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https://www.readbyqxmd.com/read/28806574/quantitative-diffusion-weighted-magnetic-resonance-imaging-assessment-of-chemotherapy-treatment-response-of-pediatric-osteosarcoma-and-ewing-sarcoma-malignant-bone-tumors
#1
Andrew J Degnan, Chul Y Chung, Amisha J Shah
OBJECTIVE: Assessment of tumor response to chemotherapy is essential in managing malignant pediatric bone tumors prior to resection. SUBJECTS AND METHODS: Pre-chemotherapy and post-chemotherapy osteosarcoma and Ewing sarcoma cases (n=18) were analyzed with apparent diffusion coefficient (ADC) values measured by two readers. RESULTS: Treated tumors demonstrated significantly greater ADC values compared to untreated tumors (p<0.001). Intraclass correlation coefficients ranged between 0...
August 5, 2017: Clinical Imaging
https://www.readbyqxmd.com/read/28806393/craf-gene-fusions-in-pediatric-low-grade-gliomas-define-a-distinct-drug-response-based-on-dimerization-profiles
#2
P Jain, T M Fierst, H J Han, T E Smith, A Vakil, P J Storm, A C Resnick, A J Waanders
Pediatric low-grade gliomas (PLGGs) are commonly associated with BRAF gene fusions that aberrantly activate the mitogen-activated protein kinase (MAPK) signaling pathway. This has led to PLGG clinical trials utilizing RAF- and MAPK pathway-targeted therapeutics. Whole-genome profiling of PLGGs has also identified rare gene fusions involving another RAF isoform, CRAF/RAF1, in PLGGs and cancers occuring in adults. Whereas BRAF fusions primarily dysregulate MAPK signaling, the CRAF fusions QKI-RAF1 and SRGAP3-RAF1 aberrantly activate both the MAPK and phosphoinositide-3 kinase/mammalian target of rapamycin (PI3K/mTOR) signaling pathways...
August 14, 2017: Oncogene
https://www.readbyqxmd.com/read/28804898/epilepsy-surgery-of-low-grade-epilepsy-associated-neuroepithelial-tumors-a-retrospective-nationwide-italian-study
#3
Marco Giulioni, Gianluca Marucci, Veronica Pelliccia, Francesca Gozzo, Carmen Barba, Giuseppe Didato, Flavio Villani, Giancarlo Di Gennaro, Pier Paolo Quarato, Vincenzo Esposito, Alessandro Consales, Matteo Martinoni, Gianfranco Vornetti, Corrado Zenesini, Carlo Efisio Marras, Nicola Specchio, Luca De Palma, Raffaele Rocchi, Flavio Giordano, Giovanni Tringali, Paolo Nozza, Gabriella Colicchio, Guido Rubboli, Giorgio Lo Russo, Renzo Guerrini, Paolo Tinuper, Francesco Cardinale, Massimo Cossu
OBJECTIVE: To analyze the attitude and results of Italian epilepsy surgery centers in the surgical management of "low grade epilepsy associated neuroepithelial tumors" (LEATs). METHODS: We conducted a retrospective study enrolling 339 consecutive patients with LEATs who underwent surgery between January 2009 and June 2015 at eight Italian epilepsy surgery centers. We compared demographic, clinical, pathologic, and surgical features of patients with favorable (Engel class I) and unfavorable (Engel class II, III, and IV) seizure outcome...
August 14, 2017: Epilepsia
https://www.readbyqxmd.com/read/28803261/advantages-in-prognosis-of-adult-patients-with-ewing-sarcoma-11-years-experiences-and-current-treatment-management
#4
Dagmar Adamkova Krakorova, Katerina Kubackova, Ladislav Dusek, Tomas Tomas, Pavel Janicek, Stepan Tucek, Jana Prausova, Igor Kiss, Iva Zambo
Ewing sarcoma (ES) is an exceptionally rare tumor in adults. Data regarding outcomes of adult patients with ES and experiences with age-adapted therapeutic strategies are very limited. The aim of this study was to evaluate prognostic factors and clinical outcome in a cohort of adult patients treated according to pediatric protocols in the Czech Republic. The records of 58 adult ES patients diagnosed between 2002 and 2013 were reviewed and factors relevant to prognosis and survival were analyzed. The median age of study cohort was 29 years (range, 18-59)...
