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ganglioneuroblastoma children

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https://www.readbyqxmd.com/read/28994241/legumain-correlates-with-neuroblastoma-differentiation-and-can-be-used-in-prodrug-design
#1
Min Zhang, Zhiteng Jiang, Sheng Chen, Zhixiang Wu, Kai Chen, Yeming Wu
Neuroblastoma (NB) is a highly malignant solid tumor in children. The cysteine endopeptidase legumain is expressed in adult solid tumors, but its expression in NB has not been examined. In this study, we assayed legumain expression in two NB cell lines and in microarrays of tumor tissues collected from 46 children with undifferentiated NB, differentiated NB and ganglioneuroblastoma. Correlation analyses showed that legumain was expressed in all NB cell lines tested, and that expression correlated with the degree of tumor differentiation...
October 10, 2017: Chemical Biology & Drug Design
https://www.readbyqxmd.com/read/28943139/diagnostic-utility-of-core-needle-biopsy-versus-open-wedge-biopsy-for-pediatric-intraabdominal-solid-tumors-results-of-a-prospective-clinical-study
#2
Scott Deeney, Camille Stewart, Amanda L Treece, Jennifer O Black, Mark A Lovell, Timothy Garrington, Frederick Karrer, Jennifer Bruny
PURPOSE: The best method for diagnosing pediatric nonnephroblastoma solid intraabdominal tumors is unknown. We hypothesized that core needle biopsy (CNB) is noninferior to open wedge biopsy (OWB) for pathologic diagnosis. METHODS: We prospectively enrolled children aged 1day to 17years with radiographic evidence of nonnephroblastoma solid intraabdominal tumors scheduled for OWB from 5/2013 to 12/2015 at a single institution. Four 16-gauge CNBs were obtained, followed by OWB...
September 4, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28845131/results-of-neuroblastoma-treatment-in-lithuania-a-single-centre-experience
#3
Austėja Juškaitė, Indrė Tamulienė, Jelena Rascon
BACKGROUND: Neuroblastoma (NB) is the most common extracranial solid tumour in children. This is a very rare disease with heterogeneous biology varying from complete spontaneous regression to a highly aggressive tumour responsible for 15% of malignancy-related death in early childhood. Analyses of survival rates in Europe have shown a considerable difference between Northern/Western and Eastern European countries. Treatment results of NB in Lithuania have never been analyzed. AIM: To assess the survival rate of children with NB according to initial spread of the disease, age at diagnosis, the MYCN amplification, risk group, and treatment period...
2017: Acta medica Lituanica
https://www.readbyqxmd.com/read/28730220/verner-morrison-syndrome-literature-review
#4
Oana Andreea Belei, Elena Rodica Heredea, Estera Boeriu, Tamara Marcela Marcovici, Simona Cerbu, Otilia Mărginean, Emil Radu Iacob, Daniela Iacob, Andrei Gheorghe Marius Motoc, Eugen Sorin Boia
Chronic diarrhea in infants is a common condition for addressability to pediatric gastroenterologists. The causes are multiple and the delay in reaching the final diagnosis can lead to complications in the general condition of the child. The purpose of this review is to present the bio-clinical and histogenetic particularities of a rare clinical entity, characterized by tumoral causes of chronic diarrhea. VIPomas are neuroendocrine tumors that autonomously secrete vasoactive intestinal peptide (VIP). Watery diarrhea, hypokalemia and achlorhydria (WDHA) syndrome caused by VIP-producing tumors only rarely occurs in adult patients with non-pancreatic disease...
2017: Romanian Journal of Morphology and Embryology, Revue Roumaine de Morphologie et Embryologie
https://www.readbyqxmd.com/read/28608056/surgical-management-and-outcomes-of-ganglioneuroma-and-ganglioneuroblastoma-intermixed
#5
Tianyou Yang, Yongbo Huang, Tao Xu, Tianbao Tan, Jiliang Yang, Jing Pan, Chao Hu, Jiahao Li, Yan Zou
PURPOSE: Clinical researches about the management and outcomes of ganglioneuroma and ganglioneuroblastoma-intermixed are limited. We report the surgical outcomes of ganglioneuroma and ganglioneuroblastoma-intermixed in a single institution. METHODS: Ganglioneuroma and ganglioneuroblastoma-intermixed diagnosed and resected between May 2009 and May 2015 in a tertiary children's hospital were retrospectively reviewed. Patients' demographic data, INSS stage, surgical complications, residual tumor size and outcomes were collected...
