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ganglioneuroblastoma

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https://www.readbyqxmd.com/read/28318056/cutaneous-and-systemic-findings-in-mosaic-neurofibromatosis-type-1
#1
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
#2
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...
March 14, 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/28208865/adult-neuroblastoma-case-report-and-literature-review
#3
Vishwapriya Mahadev Godkhindi, Maheboob M Basade, Kamran Khan, Kiran Thorat
Neuroblastoma is the most common solid malignant neoplasm in the paediatric age group; accounting for 7% of all childhood malignancies, but neuroblastoma in adult, is a rare occurrence, finding mention in aeons of medical literature with an overall incidence of 1 in 10 million adults/year. We report the case of a 24-year-old male patient presenting with the complaints of progressive abdominal distention of 2 months duration. Multiple Detector Computed Tomography (MD-CT) of abdomen revealed a huge enhancing mass in the retroperitoneum abutting the left kidney, and a subsequent CT-guided biopsy and immunohistochemistry confirmed the diagnosis of ganglioneuroblastoma...
December 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/27988912/clinical-characteristics-of-adrenal-tumors-in-children-a-retrospective-review-of-a-15-year-single-center-experience
#4
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...
December 17, 2016: 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
#5
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/27965907/a-rare-tumor-in-the-cervical-sympathetic-trunk-ganglioneuroblastoma
#6
Ozan Erol, Alper Koycu, Erdinc Aydin
Ganglioneuroblastoma is a rare tumor with moderate malignancy, which is composed of mature ganglion cells and seen in sympathetic ganglia and adrenal medulla. The diagnosis is possible after cytological and immunohistochemical studies following a needle biopsy or surgical excision. There is no consensus regarding the need for chemo- or radiotherapy after surgery. In this case report, clinical behavior and diagnosis and treatment of the rare tumor cervical ganglioneuroblastoma were discussed.
2016: Case Reports in Otolaryngology
https://www.readbyqxmd.com/read/27833499/anesthesia-in-a-child-with-kinsbourne-syndrome-does-anesthesia-technique-matters
#7
N Nisa, P Talawar, B Vasudevan
Kinsbourne syndrome is a rare neurological paraneoplastic syndrome associated with neuroblastic tumors. There are very few literatures on its anesthetic management and interaction with anesthetic agents. The epileptogenic potential of certain anesthetic agents such as ketamine, etomidate, and meperidine might trigger opsoclonus and myoclonus and have an impact on the long-term neurological outcome. The objective of this case report is to discuss the safety of anesthetic agents and their relationship in a patient with Kinsbourne syndrome...
October 2016: Saudi Journal of Anaesthesia
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
#8
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/27569100/role-of-radiotherapy-in-adult-ganglioneuroblastoma-and-ganglioneuroma
#9
LETTER
Yasemin Benderli Cihan, Aynur Aytekin, Talha Sarigoz
No abstract text is available yet for this article.
May 2016: Journal of B.U.ON.: Official Journal of the Balkan Union of Oncology
https://www.readbyqxmd.com/read/27526309/a-rare-case-of-ganglioneuroblastoma-encapsulated-in-pheochromocytoma
#10
Nathália Vieira Sousa, Luísa Coelho Marques de Oliveira, Paulo José Oliveira Cortez, Vitor Engrácia Valenti, David Mathew Garner, Roseane de Souza Candido Irulegui, Dalmo Antônio Ribeiro Moreira
Pheochromocytoma and Ganglioneuroblastoma are separate diseases and a rare combination in which the diagnosis can only be confirmed by pathological examination after tumor excision. We reported here a case of ganglioneuroblastoma encapsulated in pheochromocytoma. The patient is a woman, 73 years old, hypertensive, with hypothyroidism, associated for 15 years with hypercholesterolemia and hypertriglyceridemia, which had frequent complaints of low back pain. She underwent magnetic resonance and the findings were consistent with the diagnosis of pheochromocytoma...
2016: Acta Medica (Hradec Králové)
https://www.readbyqxmd.com/read/27506465/maturation-toward-neuronal-tissue-in-a-ewing-sarcoma-of-bone-after-chemotherapy
#11
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/27465021/treatment-and-outcome-of-ganglioneuroma-and-ganglioneuroblastoma-intermixed
#12
Boris Decarolis, Thorsten Simon, Barbara Krug, Ivo Leuschner, Christian Vokuhl, Peter Kaatsch, Dietrich von Schweinitz, Thomas Klingebiel, Ingo Mueller, Lothar Schweigerer, Frank Berthold, Barbara Hero
BACKGROUND: Ganglioneuroma (GN) and ganglioneuroblastoma intermixed (GNBI) are mature variants of neuroblastic tumors (NT). It is still discussed whether incomplete resection of GN/GNBI impairs the outcome of patients. METHODS: Clinical characteristics and outcome of localized GN/GNBI were retrospectively compared to localized neuroblastoma (NB) and ganglioneuroblastoma-nodular (GNBN) registered in the German neuroblastoma trials between 2000 and 2010. RESULTS: Of 808 consecutive localized NT, 162 (20 %) were classified as GN and 55 (7 %) as GNBI...
