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https://www.readbyqxmd.com/read/28432584/clinical-features-and-long-term-outcomes-of-pediatric-spinal-meningiomas
#1
Liang Wu, Chenlong Yang, Tie Liu, Jingyi Fang, Jun Yang, Yulun Xu
Pediatric spinal meningiomas are very rare. Most studies on pediatric spinal meningiomas are case reports with literature reviews. This study presented a surgical series of 14 pediatric patients with histologically proven sporadic spinal meningiomas, including 5 WHO grade II tumors. In this series, there were 9 male and 5 female patients with a mean age of 11.1 years. Seven tumors were located in the cervical spine, 5 in the thoracic spine, and 2 in the lumbar spine. Gross total resection (GTR) of the tumor with a well-demarcated dissection plane was achieved in 11 cases, subtotal resection was achieved in 2 cases, and partial resection was performed in 1 case...
April 21, 2017: Journal of Neuro-oncology
https://www.readbyqxmd.com/read/28432176/a-phase-i-study-of-the-cdk4-6-inhibitor-ribociclib-lee011-in-pediatric-patients-with-malignant-rhabdoid-tumors-neuroblastoma-and-other-solid-tumors
#2
Birgit Geoerger, Franck Bourdeaut, Steven G DuBois, Matthias Fischer, James I Geller, Nicholas G Gottardo, Aurélien Marabelle, Andrew D J Pearson, Shakeel Modak, Thomas Cash, Giles W Robinson, Marlyane Motta, Alessandro Matano, Suraj G Bhansali, Jason R Dobson, Sudha Parasuraman, Susan N Chi
Purpose: The cyclin-dependent kinase (CDK) 4/6 inhibitor, ribociclib (LEE011), displayed preclinical activity in neuroblastoma and malignant rhabdoid tumor (MRT) models. In this phase I study, the maximum tolerated dose (MTD) and recommended phase II dose (RP2D), safety, pharmacokinetics (PK), and preliminary activity of single-agent ribociclib were investigated in pediatric patients with neuroblastoma, MRT, or other cyclin D-CDK4/6-INK4-retinoblastoma pathway-altered tumors.Experimental Design: Patients (aged 1-21 years) received escalating once-daily oral doses of ribociclib (3-weeks-on/1-week-off)...
April 21, 2017: Clinical Cancer Research: An Official Journal of the American Association for Cancer Research
https://www.readbyqxmd.com/read/28431807/rhabdomyosarcoma-of-the-maxillofacial-region-in-children-and-adolescents-report-of-9-cases-and-literature-review
#3
Ioannis Iatrou, Nadia Theologie-Lygidakis, Ourania Schoinohoriti, Fotis Tzermpos, Anna-Maria Vessala
OBJECTIVE: To review clinical presentation, histology, treatment and survival for pediatric maxillofacial rhabdomyosarcoma (RMS) and evaluate the role of surgical treatment. STUDY DESIGN: Retrospective analysis of medical charts. METHODS: Files of patients, treated for primary maxillofacial RMS from 1997 to 2016, were examined for clinical presentation, staging, histology, treatment protocol and complications, recurrence and final outcome...
March 23, 2017: Journal of Cranio-maxillo-facial Surgery
https://www.readbyqxmd.com/read/28431179/mirna-regulation-in-gliomas-usual-suspects-in-glial-tumorigenesis-and-evolving-clinical-applications
#4
Heather Ames, Marc K Halushka, Fausto J Rodriguez
In recent years, an increasing role for noncoding small RNAs (miRNA) has been uncovered in carcinogenesis. These oligonucleotides can promote degradation and/or inhibit translation of key mRNAs. Recent studies have also highlighted a possible role for miRNAs in adult and pediatric brain tumors, including high- and low-grade gliomas, medulloblastoma, ependymoma, and neoplasms associated with neurofibromatosis type 1. Gliomas represent the most common category of primary intraparenchymal brain tumors, and, for example, manipulation of signaling pathways, through inhibition of PTEN transcription appears to be an important function of miRNA dysregulation through miR-21, miR-106b, and miR-26a...
April 1, 2017: Journal of Neuropathology and Experimental Neurology
https://www.readbyqxmd.com/read/28430083/hydrocephalus-treatment-in-children-long-term-outcome-in-975-consecutive-patients
#5
Pierre-Aurelien Beuriat, Stephanie Puget, Giuseppe Cinalli, Thomas Blauwblomme, Kevin Beccaria, Michel Zerah, Christian Sainte-Rose
OBJECTIVE Hydrocephalus remains one of the more common pathologies managed in pediatric neurosurgical units. Endoscopic third ventriculostomy (ETV) has an advantage over ventriculoperitoneal shunting as it enables patients to remain device free. Multiple shunt devices with various valve designs exist, with no one valve proven to be superior to another. The aim of this study was to describe the management of hydrocephalus and its long-term outcome. METHODS The authors retrospectively reviewed the medical records of all patients who had been treated for hydrocephalus at the Hôpital Necker-Enfants Malades in the period from 1985 to 1995...
