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Childhood sarcoma

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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
#1
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
#2
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
#3
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
#4
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-co-activator-taz-is-a-potent-mediator-of-alveolar-rhabdomyosarcoma-tumorigenesis
#5
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. 5-year survival for aRMS is <50%, with no improvement in over four decades. Although the transcriptional co-activator 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 public 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
#6
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...
March 6, 2018: Fetal and Pediatric Pathology
https://www.readbyqxmd.com/read/29491605/aggressive-high-grade-ewing-s-sarcoma-of-maxilla-a-rare-case-report
#7
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
#8
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...
February 27, 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
#9
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)
https://www.readbyqxmd.com/read/29468674/mental-health-of-long-term-survivors-of-childhood-and-young-adult-cancer-a-systematic-review
#10
REVIEW
Amanda J Friend, Richard G Feltbower, Emily J Hughes, Kristian P Dye, Adam W Glaser
Childhood cancer is increasing in prevalence whilst survival rates are improving. The prevalence of adult survivors of childhood cancer is consequently increasing. Many survivors suffer long-term consequences of their cancer treatment. Whilst many of these are well documented, relatively little is known about the mental health of survivors of childhood cancer. This article aimed to describe the prevalence and spectrum of mental health problems found in adult survivors of childhood cancer using a systematic review methodology...
February 22, 2018: International Journal of Cancer. Journal International du Cancer
https://www.readbyqxmd.com/read/29452297/lumbar-intraspinal-spindle-cell-rhabdomyosarcoma-as-a-rare-cause-of-spinal-stenosis-a-case-report
#11
Donel A Sequea, Maria E Reese
Rhabdomyosarcoma (RMS) represents the most common sarcoma in childhood, yet is extremely rare in adults with only a handful of cases reported. Here we present a case of intraspinal spindle cell RMS in an adult who presented as a typical case of spinal stenosis. To our knowledge, this is the first reported case of lumbar intraspinal spindle cell RMS in an adult patient. Furthermore, RMS phenotypically presents more aggressively in adults compared to children.
February 13, 2018: PM & R: the Journal of Injury, Function, and Rehabilitation
https://www.readbyqxmd.com/read/29434830/pediatric-sarcomas
#12
Junhua Cao, Qi An, Lei Wang
Sarcomas arise from primitive mesenchymal cells, which are classified, into two main groups: Bone and soft tissue sarcomas. We have searched all-important electronic databases including Google scholar and PubMed for the collection of latest literature pertaining to pediatric sarcomas. Latest literature confirmed that these tumors are relatively rare and represent only 1% of all malignancies but they have higher incidence in children. Pediatric sarcomas comprise about 13% of all pediatric malignancies and are ranked third in childhood cancers...
February 2018: Oncology Letters
https://www.readbyqxmd.com/read/29398803/therapy-related-aml-mds-following-treatment-for-childhood-cancer-experience-from-a-tertiary-care-centre-in-north-india
#13
Chintan Vyas, Sandeep Jain, Gauri Kapoor
Therapy-related acute myeloid leukemia/myelodysplastic syndrome (t-AML/MDS) is a devastating late effect of cancer treatment. There is limited data on incidence of t-AML/MDS from India. We retrospectively studied pediatric t AML/MDS at our institute between January 1996 and December 2015. Among 1285 children, 8 patients developed t-AML with a median age of 15.5 years. Overall incidence of t-AML/MDS was 0.62% [0.99% (4/402) in solid tumours and 0.45% (4/883) in leukemia/lymphoma, P  = 0.26] with 6390 patient years of follow up...
January 2018: Indian Journal of Hematology & Blood Transfusion
https://www.readbyqxmd.com/read/29397557/targeting-histone-deacetylase-activity-to-arrest-cell-growth-and-promote-neural-differentiation-in-ewing-sarcoma
#14
Bárbara Kunzler Souza, Patrícia Luciana da Costa Lopez, Pâmela Rossi Menegotto, Igor Araujo Vieira, Nathalia Kersting, Ana Lúcia Abujamra, André T Brunetto, Algemir L Brunetto, Lauro Gregianin, Caroline Brunetto de Farias, Carol J Thiele, Rafael Roesler
There is an urgent need for advances in the treatment of Ewing sarcoma (EWS), an aggressive childhood tumor with possible neuroectodermal origin. Inhibition of histone deacetylases (HDAC) can revert aberrant epigenetic states and reduce growth in different experimental cancer types. Here, we investigated whether the potent HDAC inhibitor, sodium butyrate (NaB), has the ability to reprogram EWS cells towards a more differentiated state and affect their growth and survival. Exposure of two EWS cell lines to NaB resulted in rapid and potent inhibition of HDAC activity (1 h, IC50 1...
February 3, 2018: Molecular Neurobiology
https://www.readbyqxmd.com/read/29397238/hereditary-swi-snf-complex-deficiency-syndromes
#15
REVIEW
Abbas Agaimy, William D Foulkes
The SWItch Sucrose non-fermentable (SWI/SNF) complex is a highly conserved multi-subunit complex of proteins encoded by numerous genes mapped to different chromosomal regions. The complex regulates the process of chromatin remodelling and hence plays a central role in the epigenetic regulation of gene expression, cell proliferation and differentiation. During the last three decades, the SWI/SNF complex has been increasingly recognized as a central molecular event driving the initiation and/or progression of several benign and malignant neoplasms of different anatomic origin and having diverse histomorphological appearance...
