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Childhood sarcoma

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https://www.readbyqxmd.com/read/29915264/recurrent-intragenic-rearrangements-of-egfr-and-braf-in-soft-tissue-tumors-of-infants
#1
Jenny Wegert, Christian Vokuhl, Grace Collord, Martin Del Castillo Velasco-Herrera, Sarah J Farndon, Charlotte Guzzo, Mette Jorgensen, John Anderson, Olga Slater, Catriona Duncan, Sabrina Bausenwein, Heike Streitenberger, Barbara Ziegler, Rhoikos Furtwängler, Norbert Graf, Michael R Stratton, Peter J Campbell, David Tw Jones, Christian Koelsche, Stefan M Pfister, William Mifsud, Neil Sebire, Monika Sparber-Sauer, Ewa Koscielniak, Andreas Rosenwald, Manfred Gessler, Sam Behjati
Soft tissue tumors of infancy encompass an overlapping spectrum of diseases that pose unique diagnostic and clinical challenges. We studied genomes and transcriptomes of cryptogenic congenital mesoblastic nephroma (CMN), and extended our findings to five anatomically or histologically related soft tissue tumors: infantile fibrosarcoma (IFS), nephroblastomatosis, Wilms tumor, malignant rhabdoid tumor, and clear cell sarcoma of the kidney. A key finding is recurrent mutation of EGFR in CMN by internal tandem duplication of the kinase domain, thus delineating CMN from other childhood renal tumors...
June 18, 2018: Nature Communications
https://www.readbyqxmd.com/read/29905027/increased-risk-of-bone-tumors-after-growth-hormone-treatment-in-childhood-a-population-based-cohort-study-in-france
#2
Amélie Poidvin, Jean-Claude Carel, Emmanuel Ecosse, Dominique Levy, Jean Michon, Joël Coste
The association between growth hormone (GH) treatment and cancer risk has not been thoroughly evaluated and there are questions about any increased risk of bone tumors. We examined cancer risk and especially bone tumor risk in a population-based cohort study of 6874 patients treated with recombinant GH in France for isolated GH deficiency, short stature associated with low birth weight or length or idiopathic short stature. Adult mortality and morbidity data obtained from national databases and from questionnaires...
June 14, 2018: Cancer Medicine
https://www.readbyqxmd.com/read/29869758/secondary-cancer-after-a-childhood-cancer-diagnosis-viewpoints-considering-primary-cancer
#3
Yasushi Ishida, Miho Maeda, Souichi Adachi, Hiroko Inada, Hiroshi Kawaguchi, Hiroki Hori, Atsushi Ogawa, Kazuko Kudo, Chikako Kiyotani, Hiroyuki Shichino, Takeshi Rikiishi, Ryoji Kobayashi, Maho Sato, Jun Okamura, Hiroaki Goto, Atsushi Manabe, Shinji Yoshinaga, Dongmei Qiu, Junichiro Fujimoto, Tatsuo Kuroda
BACKGROUNDS: Multidisciplinary therapy has increased the risk of subsequent late effects, but detailed analyses on secondary cancers in childhood cancer survivors (CCSs) are limited in Asian countries. METHODS: This was a retrospective cohort study comprising 10,069 CCSs who were diagnosed between 1980 and 2009 across 15 Japanese hospitals. We conducted secondary analyses to estimate the incidence of secondary cancer according to each primary malignancy and to elucidate the association between primary and secondary cancers...