June 12, 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28550366/minimally-invasive-resection-of-adrenal-masses-in-infants-and-children-results-of-a-european-multi-center-survey
#6
Francesco Fascetti-Leon, Giovanni Scotton, Luca Pio, Raimundo Beltrà, Paolo Caione, Ciro Esposito, Girolamo Mattioli, Amulya K Saxena, Sabine Sarnaki, Piergiorgio Gamba
BACKGROUND: Minimal access adrenal surgery (MAAS) for adrenal pathologies is the standard for many pediatric surgical centers. However, the literature offers few reports and minimal evidence from small case series. The aim of this study was to evaluate the outcomes of pediatric MAAS through a multi-center data analysis. METHOD: Pediatric patients who underwent MAAS between January 2002 and December 2013 were retrospectively included. Data analysis was conducted using Spss software (Welch's t-test, X-square, Fisher tests, multiple regression model)...
May 26, 2017: Surgical Endoscopy
https://www.readbyqxmd.com/read/28549783/irinotecan-temozolomide-with-temsirolimus-or-dinutuximab-in-children-with-refractory-or-relapsed-neuroblastoma-cog-anbl1221-an-open-label-randomised-phase-2-trial
#7
RANDOMIZED CONTROLLED TRIAL
Rajen Mody, Arlene Naranjo, Collin Van Ryn, Alice L Yu, Wendy B London, Barry L Shulkin, Marguerite T Parisi, Sabah-E-Noor Servaes, Mitchell B Diccianni, Paul M Sondel, Julia G Bender, John M Maris, Julie R Park, Rochelle Bagatell
BACKGROUND: Outcomes for children with relapsed and refractory neuroblastoma are dismal. The combination of irinotecan and temozolomide has activity in these patients, and its acceptable toxicity profile makes it an excellent backbone for study of new agents. We aimed to test the addition of temsirolimus or dinutuximab to irinotecan-temozolomide in patients with relapsed or refractory neuroblastoma. METHODS: For this open-label, randomised, phase 2 selection design trial of the Children's Oncology Group (COG; ANBL1221), patients had to have histological verification of neuroblastoma or ganglioneuroblastoma at diagnosis or have tumour cells in bone marrow with increased urinary catecholamine concentrations at diagnosis...
July 2017: Lancet Oncology
https://www.readbyqxmd.com/read/28508462/role-of-diffusion-weighted-imaging-in-distinguishing-thoracoabdominal-neuroblastic-tumours-of-various-histological-types-and-differentiation-grades
#8
Yang Wen, Yun Peng, Xiao Min Duan, Nan Zhang
INTRODUCTION: Diffusion-weighted MR imaging (DWI) has demonstrated a great potential to help distinguish benign from malignant tumours in paediatric body. Our purpose is to evaluate whether DWI allow discrimination of thoracoabdominal neuroblastic tumours of various histological types and differentiation grades. METHODS: We retrospectively analysed DWI scans of the thoracoabdominal neuroblastic tumours in 25 children (11 girls, 14 boys). DWI was performed with two b-values of 0 and 800 s/mm(2) on a 3...
May 16, 2017: Journal of Medical Imaging and Radiation Oncology
https://www.readbyqxmd.com/read/28483228/clinical-manifestations-of-neuroblastoma-with-head-and-neck-involvement-in-children
#9
Sameer Alvi, Omar Karadaghy, Michelle Manalang, Robert Weatherly
OBJECTIVE: The purpose of our study is to review our 15-year experience with pediatric patients who have been diagnosed with neuroblastoma, and to determine their most frequent head and neck manifestations and symptoms. STUDY DESIGN: Retrospective chart review of electronic medical record. SETTING: An academic, tertiary care pediatric hospital. SUBJECTS AND METHODS: IRB approval from the Office of Research Integrity at Children's Mercy Hospital was obtained...
June 2017: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/28465480/clinical-and-biological-features-of-neuroblastic-tumors-a-comparison-of-neuroblastoma-and-ganglioneuroblastoma
#10
Wen-Guang He, Yu Yan, Wen Tang, Rong Cai, Gang Ren
Neuroblastoma (NB), ganglioneuroblastoma intermixed (GNBi) and ganglioneuroblastoma nodular (GNBn) are neuroblastic tumors that present with a wide range of symptoms and variable prognoses. We retrospectively reviewed the pretreatment clinical (age, sex and tumor stage) and biological (MYCN amplification; and levels of lactate dehydrogenase, ferritin and neuron-specific enolase) characteristics of 279 patients who were diagnosed with pathologically confirmed NB and GNB from January 2005 to December 2015. The median age at diagnosis increased with grade of differentiation (NB: 28...