February 1, 2018: Seminars in Diagnostic Pathology
https://www.readbyqxmd.com/read/29367756/pax3-foxo1-drives-mir-486-5p-and-represses-mir-221-contributing-to-pathogenesis-of-alveolar-rhabdomyosarcoma
#16
Jason A Hanna, Matthew R Garcia, Alicia Lardennois, Patrick J Leavey, Dino Maglic, Alexandre Fagnan, Jonathan C Go, Jordan Roach, Yong-Dong Wang, David Finkelstein, Mark E Hatley
Rhabdomyosarcoma is the most common soft-tissue sarcoma in childhood and histologically resembles developing skeletal muscle. Alveolar rhabdomyosarcoma (ARMS) is an aggressive subtype with a higher rate of metastasis and poorer prognosis. The majority of ARMS tumors (80%) harbor a PAX3-FOXO1 or less commonly a PAX7-FOXO1 fusion gene. The presence of either the PAX3-FOXO1 or PAX7-FOXO1 fusion gene foretells a poorer prognosis resulting in clinical re-classification as either fusion-positive (FP-RMS) or fusion-negative RMS (FN-RMS)...
January 25, 2018: Oncogene
https://www.readbyqxmd.com/read/29345507/ovarian-tissue-cryopreservation-in-young-females-through-the-oncofertility-consortium-s-national-physicians-cooperative
#17
Austin G Armstrong, Bruce F Kimler, Brigid M Smith, Teresa K Woodruff, Mary Ellen Pavone, Francesca E Duncan
AIM: To characterize the clinical indications of females (<15 years old) undergoing ovarian tissue cryopreservation (OTC) through the Oncofertility Consortium's National Physicians Cooperative (OC-NPC). PATIENTS & METHODS: The clinical indications of 114 females who underwent OTC were classified, and their incidence was compared with childhood cancer databases. RESULTS: Leukemias/myeloproliferative diseases/myelodysplastic diseases and hemoglobinopathies were the most prevalent oncologic and nononcologic indications for OTC, respectively...
January 18, 2018: Future Oncology
https://www.readbyqxmd.com/read/29343557/dicer1-and-associated-conditions-identification-of-at-risk-individuals-and-recommended-surveillance-strategies
#18
Kris Ann P Schultz, Gretchen M Williams, Junne Kamihara, Douglas R Stewart, Anne K Harris, Andrew J Bauer, Joyce Turner, Rachana Shah, Katherine Schneider, Kami Wolfe Schneider, Ann Garrity Carr, Laura A Harney, Shari Baldinger, A Lindsay Frazier, Daniel Orbach, Dominik T Schneider, David Malkin, Louis P Dehner, Yoav H Messinger, Ashley Hill
Pathogenic germline DICER1 variants cause a hereditary cancer predisposition syndrome with a variety of manifestations. In addition to conferring increased cancer risks for pleuropulmonary blastoma (PPB) and ovarian sex cord-stromal tumors, particularly Sertoli-Leydig cell tumor, individuals with pathogenic germline DICER1 variants may also develop lung cysts, cystic nephroma, renal sarcoma and Wilms tumor, nodular hyperplasia of the thyroid, nasal chondromesenchymal hamartoma, ciliary body medulloepithelioma, genitourinary embryonal rhabdomyosarcoma and brain tumors including pineoblastoma and pituitary blastoma...
January 17, 2018: Clinical Cancer Research: An Official Journal of the American Association for Cancer Research
https://www.readbyqxmd.com/read/29333015/a-profile-of-pediatric-solid-tumors-a-single-institution-experience-in-kashmir
#19
Namita Sharma, Ayesha Ahmad, Gull M Bhat, Sheikh A Aziz, Mohammad Maqbool Lone, Nisar A Bhat
Aims: The purpose of this retroprospective study was to study the epidemiological characteristics and outcomes of children with solid tumors at our institution. Subjects and Methods: Three hundred and three pediatrics patients registered at Regional Cancer Centre (RCC), Sher-i-Kashmir Institute of Medical Sciences (SKIMS), Srinagar, Kashmir, between January 2008 and June 2014, were analyzed with regard to demographic status, presenting complaints, investigations, treatment, morbidity, and outcomes...
October 2017: Indian Journal of Medical and Paediatric Oncology
https://www.readbyqxmd.com/read/29280680/smarcb1-deficient-tumors-of-childhood-a-practical-guide
#20
Bruce R Pawel
The SMARCB1 gene ( INI1, BAF47) is a member of the SWItch/Sucrose Non-Fermentable (SWI/SNF) chromatin remodeling complex, involved in the epigenetic regulation of gene transcription. SMARCB1 acts as a tumor suppressor gene, and loss of function of both alleles gives rise to SMARCB1-deficient tumors. The prototypical SMARCB1-deficient tumor is the malignant rhabdoid tumor (MRT) which was first described in the kidney but also occurs in soft tissue, viscera, and the brain (where it is referred to as atypical teratoid rhabdoid tumor or AT/RT)...
January 2018: Pediatric and Developmental Pathology
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