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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
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
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
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)
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
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
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
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
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
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
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
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
B C Rivard, D W Hague, S K Joslyn, X Zhang, S Lezmi
No abstract text is available yet for this article.
March 2016: Journal of Veterinary Internal Medicine
Paolo Fagone, Ferdinando Nicoletti, Giada Maria Vecchio, Rosalba Parenti, Gaetano Magro
Neuroblastoma is the most common extracranial solid childhood tumor, which is believed to originate from primitive neuroblasts giving rise to the sympathetic nervous system. It was previously shown that cyclin D1 (CCDN1) in pediatric neuroblastic tumors (neuroblastoma, ganglioneuroblastoma, and ganglioneuroma) recapitulates its expression during the development of peripheral sympathetic nervous system (PSNS). In the present study, we performed a microarray analysis in order to evaluate the expression of cyclin D1 in neuroblastoma as compared to ganglioneuroma and ganglioneuroblastoma...
October 2015: Acta Histochemica
Yaqiong Jin, Huanmin Wang, Wei Han, Jie Lu, Ping Chu, Shujing Han, Xin Ni, Baitang Ning, Dianke Yu, Yongli Guo
With a primary mortality, neuroblastoma (NB) is the most common extracranial solid tumor in childhood. Amplification of the MYCN (v-myc avian myelocytomatosis viral oncogene neuroblastoma derived homolog) oncogene is observed in 20-30 % of NB cases, a feature which also characterizes a highly aggressive subtype of the disease. However, the systematic study of association between single nucleotide polymorphisms (SNPs) in MYCN-regulated genes and the risk of NB has not been investigated. In the current study, we scanned a set of 16 SNPs located within known or predicted MYCN binding sites in a cohort of 247 patients of Chinese origin with neuroblastic family tumors, including neuroblastoma (NB), ganglioneuroma (GN), and ganglioneuroblastoma (GNB), and in 290 cancer-free controls to determine whether any of the tested SNPs are associated with neuroblastic family tumors...
March 2016: Tumour Biology: the Journal of the International Society for Oncodevelopmental Biology and Medicine
Baran Yılmaz, Zafer Orkun Toktaş, Akın Akakın, Mustafa Kemal Demir, Ozlem Yapıcıer, Deniz Konya
Ganglioneuroma (GN) is a benign rare tumor that originates from neural crest cells. It shares a common histogenic family with ganglioneuroblastoma and neuroblastoma and represents the most benign and differentiated form of the group. The most common locations of the GNs are the mediastinum and retroperitoneum. We report an extremely rare case of lumbar spinal GN with conus medullaris invasion and extradural paraspinal extension. A 10-year-old girl presented with a history of worsening lower-back pain and an intermittent tingling sensation in the left leg...
2015: Pediatric Neurosurgery
Wei Qiu, Ting Li, Xiao Dong Sun, Guo Yue Lv
A case of adrenal ganglioneuroblastoma is presented here. This adrenal ganglioneuroblastoma was found in a 27-year-old female 7 months after delivery. CT clarified that the tumor originated retroperitoneally and was large in size (11.4 cm × 9.4 cm). The tumor was surgically removed together with pancreatic body and tail, left kidney and spleen, and pathological diagnosis was adrenal ganglioneuroblastoma-intermixed. Adrenal ganglioneuroblastoma is extremely rare in adults, with only about 9 cases documented including this case...
October 2015: Annals of Surgical Treatment and Research
Balakrishnan Ramaswamy, Ajay M Bhandarkar, Shalini S Menon, Ashish Chandra Agarwal, Suraj S Nair
Neuroblastic tumours are common in childhood and adrenal glands are the most common site. Head and neck ganglioneuroblastomas are extremely rare and nose is a very uncommon site for a ganglioneuroblastoma. The management of this primitive sympathogonic tumour may vary depending on the age of the patient and stage of the tumour. We present a middle-aged man with a ganglioneuroblastoma of skull base, management of this tumour and a review of literature.
August 2015: Journal of Clinical and Diagnostic Research: JCDR
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