June 5, 2018: International Journal of Clinical Oncology
https://www.readbyqxmd.com/read/29847298/genetic-risk-for-subsequent-neoplasms-among-long-term-survivors-of-childhood-cancer
#4
Zhaoming Wang, Carmen L Wilson, John Easton, Andrew Thrasher, Heather Mulder, Qi Liu, Dale J Hedges, Shuoguo Wang, Michael C Rusch, Michael N Edmonson, Shawn Levy, Jennifer Q Lanctot, Eric Caron, Kyla Shelton, Kelsey Currie, Matthew Lear, Aman Patel, Celeste Rosencrance, Ying Shao, Bhavin Vadodaria, Donald Yergeau, Yadav Sapkota, Russell J Brooke, Wonjong Moon, Evadnie Rampersaud, Xiaotu Ma, Ti-Cheng Chang, Stephen V Rice, Cynthia Pepper, Xin Zhou, Xiang Chen, Wenan Chen, Angela Jones, Braden Boone, Matthew J Ehrhardt, Matthew J Krasin, Rebecca M Howell, Nicholas S Phillips, Courtney Lewis, Deokumar Srivastava, Ching-Hon Pui, Chimene A Kesserwan, Gang Wu, Kim E Nichols, James R Downing, Melissa M Hudson, Yutaka Yasui, Leslie L Robison, Jinghui Zhang
Purpose Childhood cancer survivors are at increased risk of subsequent neoplasms (SNs), but the germline genetic contribution is largely unknown. We assessed the contribution of pathogenic/likely pathogenic (P/LP) mutations in cancer predisposition genes to their SN risk. Patients and Methods Whole-genome sequencing (30-fold) was performed on samples from childhood cancer survivors who were ≥ 5 years since initial cancer diagnosis and participants in the St Jude Lifetime Cohort Study, a retrospective hospital-based study with prospective clinical follow-up...
May 30, 2018: Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology
https://www.readbyqxmd.com/read/29844902/gene-expression-changes-associated-with-chemotherapy-resistance-in-ewing-sarcoma-cells
#5
Leonardo Horbach, Marialva Sinigaglia, Camila Alves Da Silva, Danielly Brufatto Olguins, Lauro José Gregianin, Algemir Lunardi Brunetto, André Tesainer Brunetto, Rafael Roesler, Caroline Brunetto De Farias
Ewing Sarcoma (ES) is a highly aggressive bone and soft tissue childhood cancer. The development of resistance to chemotherapy is common and remains the main cause of treatment failure. We herein evaluated the expression of genes associated with chemotherapy resistance in ES cell lines. A set of genes (CCAR1, TUBA1A, POLDIP2, SMARCA4 and SMARCB1) was data-mined for resistance against doxorubicin and vincristine, which are the standard drugs used in the treatment of patients with ES. The expression of each gene in SK-ES-1 ES cells was reported before and after exposure to a drug resistance-inducing protocol...
June 2018: Molecular and Clinical Oncology
https://www.readbyqxmd.com/read/29790086/testosterone-mediated-activation-of-androgenic-signalling-sustains-in-vitro-the-transformed-and-radioresistant-phenotype-of-rhabdomyosarcoma-cell-lines
#6
S Giannattasio, F Megiorni, V Di Nisio, A Del Fattore, R Fontanella, S Camero, C Antinozzi, C Festuccia, G L Gravina, S Cecconi, C Dominici, L Di Luigi, C Ciccarelli, P De Cesaris, A Riccioli, B M Zani, A Lenzi, R G Pestell, A Filippini, C Crescioli, V Tombolini, F Marampon
PURPOSE: Rhabdomyosarcoma (RMS), the most common soft-tissue sarcoma in childhood, rarely affects adults, preferring male. RMS expresses the receptor for androgen (AR) and responds to androgen; however, the molecular action of androgens on RMS is unknown. METHODS: Herein, testosterone (T) effects were tested in embryonal (ERMS) and alveolar (ARMS) RMS cell lines, by performing luciferase reporter assay, RT-PCR, and western blotting experiments. RNA interference experiments or bicalutamide treatment was performed to assess the specific role of AR...
May 22, 2018: Journal of Endocrinological Investigation
https://www.readbyqxmd.com/read/29764408/congenital-rhabdomyosarcoma-a-different-clinical-presentation-in-two-cases
#7
Ida Russo, Virginia Di Paolo, Carmelo Gurnari, Angela Mastronuzzi, Francesca Del Bufalo, Pier Luigi Di Paolo, Angela Di Giannatale, Renata Boldrini, Giuseppe Maria Milano
BACKGROUND: Rhabdomyosarcoma (RMS), one of the most common soft tissue sarcomas of childhood, is very rare in the neonatal period (0.4-2% of cases). In order to gain a deeper understanding of this disease at such age, patient and tumor features, as well as treatment modality and outcome need to be reported. CASE PRESENTATION: We describe two cases with congenital RMS treated at Bambino Gesù Children's Hospital between 2000 and 2016. They represent only 2.24% of all RMS patients diagnosed during that period in our Institution; this data is in agreement with the incidence reported in the literature...
