCR and FISH...
February 14, 2017: Virchows Archiv: An International Journal of Pathology
https://www.readbyqxmd.com/read/28186860/traumatic-rib-injury-patterns-imaging-pitfalls-complications-and-treatment
#13
Brett S Talbot, Christopher P Gange, Apeksha Chaturvedi, Nina Klionsky, Susan K Hobbs, Abhishek Chaturvedi
The ribs are frequently affected by blunt or penetrating injury to the thorax. In the emergency department setting, it is vital for the interpreting radiologist to not only identify the presence of rib injuries but also alert the clinician about organ-specific injury, specific traumatic patterns, and acute rib trauma complications that require emergent attention. Rib injuries can be separated into specific morphologic fracture patterns that include stress, buckle, nondisplaced, displaced, segmental, and pathologic fractures...
March 2017: Radiographics: a Review Publication of the Radiological Society of North America, Inc
https://www.readbyqxmd.com/read/28182004/tenovin-6-impairs-autophagy-by-inhibiting-autophagic-flux
#14
Hongfeng Yuan, Brandon Tan, Shou-Jiang Gao
Tenovin-6 has attracted significant interest because it activates p53 and inhibits sirtuins. It has anti-neoplastic effects on multiple hematopoietic malignancies and solid tumors in both in vitro and in vivo studies. Tenovin-6 was recently shown to impair the autophagy pathway in chronic lymphocytic leukemia cells and pediatric soft tissue sarcoma cells. However, whether tenovin-6 has a general inhibitory effect on autophagy and whether there is any involvement with SIRT1 and p53, both of which are regulators of the autophagy pathway, remain unclear...
February 9, 2017: Cell Death & Disease
https://www.readbyqxmd.com/read/28174375/-characteristics-of-adolescent-and-young-adult-cancer
#15
Keizo Horibe
In adolescent and young adult(AYA)population, cancer is the leading cause of death due to illness with the lowest mortality rate as well as children, and national measures for cancer was left behind. Leukemia, lymphomas, brain tumors, thyroid cancer, ovarian cancer, cervical cancer, breast cancer, testicular cancer and bone and soft tissue sarcomas account for majority of the cancers in this age group. There has been no significant improvement in AYA patients in contrast to other age groups. AYA patients with cancer are under growing independency, starting in life to the community, and reproductive age...
January 2017: Gan to Kagaku Ryoho. Cancer & Chemotherapy
https://www.readbyqxmd.com/read/28173994/intraoperative-high-dose-rate-brachytherapy-an-american-brachytherapy-society-consensus-report
#16
REVIEW
S Lloyd, K M Alektiar, S Nag, Y J Huang, C L Deufel, F Mourtada, D K Gaffney
PURPOSE: This report presents recommendations from the American Brachytherapy Society for the use of intraoperative high-dose-rate (IOHDR) brachytherapy. METHODS AND MATERIALS: Members of the American Brachytherapy Society with expertise in IOHDR formulated this document based on their clinical experience and a review of the literature. This report covers the use of IOHDR in colorectal cancer, soft tissue sarcoma, gynecologic cancers, head and neck cancers, and pediatric cancers...
February 4, 2017: Brachytherapy
https://www.readbyqxmd.com/read/28152768/improving-care-quality-for-patients-with-soft-tissue-and-bone-sarcoma-by-establishing-a-national-virtual-tumor-board-and-electronic-consultation-platform
#17
Andrew Fang, Maihgan Kavanagh, Brian Kim, Jason D Lee, Craig McCormick, Lee Jae Morse, Subir Nag, Grant Nybbaken, Peter Peng, Corey M Schwartz, Joseph Song, Denah Renee Taggart, Jeanette Cu Yu
211 Background: Bone and soft tissue sarcomas are relatively uncommon and their management is extremely complex requiring coordinated multidisciplinary expertise for optimal care. Because Kaiser Permanente operates across many geographic regions in the country, in-person tumor board is not practical. METHODS: The Kaiser Permanente Northern California medical oncology team has developed a system-based virtual tumor board (VTB) and electronic consultation (EC) platform comprised of experts from several key disciplines including musculoskeletal/surgical oncology, musculoskeletal radiology, pathology, radiation oncology, brachytherapy, medical adult and pediatric oncology...
March 2016: Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology
https://www.readbyqxmd.com/read/28141773/point-of-care-ultrasound-use-to-differentiate-hematoma-and-sarcoma-of-the-thigh-in-the-pediatric-emergency-department
#18
Cathie-Kim Le, Gregory Harvey, Lianne McLean, Jason Fischer
Hematomas and soft tissue sarcomas can be difficult to differentiate clinically, even with the addition of traditional imaging modalities. There are several case reports of sarcomas being misdiagnosed as hematomas, most commonly with a history of mild trauma. In this case report, we described a sarcoma initially misinterpreted as a hematoma on ultrasound. Key clinical features and sonographic findings that may assist clinicians using point-of-care ultrasound to correctly differentiate sarcomas from hematomas are reviewed...
February 2017: Pediatric Emergency Care
https://www.readbyqxmd.com/read/28137790/estimating-tp53-mutation-carrier-probability-in-families-with-li-fraumeni-syndrome-using-lfspro
#19
Gang Peng, Jasmina Bojadzieva, Mandy L Ballinger, Jialu Li, Amanda L Blackford, Phuong L Mai, Sharon A Savage, David M Thomas, Louise C Strong, Wenyi Wang
BACKGROUND: Li-Fraumeni syndrome (LFS) is associated with germline TP53 mutations and a very high lifetime cancer risk. Algorithms that assess a patient's risk of inherited cancer predisposition are often used in clinical counseling. The existing LFS criteria have limitations, suggesting the need for an advanced prediction tool to support clinical decision making for TP53 mutation testing and LFS management. METHODS: Based on a Mendelian model, LFSPRO estimates TP53 mutation probability through the Elston-Stewart algorithm, and consequently estimates future risk of cancer...
January 30, 2017: Cancer Epidemiology, Biomarkers & Prevention
https://www.readbyqxmd.com/read/28129857/discovering-novel-oncogenic-pathways-and-new-therapies-using-zebrafish-models-of-sarcoma
#20
M N Hayes, D M Langenau
Sarcoma is a type of cancer affecting connective, supportive, or soft tissue of mesenchymal origin. Despite rare incidence in adults (<1%), over 15% of pediatric cancers are sarcoma. Sadly, both adults and children with relapsed or metastatic disease have devastatingly high rates of mortality. Current treatment options for sarcoma include surgery, radiation, and/or chemotherapy; however, significant limitations exist with respect to the efficacy of these strategies. Strong impetus has been placed on the development of novel therapies and preclinical models for uncovering mechanisms involved in the development, progression, and therapy resistance of sarcoma...
2017: Methods in Cell Biology
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