April 21, 2017: Journal of Neurosurgery. Pediatrics
https://www.readbyqxmd.com/read/28429507/mucinous-hidradenoma-in-a-child-a-case-report-and-review-of-the-literature
#6
Aadil Ahmed, Wendy Kim, Jodi Speiser
Hidradenomas are benign tumors of the sweat glands that are rarely reported in childhood. The presence of mucinous change in hidradenomas, which consists of variable numbers of mucin-rich goblet cells, is occasionally observed in some adult clinical practices. However, it has not been previously reported in the pediatric population. Herein, we present a unique case of a mucinous hidradenoma presenting on the thigh of a 3-year old boy. The clinical presentation and histological features of the case, along with a literature review of published case reports of pediatric hidradenomas are described...
April 21, 2017: Journal of Cutaneous Pathology
https://www.readbyqxmd.com/read/28428785/interferon-gamma-induces-changes-in-natural-killer-nk-cell-ligand-expression-and-alters-nk-cell-mediated-lysis-of-pediatric-cancer-cell-lines
#7
Arianexys Aquino-López, Vladimir V Senyukov, Zlatko Vlasic, Eugenie S Kleinerman, Dean A Lee
Natural killer (NK) cells have therapeutic potential for cancer due to their capacity for targeting tumor cells without prior sensitization. Our laboratory has developed an NK cell expansion protocol that generates large quantities of NK cells for therapeutic infusion that secret 20 times the amount of interferon gamma (IFNγ) than resting NK cells. IFNγ can upregulate major histocompatibility complex (MHC)-class I, an inhibitory ligand for NK cells, but can also upregulate intercellular adhesion molecule 1 (ICAM-1) which promotes NK:target cell interaction for an efficient lysis...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/28427232/embryonic-signature-distinguishes-pediatric-and-adult-rhabdoid-tumors-from-other-smarcb1-deficient-cancers
#8
Wilfrid Richer, Julien Masliah-Planchon, Nathalie Clement, Irene Jimenez, Laetitia Maillot, David Gentien, Benoît Albaud, Walid Chemlali, Christine Galant, Frederique Larousserie, Pascaline Boudou-Rouquette, Amaury Leruste, Celine Chauvin, Zhi Yan Han, Jean-Michel Coindre, Pascale Varlet, Paul Freneaux, Dominique Ranchère-Vince, Olivier Delattre, Franck Bourdeaut
Extra-cranial rhabdoid tumors (RT) are highly aggressive malignancies of infancy, characterized by undifferentiated histological features and loss of SMARCB1 expression. The diagnosis is all the more challenging that other poorly differentiated cancers lose SMARCB1 expression, such as epithelioid sarcomas (ES), renal medullary carcinomas (RMC) or undifferentiated chordomas (UC). Moreover, late cases occurring in adults are now increasingly reported, raising the question of differential diagnoses and emphasizing nosological issues...
March 6, 2017: Oncotarget
https://www.readbyqxmd.com/read/28426529/diagnostic-utility-of-pax8-pax2-and-ngfr-immunohistochemical-expression-in-pediatric-renal-tumors
#9
Nicoleta C Arva, Jeffrey Bonadio, Elizabeth J Perlman, Mariana M Cajaiba
Pediatric renal tumors (PRT) with small round blue or spindle cell morphology can be diagnostically challenging and only a limited number of immunohistochemical markers have been documented to help in the diagnosis: paired box (Pax) 2 and nerve growth factor receptor (NGFR) positivity have been demonstrated in Wilms tumor (WT) and clear cell sarcoma of the kidney (CCSK), respectively. However, the immunohistochemical expression of these markers in other PRT remains unknown. This study investigated Pax8, Pax2, and NGFR immunophenotype in a large series of PRT...
April 19, 2017: Applied Immunohistochemistry & Molecular Morphology: AIMM
https://www.readbyqxmd.com/read/28426528/comparative-analysis-of-multicolor-flow-cytometry-and-immunohistochemistry-for-the-detection-of-disseminated-tumor-cells
#10
Eszter Szánthó, Bettina Kárai, Gergely Ivády, Judit Bedekovics, István Szegedi, Miklós Petrás, György Ujj, Anikó Ujfalusi, Csongor Kiss, János Kappelmayer, Zsuzsanna Hevessy
Disseminating cells of a primary solid tumor may represent the origin of metastases and relapses. We aimed at comparing the diagnostic efficacy of multicolor flow cytometry (MFC) and morphology/immunohistochemistry (IHC) in the detection of disseminated tumor cells in the bone marrow (BM) and body fluids of patients with solid tumors, and in pediatric neuroblastoma cases. We investigated 72 samples retrospecively from 50 patients by MFC. Morphology/IHC data were available in 48 cases. In the first cohort, 36 samples derived from 34 patients with various forms of suspected and proven solid tumors and in the second cohort, 36 samples of 16 children with suspected and proven neuroblastoma were analyzed at diagnosis or during follow-up in a 4-color setting by MFC, and the results were compared with those obtained by IHC...