June 6, 2017: Oncotarget
https://www.readbyqxmd.com/read/28409885/long-term-results-of-the-transmanubrial-osteomuscular-sparing-approach-for-pediatric-tumors
#11
Aziz El Madi, Sabine Irtan, Frédérique Sauvat, Michel Zérah, Gudrun Schleiermacher, Louise Galmiche-Roland, Véronique Minard-Colin, Hervé Brisse, Sabine Sarnacki
BACKGROUND: The complete and safe resection of pediatric cervicothoracic tumors, mostly represented by neurogenic tumors, remains a surgical challenge because of the complex anatomy of this region. The transmanubrial osteomuscular-sparing approach (TOSA) is an alternative to isolated or combined cervical and thoracic approaches enabling the control of supra-aortic vessels and nerves through the thoracic inlet. METHODS: We retrospectively reviewed the tumor characteristics, completeness of resection, morbidity, and long-term outcome of patients with cervicothoracic tumors removed by TOSA between 2000 and 2012 in our institution...
October 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28318056/cutaneous-and-systemic-findings-in-mosaic-neurofibromatosis-type-1
#12
Igor Vázquez-Osorio, Anna Duat-Rodríguez, Francisco Javier García-Martínez, Antonio Torrelo, Lucero Noguera-Morel, Angela Hernández-Martín
BACKGROUND/OBJECTIVES: Mosaic neurofibromatosis type 1 (MNF1) is a variant of neurofibromatosis type 1 (NF1) in which clinical manifestations are limited to one or several body segments. The objective was to characterize the cutaneous features and associated systemic findings in a cohort of children with MNF1. METHODS: We performed a retrospective study of 40 children diagnosed with MNF1 at the Department of Dermatology, Hospital Infantil Niño Jesús, Madrid, Spain, from January 1, 1986, to October 31, 2015...
March 20, 2017: Pediatric Dermatology
https://www.readbyqxmd.com/read/28293736/5ala-in-pediatric-brain-tumors-is-not-routinely-beneficial
#13
Jonathan Roth, Shlomi Constantini
PURPOSE: Over recent years, 5-aminoluvolinic acid (5ALA) has been increasingly used for resection guidance in adult high-grade gliomas. However, amongst pediatric patients, publication of intraoperative fluorescence has been limited, with inconsistent outcomes. We describe our experience and intraoperative finding amongst children with various brain tumors that were given 5ALA prior to tumor resection. METHODS: Since October 2014, data regarding intraoperative findings amongst children that received 5ALA prior to tumor resection were prospectively collected...
May 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/27988912/clinical-characteristics-of-adrenal-tumors-in-children-a-retrospective-review-of-a-15-year-single-center-experience
#14
Xiaokun Lin, Dazhou Wu, Congde Chen, Na Zheng
OBJECTIVE: Adrenal tumors are rare in children. The aim of this study is to review and analyze clinical data on the diagnosis and management of adrenal tumors in children. METHODS: Between 2001 and 2015, 48 pediatric patients (<14 years old) admitted to our institute with adrenal tumors were reviewed. Clinical features, imaging studies, surgical approaches, as well as pathological diagnoses were recorded. RESULTS: The series comprised 28 males and 20 females...
March 2017: International Urology and Nephrology
https://www.readbyqxmd.com/read/27984122/diagnostic-utility-of-cyclin-d1-in-the-diagnosis-of-small-round-blue-cell-tumors-in-children-and-adolescents
#15
Gaetano Magro, Lucia Salvatorelli, Rita Alaggio, Velia D'Agata, Ferdinando Nicoletti, Andrea Di Cataldo, Rosalba Parenti
Small round blue cell tumors (SRBCTs) of children and adolescents are often diagnostically challenging lesions. With the increasing diagnostic approach based on small biopsies, there is the need of specific immunomarkers that can help in the differential diagnosis among the different tumor histotypes to assure the patient a correct diagnosis for proper treatment. Based on our recent studies showing cyclin D1 overexpression in both Ewing sarcoma/primitive peripheral neuroectodermal tumor (EWS/pPNET) and peripheral neuroblastic tumors (neuroblastoma and ganglioneuroblastoma), we immunohistochemically assessed cyclin D1 immunoreactivity in 128 cases of SRBCTs in children and adolescents to establish its potential utility in the differential diagnosis...
