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ganglioneuroblastoma children

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 cases of the presence of ANNA-1/anti-Hu antibodies in children with neuroblastoma and opsoclonus-myoclonus, all in children aged less than 3 years. METHODS: We report 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...
September 2, 2016: Pediatric Neurology
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...
2016: Diagnostic Pathology
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
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
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
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
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
M Tulla, F Berthold, N Graf, S Rutkowski, D von Schweinitz, C Spix, P Kaatsch
BACKGROUND: Central nervous system (CNS) and non-CNS embryonal tumors occur principally in children and are rarely seen in adults. The incidence rates for rare entities such as atypical teratoid/rhabdoid tumors (AT/RT) or primitive neuroectodermal tumors in the CNS are rarely published. Incidence rates for certain subgroups, such as hepatoblastomas, have been increasing in some countries. METHODS: Data of 8337 embryonal tumors, registered in children (0-14 years) between 1991 and 2012 (for AT/RT 2000-2012) in the population-based German Childhood Cancer Registry with complete national coverage were analyzed for incidence rates, time trends, and survival...
September 2015: Pediatrics
Yu-Tong Zhang, Li-Hua Feng, Zhen Zhang, Xiao-Dan Zhong, Jian Chang
BACKGROUND: Clinical presentations of paraneoplastic syndromes in neuroblastoma may multiply. Review of the clinical data and the literature on this syndrome may help in the diagnosis of neuroblastoma. OBJECTIVES: In order to make more accurate diagnosis, we reviewed the clinical data and the literature on this syndrome. PATIENTS AND METHODS: Between April 2007 and April 2012, 68 children were diagnosed with neuroblastoma or ganglioneuroblastoma in our institution, 9 of which presented exclusively with paraneoplastic syndromes and were not treated with chemotherapy prior to diagnosis...
February 2015: Iranian Journal of Pediatrics
Wei Han, Huan-Min Wang
Neuroblastoma (NB) is the most common extracranial solid tumor in children. Diarrheal NB is quite rare and is not easy to diagnose in the early stage. Six cases of diarrheal NB in our hospital treated from 1996 to 2006 were retrospectively analyzed, including characteristics such as electrolyte imbalance, pathologic features, vasoactive intestinal peptide (VIP) immunohistochemical staining results, treatment, and prognosis. All patients were boys with 3-8 loose or watery stools each day and routine fecal tests were normal...
July 7, 2015: World Journal of Gastroenterology: WJG
Joseph G Manjaly, Victoria R C Alexander, Christopher M Pepper, Sonna N Ifeacho, Richard J Hewitt, Benjamin E J Hartley
OBJECTIVES: Ganglioneuroblastomas represent a histological subgroup of the rare neuroblastic tumours with intermediate malignant potential arising from neural crest progenitor cells of sympathetic nerves. Diagnosis can often be difficult based on imaging alone. We describe 4 cases of children presenting with a solitary neck mass with histology ultimately revealing ganglioneuroblastoma. METHODS: A retrospective case note review was carried out of all patients with cervical ganglioneuroblastoma seen at Great Ormond Street Hospital, UK...
July 2015: International Journal of Pediatric Otorhinolaryngology
Bo Zhuang, Deng-Kun Lv, Si-Ju Gao, Jing-Jing Meng
OBJECTIVE: To investigate the differential features of CT images in children with neuroblastomas (N) and ganglioneuroblastomas (G). MATERIALS AND METHODS: Clinical data of 12 children in group G and 15 in group N undergoing CT examination and definitely diagnosed by pathology were retrospectively analyzed. The focal conditions were observed and compared in the two groups, including location, size, boundaries, morphology, enhanced degree and mode, abdominal vascular involvement, presence or absence of spanning the midline, infiltration of peripheral organs, angiography manifestations in tumors or surroundings, presence or absence of calcification and vascular tumor emboli as well as metastases of distal organs and lymph nodes...
2014: Asian Pacific Journal of Cancer Prevention: APJCP
Mark A Applebaum, Tara O Henderson, Sang Mee Lee, Navin Pinto, Samuel L Volchenboum, Susan L Cohn
BACKGROUND: To investigate the incidence of second malignant neoplasms (SMN) for patients with neuroblastoma, we analyzed patients from the SEER database according to three treatment eras (Era 1: 1973-1989, Era 2: 1990-1996, and Era 3: 1997-2006) corresponding to the introduction of multi-agent chemotherapy, risk-based treatment, and stem cell transplant. PROCEDURES: The SEER database was mined for all patients with neuroblastoma or ganglioneuroblastoma. Cumulative incidence of SMN was calculated with death as a competing risk...
January 2015: Pediatric Blood & Cancer
Weihong Zhao, Qing Sun, Yao Xie, Ying Hua, Hui Xiong, Jun Jia, Xintian Lu
OBJECTIVE: To investigate the efficacy of combined modality therapy for neuroblastoma in children associated with opsoclonus-myoclonus syndrome (OMS-NB). METHOD: From May 2011 to December 2013, 6 consecutive patients (4 boys and 2 girls) diagnosed as OMS-NB underwent surgery and chemotherapy in the First Hospital, Peking University. The median age of onset was 19.5 months (range 13-24 months) and misdiagnosis occurred 7.5 months (range 2-14 months) ago. A retrospective analysis for the location, stage, pathological type, treatment way and outcome of neuroblastoma was done...