April 19, 2017: Applied Immunohistochemistry & Molecular Morphology: AIMM
https://www.readbyqxmd.com/read/28426458/anti-tnf%C3%AE-treatment-after-surgical-resection-for-crohn-s-disease-is-effective-despite-previous-pharmacodynamic-failure
#11
Amit Assa, Jiri Bronsky, Kaija-Leena Kolho, Kristyna Zarubova, Tim de Meij, Oren Ledder, Margaret Sladek, Stephanie van Biervliet, Caterina Strisciuglio, Raanan Shamir
BACKGROUND: The outcome of patients with Crohn's disease who failed anti-tumor necrosis factor alpha (anti-TNFα) therapy despite adequate serum drug levels (pharmacodynamic failure) is unclear. We aimed to assess such pediatric patients who underwent intestinal resection and were re-treated with the same anti-TNFα agent postoperatively. METHODS: Pediatric patients with Crohn's disease who underwent intestinal resection and were treated with anti-TNFα agents postoperatively were assessed retrospectively...
May 2017: Inflammatory Bowel Diseases
https://www.readbyqxmd.com/read/28426344/primary-pediatric-breast-lesions-comparing-the-prevalence-of-malignancies-in-the-middle-east-with-north-america-review-of-1031-cases
#12
Justine S Broecker, Nasim Khoshnam, Laura Thompson, Shady Anis, Nora Kamal, Scott Gillespie, Diana Kantarovich, Diana Metry, Mary Pate Mills, Rachel Drummey, Brianna Williams, Bahig M Shehata
Benign breast masses are uncommon but are becoming more recognized among the pediatric population. Malignant breast lesions are very rare. The aim of our study was to review and compare the demographics, clinical presentation, treatment, and outcomes of breast lesions, including primary malignancies, and to discuss theories that may explain why there is an increased rate of breast cancers diagnosed at a younger age in the Egyptian population. A total of 1031 cases were reviewed. Comparisons were made between the Egyptian (n = 846) and US (n = 185) cohorts...
April 2017: Fetal and Pediatric Pathology
https://www.readbyqxmd.com/read/28425203/educational-and-career-goal-attainments-in-young-adult-childhood-cancer-survivors
#13
Lisa Bashore, Emma Breyer
PURPOSE: Examine the educational and career outcomes of young adult childhood cancer survivors. DESIGN AND METHODS: Descriptive study design using a survey sent to 336 survivors. Quantitative and qualitative data were analyzed separately. RESULTS: Only 50 completed surveys were returned. Having a central nervous system tumor and radiation were associated with physical impairments. Four themes also emerged: future as medical professionals, dreams fallen short, peer relationships, and positive outlook on life...
April 2017: Journal for Specialists in Pediatric Nursing: JSPN
https://www.readbyqxmd.com/read/28423674/a-single-center-clinical-analysis-of-children-with-high-risk-neuroblastoma
#14
Xiangdong Tian, Yanna Cao, Jingfu Wang, Jie Yan, Yao Tian, Zhongyuan Li, Huijuan Wang, Xiaofeng Duan, Yan Jin, Qiang Zhao
The current multidisciplinary treatment for patients with high-risk neuroblastoma (NB) is the common census. However, protocols and opinions are different in different regions and institutions. We aimed to assess the protocol formulated by Chinese Children's Cancer Group study in 2009, and the impact of surgery extent was highlightly evaluated. METHODS: This study enrolled patients with high-risk neuroblastoma between 2009 and 2014 in Department of Pediatric Oncology of Tianjin Medical University Cancer Institute and Hospital...
March 7, 2017: Oncotarget
https://www.readbyqxmd.com/read/28423091/an-unusual-abdominal-wall-mass-in-a-child
#15
Amalia Maria do Espirito Santo Souza, Tomas Marzagão Barbuto, Flávia Alessandra Freitas, Nathalia Fernandes Vianna, Carla Maria Costa Zanchetta, Silvana Forsait, Claudio Borba, Alessandra Milani Prandini de Azambuja, Lilian Maria Cristofani, Vicente Odone
Abdominal tumors are one of the most common types of pediatric cancer. Therefore, they should always be included in the differential diagnosis of abdominal masses. Here, we present the case of a child whose initial hypothesis of diagnosis contemplated this possibility. Later, it was demonstrated that the abdominal mass found was secondary to a common parasitosis. A 2-year old, moderately malnourished and pale white boy was referred with a history of a rapidly growing, well-limited, middle abdominal mass. The mass was 10 by 3 cm, hard and poorly movable, apparently involving both abdominal rectus muscles...