May 15, 2018: BMC Pediatrics
https://www.readbyqxmd.com/read/29755264/an-update-on-mesenchymal-tumours-of-the-orbit-with-an-emphasis-on-the-value-of-molecular-cytogenetic-testing
#8
REVIEW
F Roberts, E M MacDuff
Mesenchymal tumours of the orbit are uncommon. Beyond childhood primary sarcomas are extremely rare and the literature is limited to case reports and short case series. However there is a diverse assortment of benign and malignant soft tissue tumours that may involve the orbit. Techniques to identify tumour specific cytogenetic or molecular genetic abnormalities often resulting in over- expressed proteins are becoming an increasingly important ancillary technique for these tumours. This review focuses on 3 specific areas: 1...
January 2018: Saudi Journal of Ophthalmology: Official Journal of the Saudi Ophthalmological Society
https://www.readbyqxmd.com/read/29729040/demographic-and-clinicopathologic-distribution-of-head-and-neck-malignant-tumors-in-pediatric-patients-from-a-brazilian-population-a-retrospective-study
#9
Lady Paola Aristizabal Arboleda, Iva Loureiro Hoffmann, Izilda Aparecida Cardinalli, Alan Roger Santos-Silva, Regina Maria Holanda de Mendonça
BACKGROUND: The incidence of pediatric head and neck cancer (PHNC) is increasing worldwide, especially when compared with childhood cancer in general. However, there is still a lack of knowledge about the demographic profile of such patients across the globe. Therefore, the aim of this study was to describe demographic, topographic and histopathological features of pediatric head and neck cancer patients from a single Brazilian institution. METHODS: Medical records were retrospectively reviewed for all cancer cases diagnosed from 1986 to 2016 affecting patients aged 19-years and younger...
May 4, 2018: Journal of Oral Pathology & Medicine
https://www.readbyqxmd.com/read/29722363/pediatric-kaposi-sarcoma-in-context-of-the-hiv-epidemic-in-sub-saharan-africa-current-perspectives
#10
REVIEW
Nader Kim El-Mallawany, Casey L McAtee, Liane R Campbell, Peter N Kazembe
The global experience with pediatric Kaposi sarcoma (KS) has evolved immensely since the onset of HIV (human immunodeficiency virus). In this review, current perspectives on childhood KS are discussed in the context of the HIV epidemic in sub-Saharan Africa. Endemic (HIV-unrelated) KS was first described over 50 years ago in central and eastern Africa, regions where human herpesvirus-8, the causative agent of KS, is endemic. With the alarming rise in HIV prevalence over the past few decades, KS has become not only the most common HIV-related malignancy in Africa, but also one of the most common overall childhood cancers throughout the central, eastern, and southern regions of the continent...
2018: Pediatric Health, Medicine and Therapeutics
https://www.readbyqxmd.com/read/29716532/limb-saving-surgery-for-ewing-s-sarcoma-of-the-distal-tibia-a-case-report
#11
Naoki Mizoshiri, Toshiharu Shirai, Ryu Terauchi, Shinji Tsuchida, Yuki Mori, Yusei Katsuyama, Daichi Hayashi, Yoshinobu Oka, Toshikazu Kubo
BACKGROUND: Ewing's sarcoma is a primary malignant tumor of bone occurring mostly in childhood. Few effective reconstruction techniques are available after wide resection of Ewing's sarcoma at the distal end of the tibia. Reconstruction after wide resection is especially difficult in children, as it is necessary to consider the growth and activity of the lower limbs. CASE PRESENTATION: A 12-year-old Japanese boy had presented with right lower leg pain at age 8 years...
May 2, 2018: BMC Cancer
https://www.readbyqxmd.com/read/29675944/advanced-mr-diffusion-imaging-and-chemotherapy-related-changes-in-cerebral-white-matter-microstructure-of-survivors-of-childhood-bone-and-soft-tissue-sarcoma
#12
Charlotte Sleurs, Jurgen Lemiere, Daan Christiaens, Thibo Billiet, Ronald Peeters, Stefan Sunaert, Anne Uyttebroeck, Sabine Deprez
With the increase of survival rates of pediatric cancer patients, the number of children facing potential cognitive sequelae has grown. Previous adult studies suggest that white matter (WM) microstructural changes may contribute to cognitive impairment. This study aims to investigate WM microstructure in childhood bone and soft tissue sarcoma. Differences in (micro-)structure can be investigated using diffusion MRI (dMRI). The typically used diffusion tensor model (DTI) assumes Gaussian diffusion, and lacks information about fiber populations...