July 27, 2016: BMC Cancer
https://www.readbyqxmd.com/read/27451875/the-clinical-management-and-outcomes-of-cervical-neuroblastic-tumors
#13
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
#14
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/27203570/resolution-of-elevated-urine-glycosaminoglycans-and-clinical-features-of-mucopolysaccharidosis-after-successful-treatment-of-neuroblastoma
#15
Megan V Hilgers, Chester B Whitley, Christopher L Moertel
We report a patient with stage 3 ganglioneuroblastoma who initially presented with clinical and laboratory features consistent with mucopolysaccharidosis including coarse facial features, developmental delay, and an elevated quantitative urine glycosaminoglycan (GAG) level. All mucopolysaccharidosis features resolved following successful treatment of neuroblastoma. High GAG levels have been documented in the pediatric oncology literature, yet not as a potential marker of malignancy or other target for clinical utility...
August 2016: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/27132425/ganglioneuroblastoma-during-pregnancy-a-rare-case-report
#16
M A Moga, A Daniilidis, N F Bigiu, C Andrei, K Dinas, D G Festila
PURPOSE: To report a rare case of ganglioneuroblastoma encountered rarely in adults, especially during pregnancy. Materials and METHODS: The authors present a case of ganglioneuroblastoma relapse during the third trimester of pregnancy in a patient previously treated for ganglioneuroblastoma who had a eight-year disease-free interval. Late manifestation of neurological symptoms (vestibular syndrome, nystagmus, slightly right motor deficit) was perhaps influenced by the hormonal pregnancy effects...
2016: Clinical and Experimental Obstetrics & Gynecology
https://www.readbyqxmd.com/read/27051319/childhood-neuroblastoma-masquerading-as-pheochromocytoma-case-report
#17
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
https://www.readbyqxmd.com/read/27051110/ganglioneuroblastoma-unusual-presentation-as-a-pleural-mass-mimicking-mesothelioma
#18
Bhawna Bhutoria Jain, Sanchita Ghosh, Murari Mohan Das, Sarbani Chattopadhyay
Ganglioneuroblastoma (GNB) is a rare peripheral neuroblastic tumor that is derived from developing neuronal cells of the sympathetic nervous system, and usually occurs in young children. We present a case of GNB occurring as pleural mass in a 2-year-old boy, which led to diagnostic confusion. On fine-needle aspiration cytology (FNAC), it was misinterpreted as mesothelioma. He underwent thoracotomy with excision of the mass. Histopathological findings showed features of a biphasic tumor suggestive of mesothelioma...
March 2016: Lung India: Official Organ of Indian Chest Society
https://www.readbyqxmd.com/read/27014421/expression-profile-of-wilms-tumor-1-wt1-isoforms-in-undifferentiated-and-all-trans-retinoic-acid-differentiated-neuroblastoma-cells
#19
Grazia Maugeri, Agata Grazia D'Amico, Daniela Maria Rasà, Rita Reitano, Salvatore Saccone, Concetta Federico, Rosalba Parenti, Gaetano Magro, Velia D'Agata
Wilms tumor 1 gene (WT1) is a tumor suppressor gene originally identified in nephroblastoma. It is also expressed in neuroblastoma which represents the most aggressive extracranial pediatric tumor. Many evidences have shown that neuroblastoma may undergo maturation, by transforming itself in a more differentiated tumors such as ganglioneuroblastoma and ganglioneuroma, or progressing into a highly aggressive metastatic malignancy. To date, 13 WT1 mRNA alternative splice variants have been identified. However, most of the studies have focused their attention only on isoform of ∼49 kDa...
January 2016: Genes & Cancer
https://www.readbyqxmd.com/read/26872011/frequent-hras-mutations-in-malignant-ectomesenchymoma-overlapping-genetic-abnormalities-with-embryonal-rhabdomyosarcoma
#20
Shih-Chiang Huang, Rita Alaggio, Yun-Shao Sung, Chun-Liang Chen, Lei Zhang, Yu-Chien Kao, Narasimhan P Agaram, Leonard H Wexler, Cristina R Antonescu
Malignant ectomesenchymoma (MEM) is an exceedingly rare pediatric sarcoma with a predilection for infants and young children and is composed of dual malignant mesenchymal and neuroectodermal components. Microscopically, MEM displays areas of rhabdomyosarcoma (RMS) with intermixed neuronal/neuroblastic foci. The molecular alterations associated with MEM and its relationship with embryonal RMS (ERMS) and malignant peripheral nerve sheath tumor (MPNST) have not yet been elucidated. In this study we used whole-transcriptome sequencing in 2 MEM index cases with available frozen tissue, followed by screening of the identified genetic abnormalities in 5 additional cases...
July 2016: American Journal of Surgical Pathology
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