February 2017: Human Pathology
https://www.readbyqxmd.com/read/27707529/antineuronal-nuclear-autoantibody-type-1-anti-hu-associated-opsoclonus-myoclonus-and-epilepsia-partialis-continua-case-report-and-literature-review
#16
Michael Sweeney, Matthew Sweney, M Mateo Paz Soldán, Stacey L Clardy
BACKGROUND: Opsoclonus-myoclonus syndrome is a rare clinical condition that has been associated with neuroblastoma. There are few reported examples of ANNA-1/anti-Hu antibodies in children with neuroblastoma and opsoclonus-myoclonus, all in children aged less than three years of age. METHODS: We describe the new onset of focal seizures without alteration of consciousness and opsoclonus-myoclonus in an 11-year-old girl with ANNA-1/anti-Hu positivity and a paraspinal ganglioneuroblastoma...
December 2016: Pediatric Neurology
https://www.readbyqxmd.com/read/27506465/maturation-toward-neuronal-tissue-in-a-ewing-sarcoma-of-bone-after-chemotherapy
#17
Maria Carolina Wilhelmina Salet, Rob Vogels, Paul Brons, Bart Schreuder, Uta Flucke
BACKGROUND: Ewing sarcoma is the second most common bone tumor, occurring mainly in children and young adults. It shows a typical primitive, small round cell morphology and a characteristic fusion oncogene involving EWSR1 and members of the ETS family in most of the cases. Neuronal maturation after chemotherapy is a rare phenomenon and we herein describe such an exceptional case. CASE PRESENTATION: An 8-year old boy was diagnosed with a Ewing sarcoma in the left femur...
August 9, 2016: Diagnostic Pathology
https://www.readbyqxmd.com/read/27451875/the-clinical-management-and-outcomes-of-cervical-neuroblastic-tumors
#18
Jeremy R Jackson, Hung C Tran, James E Stein, Hiroyuki Shimada, Ankur M Patel, Araz Marachelian, Eugene S Kim
BACKGROUND: Although patients with peripheral neuroblastoma (NB; pelvic and thoracic) typically have better outcomes and less aggressive disease compared with patients with abdominal disease, little has been published with regard to the management and outcomes of patients with cervical NB. Herein, we sought to determine the characteristics of cervical neuroblastic tumors and the effect of extent of resection on survival and outcomes. METHODS: We performed a retrospective review of 325 children with neuroblastic tumors at Children's Hospital Los Angeles over a 15-y period (January 1990-February 2015)...
July 2016: Journal of Surgical Research
https://www.readbyqxmd.com/read/27418194/diffusion-weighted-imaging-in-differentiating-malignant-and-benign-neuroblastic-tumors
#19
Halil Ibrahim Serin, Sureyya Burcu Gorkem, Selim Doganay, Saliha Cıracı, Ekrem Unal, Mahmut Guzel, Ahmet Kucuk, Ali Kurtsoy, Abdulhakim Coskun
PURPOSE: Our aim was to assess diffusion weighted imaging (DWI) of neuroblastic tumors and whether apparent diffusion coefficient (ADC) value may have a role in discrimination among neuroblastoma, ganglioneuroblastoma and ganglioneuroma. MATERIAL AND METHODS: The DWIs (b = 0-800 s/mm(2)) of 24 children (13 girls, 11 boys) who were diagnosed neuroblastic tumors on histopathological examination (neuroblastoma = 15, ganglioneuroblastoma = 5, ganglioneuroma = 4) were evaluated retrospectively...
September 2016: Japanese Journal of Radiology
https://www.readbyqxmd.com/read/27051319/childhood-neuroblastoma-masquerading-as-pheochromocytoma-case-report
#20
Suk-Bae Moon
Neuroblastoma is the most common extracranial solid tumor in children. Mild hypertension is a frequent symptom, presumably an effect of catecholamines that tumors release. Reported herein is the rare occurrence of severe hypertension and subsequent heart failure attributable to adrenal gland neuroblastoma. A 3-year-old boy presented with anterior chest wall protrusion. Physical examination revealed severe hypertension, and left-sided cardiac failure was evident by echocardiography. Catecholamine metabolite (norepinephrine) levels were increased in serum (>2,000 pg/mL) and in urine (1,350...
2016: International Medical Case Reports Journal
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