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https://www.readbyqxmd.com/read/29103425/cancer-and-autoimmunity-paraneoplastic-neurological-disorders-associated-with-neuroblastic-tumors
#1
Wendy G Mitchell, Franz Blaes
Cancer and autoimmunity come together in paraneoplastic syndromes (PNS), which reflect the remote, not direct, effects of cancer. In the pediatric population, a variety of PNS have been described, but the most common of these rare disorders are instigated by neuroblastic tumors, such as neuroblastoma, ganglioneuroblastoma, and ganglioneuroma. The main pediatric-onset neurological PNS are ROHHAD syndrome, anti-ANNA1 (anti-Hu), and opsoclonus-myoclonus syndrome. They manifest distinctive neurological features, which aid the diagnosis, though under-recognition still poses serious challenges and risks...
August 2017: Seminars in Pediatric Neurology
https://www.readbyqxmd.com/read/28994241/legumain-correlates-with-neuroblastoma-differentiation-and-can-be-used-in-prodrug-design
#2
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/28986989/utility-of-phox2b-immunohistochemical-stain-in-neural-crest-tumors-and-non-neural-crest-tumors-in-pediatric-patients
#3
Mikako Warren, Ryosuke Matsuno, Henry Tran, Hiroyuki Shimada
BACKGROUND: This study evaluated the utility of Phox2b in pediatric tumors. Previously tyrosine hydroxylase (TH) was the most widely utilized sympathoadrenal marker specific for neural crest tumors with neuronal/neuroendocrine differentiation. However, its sensitivity is insufficient. Recently Phox2b has emerged as another specific marker for this entity. METHODS: Phox2b IHC was performed on 159 pediatric tumors including (1) 65 neural crest tumors with neuronal differentiation [peripheral neuroblastic tumors (pNT)]: 15 neuroblastoma undifferentiated (NB-UD), 10 NB poorly differentiated (NB-PD), 10 NB differentiating (NB-D), 10 ganglioneuroblastoma intermixed (GNBi), 10 GNB nodular (GNBn), and 10 ganglioneuroma (GN); (2) 23 neural crest tumors with neuroendocrine differentiation [pheochromocytoma/paraganglioma (PCC/PG)]; (3) 27 other neural crest tumors including one composite rhabdomyosarcoma/neuroblastoma; and (4) 44 non-neural crest tumors...
October 7, 2017: Histopathology
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
#4
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/28922691/adrenal-ganglioneuroblastoma-in-an-adult
#5
James Lonie, Rachael Boles, Jason Boldery
No abstract text is available yet for this article.
September 18, 2017: ANZ Journal of Surgery
https://www.readbyqxmd.com/read/28914675/ganglioneuroblastoma-arising-in-an-ovarian-dermoid-cyst-first-report-in-the-literature
#6
Simon Rajendran, Debra Jensen, W Glenn McCluggage
The development of a somatic neoplasm within an ovarian dermoid cyst (mature cystic teratoma) is a rare, but well described, phenomenon which occurs in approximately 1% of all cases. Any of the tissue components of a dermoid cyst has the potential to undergo neoplastic transformation with carcinoid tumors and squamous cell carcinomas being among the most common neoplasms. We report a case of a ganglioneuroblastoma arising within an ovarian dermoid cyst, an association which, as far as we are aware, has not been described previously...
September 29, 2017: International Journal of Gynecological Pathology
https://www.readbyqxmd.com/read/28845131/results-of-neuroblastoma-treatment-in-lithuania-a-single-centre-experience
#7
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/28806253/variable-mibg-activity-in-the-same-renal-cyst
#8
Xiaofei Zhao, Hongming Zhuang
A series of I-MIBG scanning was performed in a 33-year-old woman with a history of ganglioneuroblastoma and a known left renal cyst. The initial studies did not reveal any MIBG activity in the renal cyst. However, increased MIBG activity was noted in the same renal cyst in the subsequent follow-up scans.
August 12, 2017: Clinical Nuclear Medicine
https://www.readbyqxmd.com/read/28752484/composite-pheochromocytoma-paraganglioma-ganglioneuroma-a-clinicopathologic-study-of-eight-cases-with-analysis-of-succinate-dehydrogenase
#9
Sounak Gupta, Jun Zhang, Lori A Erickson
Ganglioneuromas represent the most well-differentiated spectrum of neoplasia arising from the sympathetic nervous system, while neuroblastomas represent the most poorly differentiated counterpart, and ganglioneuroblastomas represent intermediate stages of differentiation. Small series of cases have documented the co-occurrence of ganglioneuroma with a pheochromocytoma (Pheo)/paraganglioma (PGL) component. We report the clinicopathologic features of eight such cases, diagnosed between 2003 and 2015 with a mean follow-up of 22 months (1-47), which were evaluated for syndrome associations, SDHB expression, and clinical outcome...