August 12, 2017: Pathology Oncology Research: POR
https://www.readbyqxmd.com/read/28803259/opportunities-and-challenges-in-the-immunological-therapy-of-pediatric-malignancy-a-concise-snapshot
#5
REVIEW
Francesco Ceppi, Maja Beck-Popovic, Jean-Pierre Bourquin, Raffaele Renella
Over the last 50 years, collaborative clinical trials have reduced the number of children dying from pediatric cancer significantly. Unfortunately, certain tumor types have remained resistant to conventional surgical, radiotherapy and chemotherapy combinations, and relapsing and/or refractory disease remains associated with dismal outcomes. Recently, renewed attention has been given to the role for immunotherapies in pediatric oncology. In fact, these combine several attractive features, including (but possibly not limited to) the specificity for cancer cells, potentially in vivo persistence and longevity, and potency against refractory disease...
August 12, 2017: European Journal of Pediatrics
https://www.readbyqxmd.com/read/28801451/adult-high-grade-b-cell-lymphoma-with-burkitt-lymphoma-signature-genomic-features-and-potential-therapeutic-targets
#6
Alyssa Bouska, Chengfeng Bi, Waseem Lone, Weiwei Zhang, Ambreen Kedwaii, Tayla Heavican, Cynthia M Lachel, Jiayu Yu, Roberto Ferro, Nanees Eldorghamy, Timothy C Greiner, Julie Vose, Dennis D Weisenburger, Randy D Gascoyne, Andreas Rosenwald, German Ott, Elias Campo, Lisa M Rimsza, Elaine S Jaffe, Rita M Braziel, Reiner Siebert, Rodney R Miles, Sandeep Dave, Anupama Reddy, Jan Delabie, Louis M Staudt, Joo Y Song, Timothy W McKeithan, Kai Fu, Michael Green, Wing C Chan, Javeed Iqbal
The adult high-grade B-cell lymphomas sharing molecular features with Burkitt lymphoma (BL) are highly aggressive lymphomas with poor clinical outcome. High-resolution structural and functional genomic analysis of adult Burkitt lymphoma (BL) and high-grade B-cell lymphoma with BL gene-signature (adult-mBL), revealed the MYC-ARF-p53 axis as the primary deregulated pathway. Adult-mBL had either unique or more frequent genomic aberrations (del13q14, del17p, gain8q24 and gain18q21) compared with pediatric-mBL, but shared commonly mutated genes...
August 11, 2017: Blood
https://www.readbyqxmd.com/read/28801324/mechanical-thrombectomy-for-repeated-cerebral-tumor-embolism-from-a-thoracic-sarcomatoid-carcinoma
#7
Raoul Pop, Dan Mihoc, Monica Manisor, Johann Sebastian Richter, Veronique Lindner, Ralf Janssen-Langenstein, Mihaela Simu, Valerie Wolff, Remy Beaujeux
Cerebral embolism originating from intracardiac tumors represents a rare cause of stroke and has been documented in both adult and pediatric populations. We present a patient recently diagnosed with a right pulmonary hilum tumor, invading the pulmonary veins and the left atrium. Two consecutive episodes of large cerebral vessel occlusion in separate vascular territories occurred in the same day and were treated by mechanical thrombectomy. Embolic material retrieved on both occasions contained tumor fragments with peripheral endothelialization...
August 11, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28800318/mir-513c-suppresses-neuroblastoma-cell-migration-invasion-and-proliferation-through-direct-targeting-glutaminase-gls
#8
Hong-Liang Xia, Yao Lv, Chun-Wei Xu, Ming-Cui Fu, Ting Zhang, Xiang-Ming Yan, Shu Dai, Qian-Wei Xiong, Yun Zhou, Jian Wang, Xu Cao
Neuroblastoma is a brain malignancy of childhood and accounts for 7-10% of childhood cancers, leading to approximately 15% of pediatric cancer deaths. MicroRNAs (miRNAs) are a family of short (about 18-25 nucleotides), noncoding and single stranded endogenous RNAs, which complementarily bind to the 3' untranslated regions of their target genes. Recently, glutamine metabolism has been recognized as an important nutrition source for tumor cells, and hence targeting glutamine metabolism could benefit to development of anti-cancer agents...
July 2, 2017: Cancer Biomarkers: Section A of Disease Markers
https://www.readbyqxmd.com/read/28798218/imaging-biomarkers-for-adult-medulloblastomas-genetic-entities-may-be-identified-by-their-mr-imaging-radiophenotype
#9
V C Keil, M Warmuth-Metz, C Reh, S J Enkirch, C Reinert, D Beier, D T W Jones, T Pietsch, H H Schild, E Hattingen, P Hau
BACKGROUND AND PURPOSE: The occurrence of medulloblastomas in adults is rare; nevertheless, these tumors can be subdivided into genetic and histologic entities each having distinct prognoses. This study aimed to identify MR imaging biomarkers to classify these entities and to uncover differences in MR imaging biomarkers identified in pediatric medulloblastomas. MATERIALS AND METHODS: Eligible preoperative MRIs from 28 patients (11 women; 22-53 years of age) of the Multicenter Pilot-study for the Therapy of Medulloblastoma of Adults (NOA-7) cohort were assessed by 3 experienced neuroradiologists...