April 20, 2018: Human Brain Mapping
https://www.readbyqxmd.com/read/29607435/renal-clear-cell-sarcoma-presenting-as-a-spontaneous-renal-hematoma-a-rare-presentation
#13
Zeynep Canan Ozdemir, Burcu Ayvaci, Yeter Duzenli Kar, Mehmet Oguzman, Mehmet Surhan Arda, Mustafa Fuat Acikalin, Ozcan Bor
Clear cell sarcoma of the kidney (CCSK) is an uncommon renal neoplasm of childhood. It represents between 2% and 9% of all pediatric renal tumors, and generally arises before the age of 5 years. It often mimics other pediatric renal tumors. Presently described is the case of a 7-year-old girl who presented with complaints of vomiting and abdominal pain. Abdominal ultrasonography revealed a right renal mass, and the patient developed a renal hematoma a few hours after admission. The patient underwent a nephroureterectomy with a provisional diagnosis of Wilms tumor; however, histopathological examination of a specimen revealed CCSK...
2018: Northern Clinics of Istanbul
https://www.readbyqxmd.com/read/29544999/overweight-in-childhood-cancer-patients-at-diagnosis-and-throughout-therapy-a-multicentre-cohort-study
#14
Fabiën N Belle, Juliane Wenke-Zobler, Eva Cignacco, Ben D Spycher, Roland A Ammann, Claudia E Kuehni, Karin Zimmermann
BACKGROUND: Childhood cancer patients (CCP) have been reported to be at increased risk of becoming overweight during treatment. We assessed prevalence of overweight in CCP at diagnosis and at the end of treatment, determined risk factors, and identified weight change during treatment by type of cancer. METHODS: In a multicentre cohort study, we collected height and weight measurements of CCP at diagnosis and repeatedly during treatment. We calculated age- and sex-adjusted BMI Z-scores using references of the International Obesity Taskforce for children...
March 2, 2018: Clinical Nutrition: Official Journal of the European Society of Parenteral and Enteral Nutrition
https://www.readbyqxmd.com/read/29539615/epidermal-growth-factor-receptor-regulation-of-ewing-sarcoma-cell-function
#15
Nathália Kersting, Bárbara Kunzler Souza, Igor Araujo Vieira, Rafael Pereira Dos Santos, Danielly Brufatto Olguins, Lauro José Gregianin, André Tesainer Brunetto, Algemir Lunardi Brunetto, Rafael Roesler, Caroline Brunetto de Farias, Gilberto Schwartsmann
OBJECTIVE: Ewing sarcoma (ES) is a type of childhood cancer probably arising from stem mesenchymal or neural crest cells. The epidermal growth factor receptor (EGFR) acts as a driver oncogene in many types of solid tumors. However, its involvement in ES remains poorly understood. METHODS: Human SK-ES-1 and RD-ES ES cells were treated with EGF, the EGFR inhibitor tyrphostin (AG1478), or phosphoinositide 3-kinase (PI3K) or extracellular-regulated kinase (ERK)/mitogen-activated kinase (MAPK) inhibitors...
March 14, 2018: Oncology
https://www.readbyqxmd.com/read/29514840/the-transcriptional-coactivator-taz-is-a-potent-mediator-of-alveolar-rhabdomyosarcoma-tumorigenesis
#16
Michael D Deel, Katherine K Slemmons, Ashley R Hinson, Katia C Genadry, Breanne A Burgess, Lisa E S Crose, Nina Kuprasertkul, Kristianne M Oristian, Rex C Bentley, Corinne M Linardic
Purpose: Alveolar rhabdomyosarcoma (aRMS) is a childhood soft tissue sarcoma driven by the signature PAX3-FOXO1 (P3F) fusion gene. Five-year survival for aRMS is <50%, with no improvement in over 4 decades. Although the transcriptional coactivator TAZ is oncogenic in carcinomas, the role of TAZ in sarcomas is poorly understood. The aim of this study was to investigate the role of TAZ in P3F-aRMS tumorigenesis. Experimental Design: After determining from publicly available datasets that TAZ is upregulated in human aRMS transcriptomes, we evaluated whether TAZ is also upregulated in our myoblast-based model of P3F-initiated tumorigenesis, and performed IHC staining of 63 human aRMS samples from tissue microarrays...