July 27, 2017: Endocrine Pathology
https://www.readbyqxmd.com/read/28744687/lin28a-a-sensitive-immunohistochemical-marker-for-embryonal-tumor-with-multilayered-rosettes-etmr-is-also-positive-in-a-subset-of-atypical-teratoid-rhabdoid-tumor-at-rt
#10
Shilpa Rao, R T Rajeswarie, T Chickabasaviah Yasha, Bevinahalli N Nandeesh, Arimappamagan Arivazhagan, Vani Santosh
INTRODUCTION: CNS embryonal tumors comprise a group of highly malignant neoplasms with a wide spectrum of histomorphological entities that includes Medulloblastoma (MB), Atypical Teratoid/Rhabdoid Tumor (AT/RT), Neuroblastoma (NB), Ganglioneuroblastoma (GNB), Embryonal Tumor with Multilayered Rosettes (ETMR), and the embryonal tumor-Not Otherwise Specified (NOS). The entity ETMR includes previously described histopathologic patterns-Embryonal Tumor with Abundant Neuropil and True Rosettes (ETANTR), Ependymoblastoma (EBL), and Medulloepithelioma (MEPL)...
November 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/28730220/verner-morrison-syndrome-literature-review
#11
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/28713603/adrenal-ganglioneuroblastoma-in-adults-a-case-report-and-review-of-the-literature
#12
Stefano Benedini, Giorgia Grassi, Carmen Aresta, Antonietta Tufano, Luca Fabio Carmignani, Barbara Rubino, Livio Luzi, Sabrina Corbetta
Incidentally discovered adrenal masses are very common given the increased number of imaging studies performed in recent years. We here report a clinical case of a 20-year-old woman who presented with left flank pain. Ultrasound examination revealed a contralateral adrenal mass, which was confirmed at computed tomography (CT) scan. Hormonal hypersecretion was excluded. Given the size (11 × 10 × 7 cm) and the uncertain nature of the mass, it was surgically removed and sent for pathological analyses. Conclusive diagnosis was ganglioneuroblastoma...
2017: Case Reports in Endocrinology
https://www.readbyqxmd.com/read/28630389/a-case-report-of-the-first-ganglioneuroblastoma-of-the-parotid-gland-of-an-the-adult
#13
Aldo Bove, Rossana Percario, Andrea De Carlo, Barbara Zappacosta
The ganglioneuroblastoma is a rare form of pediatric tumor, involving innerved tissues of the nervous sympathetic system, whose evolution is very hard to foresee. A 38 year-old patient whose histologic exam unexpectedly showed ganglioneuroblastoma, underwent a parotidectomy. Three years after surgery the patient is in optimal clinical conditions, without any radiological signs or clinical relapses. KEY WORDS: Ganglioneuroblastoma, Parotid gland, Parodidectomy.
May 26, 2017: Annali Italiani di Chirurgia
https://www.readbyqxmd.com/read/28608056/surgical-management-and-outcomes-of-ganglioneuroma-and-ganglioneuroblastoma-intermixed
#14
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
#15
Francesco Fascetti-Leon, Giovanni Scotton, Luca Pio, Raimundo Beltrà, Paolo Caione, Ciro Esposito, Girolamo Mattioli, Amulya K Saxena, Sabine Sarnacki, 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)...
November 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
#16
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/28511265/diagnostic-value-of-diffusion-weighted-mri-for-tumor-characterization-differentiation-and-monitoring-in-pediatric-patients-with-neuroblastic-tumors
#17
Henning Neubauer, Mengxia Li, Verena Rabea Müller, Thomas Pabst, Meinrad Beer
Purpose We explored the diagnostic value of diffusion-weighted MRI (DWI) for tumor characterization, differentiation and therapy monitoring in pediatric patients with extracranial neuroblastic tumors. Materials and Methods All 29 patients (14 girls, median age: 3 years) with neuroblastoma (NB, n = 19), ganglioneuroblastoma (GNB, n = 4) and ganglioneuroma (GN, n = 6) who had had at least one in-house DWI examination since 2005 were identified and retrospectively analyzed. Two independent blinded readers measured ADC values (unit: 10-3 mm(2)/s) and signal intensity ratios (SIRs) of the primary tumor and, if applicable, of the tumor after chemotherapy, metastases and tumor relapse...
July 2017: RöFo: Fortschritte Auf Dem Gebiete der Röntgenstrahlen und der Nuklearmedizin
https://www.readbyqxmd.com/read/28508462/role-of-diffusion-weighted-imaging-in-distinguishing-thoracoabdominal-neuroblastic-tumours-of-various-histological-types-and-differentiation-grades
#18
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
#19
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/28477311/update-on-adrenal-tumours-in-2017-world-health-organization-who-of-endocrine-tumours
#20
REVIEW
Alfred King-Yin Lam
The fourth edition of the World Health Organization (WHO) classification of endocrine tumours contains substantial new findings for the adrenal tumours. The tumours are presented in two chapters labelled as "Tumours of the adrenal cortex" and "Tumours of the adrenal medulla and extra-adrenal paraganglia." Tumours of the adrenal cortex are classified as cortical carcinoma, cortical adenoma, sex cord stromal tumours, adenomatoid tumour, mesenchymal and stromal tumours (myelolipoma and schwannoma), haematological tumours, and secondary tumours...
May 6, 2017: Endocrine Pathology
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