August 10, 2017: AJNR. American Journal of Neuroradiology
https://www.readbyqxmd.com/read/28797854/why-computerized-adaptive-testing-in-pediatric-brain-tumor-clinics
#10
Jin-Shei Lai, Jennifer L Beaumont, Cindy J Nowinski, David Cella, William F Hartsell, John Han-Chih Chang, Peter E Manley, Stewart Goldman
CONTEXT: Monitoring of health-related quality of life (HRQOL) and symptoms of patients with brain tumors is needed yet not always feasible. This is partially due to lack of brief-yet-precise assessments with minimal administration burden that are easily incorporated into clinics. Dynamic computerized adaptive testing (CAT) or static fixed-length short-forms, derived from psychometrically-sound item banks, are designed to fill this void. OBJECTIVE: This study evaluated the comparability of scores obtained from CATs and short-forms...
August 7, 2017: Journal of Pain and Symptom Management
https://www.readbyqxmd.com/read/28796925/neurological-comorbidity-in-children-with-neurofibromatosis-type-1
#11
Keiko Hirabaru, Muneaki Matsuo
OBJECTIVE: To determine the frequency of central nervous system comorbidities in children with neurofibromatosis type 1 (NF1). METHODS: We performed a nationwide survey to investigate neurological comorbidities in 3-15-year-old children with NF1 in Japan by sending questionnaires to pediatricians and pediatric neurologists. A secondary questionnaire was sent to the parents of identified NF1 patients to assess neurological comorbidities including headache, attention deficit hyperactivity disorder (ADHD)-Rating Scale (RS), and the Social Responsiveness Scale -2...
August 10, 2017: Pediatrics International: Official Journal of the Japan Pediatric Society
https://www.readbyqxmd.com/read/28794343/comparative-histopathological-analysis-of-sporadic-pediatric-papillary-thyroid-carcinoma-from-japan-and-ukraine
#12
Tetiana I Bogdanova, Vladimir A Saenko, Mitsuyoshi Hirokawa, Masahiro Ito, Liudmyla Yu Zurnadzhy, Toshitetsu Hayashi, Tatiana I Rogounovitch, Akira Miyauchi, Mykola D Tronko, Shunichi Yamashita
This study set out to compare structural and invasive characteristics of sporadic papillary thyroid carcinoma (PTC) in age-matched groups of children and adolescents of Japan and Ukraine to provide detailed histopathological analysis of tumors from different geographical areas with different iodine intake. A total of 348 (160 Japanese and 188 Ukrainian) PTCs from patients without radiation history were analyzed initially as a combined pediatric group and then subdivided into childhood (aged ≤14 years) and adolescent (aged from 15 to ≤18 years) age series...
August 10, 2017: Endocrine Journal
https://www.readbyqxmd.com/read/28792662/comparing-oncologic-outcomes-after-minimally-invasive-and-open-surgery-for-pediatric-neuroblastoma-and-wilms-tumor
#13
Brian Ezekian, Brian R Englum, Brian C Gulack, Kristy L Rialon, Jina Kim, Lindsay J Talbot, Obinna O Adibe, Jonathan C Routh, Elisabeth T Tracy, Henry E Rice
BACKGROUND: Minimally invasive surgery (MIS) has been widely adopted for common operations in pediatric surgery; however, its role in childhood tumors is limited by concerns about oncologic outcomes. We compared open and MIS approaches for pediatric neuroblastoma and Wilms tumor (WT) using a national database. METHODS: The National Cancer Data Base from 2010 to 2012 was queried for cases of neuroblastoma and WT in children ≤21 years old. Children were classified as receiving open or MIS surgery for definitive resection, with clinical outcomes compared using a propensity matching methodology (two open:one MIS)...
August 9, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28792659/molecular-alterations-in-pediatric-brainstem-gliomas
#14
Mikaela Porkholm, Anna Raunio, Reetta Vainionpää, Tarja Salonen, Juha Hernesniemi, Leena Valanne, Jarno Satopää, Atte Karppinen, Minna Oinas, Olli Tynninen, Virve Pentikäinen, Sanna-Maria Kivivuori
BACKGROUND: Diffuse intrinsic pontine gliomas (DIPGs) have a dismal prognosis. Previously, diagnosis was based on a typical clinical presentation and magnetic resonance imaging findings. After the start of the era of biopsies, DIPGs bearing H3 K27 mutations have been reclassified into a novel entity, diffuse midline glioma, based on the presence of this molecular alteration. However, it is not well established how clinically diagnosed DIPG overlap with H3 K27-mutated diffuse midline gliomas, and whether rare long-term survivors also belong to this group...