March 7, 2018: Clinical Cancer Research: An Official Journal of the American Association for Cancer Research
https://www.readbyqxmd.com/read/29509095/delayed-metastasis-of-clear-cell-sarcoma-of-kidney-to-bladder-after-7-disease-free-years
#17
Adam Lang, Louis P Dehner
INTRODUCTION: Clear cell sarcoma of the kidney (CCSK) is childhood neoplasm with its own distinctive pattern of metastasis and may appear after a disease free interval of 5 years or more. MATERIALS AND METHODS: Histopathology and immunohistochemistry were available from the radical nephrectomy and the later partial cystectomy, which was performed after a seven disease-free interval. RESULTS: The pathologic features of the primary tumor were those of a classic CCSK with a monotypic pattern of uniform rounded to ovoid tumor cells with a background network of delicate blood vessels...
April 2018: Fetal and Pediatric Pathology
https://www.readbyqxmd.com/read/29491605/aggressive-high-grade-ewing-s-sarcoma-of-maxilla-a-rare-case-report
#18
T L Yogesh, Akshay Shetty, Hema Keswani, Diljith Rishi
Ewing's sarcoma (ES) is an uncommon malignancy of childhood and adults that constitutes 6%-8% of all primary malignant tumors and the third-most common tumor after osteosarcoma and chondrosarcoma, frequently involves the mandible among jaw bones. This article presents a rare case of ES of maxilla in a 22-year-old male patient showing extensive lesion into skull base which was confirmed with computed tomography, dilemmatic histopathologic features in H and E which is not a frequent presentation. Histopathologic features showed monotonous round cells with hypo- and hyper-cellular areas, intralesional hemorrhage and necrosis with lesional cells positive for CD99...
January 2018: Journal of Oral and Maxillofacial Pathology: JOMFP
https://www.readbyqxmd.com/read/29485128/position-paper-rationale-for-the-treatment-of-children-with-ccsk-in-the-umbrella-siop-rtsg-2016-protocol
#19
REVIEW
Saskia L Gooskens, Norbert Graf, Rhoikos Furtwängler, Filippo Spreafico, Christophe Bergeron, Gema L Ramírez-Villar, Jan Godzinski, Christian Rübe, Geert O Janssens, Gordan M Vujanic, Ivo Leuschner, Aurore Coulomb-L'Hermine, Anne M Smets, Beatriz de Camargo, Sara Stoneham, Harm van Tinteren, Kathy Pritchard-Jones, Marry M van den Heuvel-Eibrink
The International Society of Paediatric Oncology-Renal Tumour Study Group (SIOP-RTSG) has developed a new protocol for the diagnosis, treatment, and follow-up monitoring of childhood renal tumours - the UMBRELLA SIOP-RTSG 2016 protocol (the UMBRELLA protocol). This protocol has been designed to continue international collaboration in the treatment of childhood renal tumours and will be implemented in over 50 different countries. Clear cell sarcoma of the kidney, which is a rare paediatric renal tumour that most commonly occurs in children between 2 and 4 years of age, is specifically addressed in the UMBRELLA protocol...
May 2018: Nature Reviews. Urology
https://www.readbyqxmd.com/read/29480840/clinical-management-of-a-unique-case-of-pnet-of-the-uterus-during-pregnancy-and-review-of-the-literature
#20
REVIEW
Rosalba De Nola, Edoardo Di Naro, Luca Maria Schonauer, Giuseppe Lucarelli, Michele Battaglia, Maria Grazia Fiore, Salvatore Andrea Mastrolia, Giuseppe Loverro
RATIONALE: PNETs (primitive neuroectodermal tumors) are a family of highly malignant neoplasms characterized by small round cells of neuroepithelial origin. They usually involve bone and soft tissues, and have a higher incidence in childhood. PATIENT CONCERNS: In this case report, we describe the obstetric and oncological outcome of a huge mass diagnosed as a leiomyoma in a 39-year-old pregnant woman who complained of low back pain, dysuria, and urinary frequency at 22 weeks of gestation...
January 2018: Medicine (Baltimore)
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