April 13, 2017: Revista do Instituto de Medicina Tropical de São Paulo
https://www.readbyqxmd.com/read/28422946/pediatric-cushing-disease-disparities-in-disease-severity-and-outcomes-in-the-hispanic-and-african-american-populations
#16
Alexandra Gkourogianni, Ninet Sinaii, Sharon H Jackson, Alexander S Karageorgiadis, Charalampos Lyssikatos, Elena Belyavskaya, Margaret F Keil, Mihail Zilbermint, Prashant Chittiboina, Constantine A Stratakis, Maya B Lodish
BACKGROUND: Little is known about the contribution of racial and socioeconomic disparities to severity and outcomes for children with Cushing disease (CD). METHODS: 129 children with CD, 45 Hispanic/Latino or African American (HI/AA) and 84 non-Hispanic White (non-HW), are included. A 10-point index for rating severity (CD-severity) incorporated degree of hypercortisolemia, glucose tolerance, hypertension, anthropomorphic measurements, disease duration, and tumor characteristics...
April 19, 2017: Pediatric Research
https://www.readbyqxmd.com/read/28421251/defining-the-ultrasound-longitudinal-natural-history-of-newly-diagnosed-pediatric-small-bowel-crohn-disease-treated-with-infliximab-and-infliximab-azathioprine-combination-therapy
#17
Jonathan R Dillman, Soudabeh Fazeli Dehkordy, Ethan A Smith, Michael A DiPietro, Ramon Sanchez, Vera DeMatos-Maillard, Jeremy Adler, Bin Zhang, Andrew T Trout
BACKGROUND: Little is known about changes in the imaging appearances of the bowel and mesentery over time in either pediatric or adult patients with newly diagnosed small bowel Crohn disease treated with anti-tumor necrosis factor-alpha (anti-TNF-α) therapy. OBJECTIVE: To define how bowel ultrasound findings change over time and correlate with laboratory inflammatory markers in children who have been newly diagnosed with pediatric small bowel Crohn disease and treated with infliximab...
April 18, 2017: Pediatric Radiology
https://www.readbyqxmd.com/read/28420320/pediatric-non-vestibular-schwannoma
#18
Cory Broehm, Alyaa Al-Ibraheemi, Karen J Fritchie
While the clinicopathologic features of pediatric vestibular schwannomas, often in the context of neurofibromatosis type 2 (NF2), have been well studied, there is less data regarding the characteristics of pediatric non-vestibular schwannomas (NVS). Additionally, the rate of loss of SMARCB1/INI1 expression in this population has not been systematically evaluated. Our institutional archives were searched for cases of NVS arising in patients 18 years or younger. Clinicopathologic features including SMARCB1/INI1 status were assessed for each case...
January 1, 2017: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/28419087/novel-targeted-therapy-for-neuroblastoma-silencing-the-mxd3-gene-using-sirna
#19
Connie Duong, Sakiko Yoshida, Cathy Chen, Gustavo Barisone, Elva Diaz, Yueju Li, Laurel Beckett, Jong Chung, Reuben Antony, Jan Nolta, Nitin Nitin, Noriko Satake
BACKGROUND: Neuroblastoma is the second most common extracranial cancer in children. Current therapies for neuroblastoma, which use a combination of chemotherapy drugs, have limitations for high-risk subtypes and can cause significant long-term adverse effects in young patients. Therefore, a new therapy is needed. In this study, we investigated the transcription factor MXD3 as a potential therapeutic target in neuroblastoma. METHODS: MXD3 expression was analyzed in five neuroblastoma cell lines by immunocytochemistry and quantitative real time reverse transcription PCR and in 18 primary patient tumor samples by immunohistochemistry...
April 18, 2017: Pediatric Research
https://www.readbyqxmd.com/read/28417541/clinical-efficacy-of-cabozantinib-in-two-pediatric-patients-with-recurrent-renal-cell-carcinoma
#20
Mary Frances Wedekind, Mark Ranalli, Nilay Shah
Advanced-stage renal cell carcinoma (RCC) carries a dismal prognosis for pediatric patients with few studied therapeutic options. Cabozantinib is a small molecule tyrosine kinase inhibitor against the oncoprotein MET. It is currently approved by the U.S. Food and Drug administration for second-line treatment of RCC in adults. There is no published data on its use in children with RCC. We report here two pediatric patients with recurrent metastatic RCC whose tumors expressed MET and were treated with cabozantinib...
April 18, 2017: Pediatric Blood & Cancer
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