August 9, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28791279/pneumocystis-jirovecii-pneumonia-in-pediatric-inflammatory-bowel-disease-a-case-report-and-literature-review
#15
Sally J Lawrence, Manish Sadarangani, Kevan Jacobson
Immunosuppressive therapy is a known risk factor for opportunistic infections. We report the first case of severe Pneumocystis jirovecii infection requiring intensive care in a pediatric patient with inflammatory bowel disease (IBD). The literature was reviewed and there were 92 reported cases of Pneumocystis pneumonia (PCP) in patients with IBD. Most sources were case reports and there was likely reporting bias toward patients receiving immunomodulators, anti-tumor necrosis factor (anti-TNF) therapy, and those who died...
2017: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/28791278/venous-thromboembolism-in-pediatric-vascular-anomalies
#16
REVIEW
Taizo A Nakano, Chadi Zeinati
The presence of a vascular anomaly suggests that capillaries, veins, arteries, and/or lymphatic vessels have demonstrated abnormal development and growth. Often dilated and misshaped, these vessels augment normal flow of blood and lymphatic fluids that increases the overall risk to develop intralesional thrombosis. Abnormal endothelial and lymphoendothelial cells activate hemostasis and hyperfibrinolytic pathways through poorly understood mechanisms, which contribute to the development of localized intravascular coagulopathy...
2017: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/28790477/pediatric-lumbar-pedicle-screw-placement-using-navigation-templates-a-cadaveric-study
#17
Xing Wang, Jun Shi, Shojie Zhang, Zhifeng Zhang, Xiaohe Li, Zhijun Li
BACKGROUND: Pedicle screw technique is commonly used treatment of adult spinal trauma, tumor, degeneration. The application of pedicle screws is very challenging in children because children have a fast growing spine and spinal pedicle morphology of children and adult has large difference. 3 D reconstruction individual navigation templates improve the success rate of pediatric pedicle screw system. This study is aimed to provide a precise method for lumbar spine pedicle screw placement in children using computer-aided design and rapid prototyping technology...
July 2017: Indian Journal of Orthopaedics
https://www.readbyqxmd.com/read/28790217/-an-elderly-patient-with-pilocytic-astrocytoma-operated-on-14-years-after-initial-detection-a-case-study
#18
Naoyuki Isobe, Takeshi Nishimoto, Takeshi Ueda
Pilocytic astrocytomas are found predominantly in the pediatric population and are extremely rare in elderly patients. We describe a case of pilocytic astrocytoma in an elderly patient who presented with the symptoms of an enlarged tumoral cyst. A 70-year-old woman was found to have an asymptomatic small solid tumor with a cystic component in the right frontal lobe at "Ningen Dock(medical checkup)of the Brain". She was hospitalized and underwent further examinations including cerebral angiography. At that time, she was opposed to undergoing an operation for tumor removal owing to lack of symptoms...
August 2017: No Shinkei Geka. Neurological Surgery
https://www.readbyqxmd.com/read/28783147/marrow-ablative-chemotherapy-followed-by-tandem-autologous-hematopoietic-cell-transplantation-in-pediatric-patients-with-malignant-brain-tumors
#19
J A Guerra, G Dhall, A Marachelian, E Castillo, J Malvar, K Wong, R Sposto, J L Finlay
To improve survival in young children with malignant brain tumors, irradiation-avoiding or -minimizing marrow-ablative chemotherapy (HDCx) with autologous hematopoietic cell transplantation (AuHCT) has been investigated. We evaluated the outcome of 44 children with malignant brain tumors treated with HDCx and tandem AuHCT at Children's Hospital Los Angeles between June 1999 and July 2012. Forty-four children with malignant brain tumors were studied. Twenty-one had medulloblastoma/primitive neuro-ectodermal tumor, eight atypical teratoid/rhabdoid tumor (ATRT), five high-grade glioma, four malignant germ cell tumor, three ependymoma and three choroid plexus carcinoma...
August 7, 2017: Bone Marrow Transplantation
https://www.readbyqxmd.com/read/28781917/long-term-recurrence-of-dysembryoplastic-neuroepithelial-tumor-clinical-case-report
#20
Daniel A Tonetti, William J Ares, R Mark Richardson, Ronald L Hamilton, Frank S Lieberman
BACKGROUND: Dysembryoplastic neuroepithelial tumors (DNETs) are rare, benign brain neoplasms that typically arise in children and adolescents and classically present with intractable, partial complex seizures. DNETs are classically associated with a favorable prognosis after complete surgical resection. CASE DESCRIPTION: We describe a case of long-term recurrence of a DNET, which initially resected and diagnosed as an oligodendroglioma prior to the recognition of DNETs...
2017: Surgical Neurology International
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