July 2014: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
Kieuhoa T Vo, Katherine K Matthay, John Neuhaus, Wendy B London, Barbara Hero, Peter F Ambros, Akira Nakagawara, Doug Miniati, Kate Wheeler, Andrew D J Pearson, Susan L Cohn, Steven G DuBois
PURPOSE: Neuroblastoma (NB) is a heterogeneous tumor arising from sympathetic tissues. The impact of primary tumor site in influencing the heterogeneity of NB remains unclear. PATIENTS AND METHODS: Children younger than age 21 years diagnosed with NB or ganglioneuroblastoma between 1990 and 2002 and with known primary site were identified from the International Neuroblastoma Risk Group database. Data were compared between sites with respect to clinical and biologic features, as well as event-free survival (EFS) and overall survival (OS)...
October 1, 2014: Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology
Y Peeters, M Van de Velde, A P Neyrinck, K Vermeylen
One lung ventilation (OLV) in children is a challenge and requires creative solutions. A case of OLV with bronchial placement of a fiberscope inspection-guided vascular embolectomy catheter in a three-year-old girl, scheduled for the resection of an intrathoracic tumor through thoracotomy is described. The availability of a broad range of vascular catheters as well as of fiberscope inspection material was decisive in managing the airway intra-operatively. Over the last 20 years, the need for OLV in children has increased, and various methods for performing it have been reported...
2014: Acta Anaesthesiologica Belgica
Dimos Karangelis, Nicolas Nikolaidis, Apostolos Roubelakis, David F Weeden
The most common malignant neurogenic tumors in children are neuroblastic tumors, classified as ganglioneuroblastoma or neuroblastoma. Ganglioneuroblastomas usually occur at the sympathetic ganglia in the mediastinum, whereas neuroblastomas occur in the abdominal cavity. We describe a case of large posterior mediastinal ganglioneuroblastoma extending from the aortic arch to the left renal hilum in a 17-year-old boy. Despite chemotherapy, post-treatment computed tomography showed disease progression. The patient underwent a thoracolaparotomy incision and excision of the tumor...
July 2014: Asian Cardiovascular & Thoracic Annals
Scott S Short, Bindi J Naik-Mathuria, Catherine J Hunter, Stephanie Papillon, James E Stein
INTRODUCTION: Data support use of video-assisted thoracoscopic surgery (VATS) for safe and effective resection of paraspinal masses in children. Our aim was to describe outcomes following this operation using a novel technique and to compare its use with that of other established techniques. SUBJECTS AND METHODS: We performed a retrospective chart review of all children (<18 years old) undergoing thoracoscopic resection of paraspinal masses in 2000-2011. Demographics, operative details, and clinical outcomes were summarized and compared between those undergoing resection using a Harmonic (Ethicon Endo-Surgery, Blue Ash, OH) blade scalpel (HBS) and those who did not...
April 2014: Journal of Laparoendoscopic & Advanced Surgical Techniques. Part A
Marcius Vinícius M Maranhão, Allana Cavalcanti Fulgino de Holanda, Felipe Lira Tavares
BACKGROUND AND OBJECTIVES: Kinsbourne syndrome is a rare neurological disorder that primarily affects children previously healthy and aged between 6 and 36 months. It is characterized by opsoclonus (rapid, irregular, horizontal and vertical eye movements) and myoclonus that may affect trunk, limbs or face, and cerebellar ataxia. It may be considered a paraneoplastic syndrome by association with neuroblastomas, hepatoblastomas and, rarely, ganglioneuromas. The aim of this paper was to present the most relevant aspects of Kinsbourne syndrome, as well as the technique used for resection of mediastinal tumor in a child with this syndrome...
May 2013: Brazilian Journal of Anesthesiology
Velia D'Angelo, Giulia Pecoraro, Paolo Indolfi, Adriana Iannotta, Vittoria Donofrio, Maria Elena Errico, Cristiana Indolfi, Maria Ramaglia, Angela Lombardi, Martina Di Martino, Vincenzo Gigantino, Alfonso Baldi, Michele Caraglia, Antonio De Luca, Fiorina Casale
Neuroblastoma (NB) is a paediatric tumor that arises from neural crest and shows heterogeneous clinical and biological features. The serine-protease high temperature requirement A1 (HtrA1) has a pivotal role in both cell proliferation and differentiation. Here we report the expression and localization of HtrA1 in NB tumor samples to assess HtrA1 role as a possible new biomarker of cellular differentiation in NB patients. HtrA1 protein expression by Western Blot assay was performed in 60 tissue samples of 50 children with NB and 10 children with ganglioneuroblastoma (GNB)...
April 2014: Journal of Neuro-oncology
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