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Ganglioneuromas

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https://www.readbyqxmd.com/read/28382483/images-in-endocrine-pathology-unique-composite-adrenal-adenomatoid-tumor-ganglioneuroma-myelolipoma-and-cortical-nodular-hyperplasia
#1
Eleonora Duregon, Marco Volante, Stefano Guzzetti, Ida Rapa, Simona Vatrano, Mauro Papotti
No abstract text is available yet for this article.
April 5, 2017: Endocrine Pathology
https://www.readbyqxmd.com/read/28382275/harlequin-syndrome-following-resection-of-mediastinal-ganglioneuroma
#2
Yeong Jeong Jeon, Jongbae Son, Jong Ho Cho
Harlequin syndrome is a rare disorder of the sympathetic nervous system characterized by unilateral facial flushing and sweating. Although its etiology is unknown, this syndrome appears to be a dysfunction of the autonomic nervous system. To the best of our knowledge, thus far, very few reports on perioperative Harlequin syndrome after thoracic surgery have been published in the thoracic surgical literature. Here, we present the case of a 6-year-old patient who developed this unusual syndrome following the resection of a posterior mediastinal mass...
April 2017: Korean Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28374498/utility-of-gata3-in-the-differential-diagnosis-of-pheochromocytoma
#3
Carmen M Perrino, Alex Ho, Christopher P Dall, Debra L Zynger
AIMS: GATA3 is a relatively new immunohistochemical marker which shows consistent nuclear expression in a variety of tumors, including breast and urothelial carcinoma. The staining pattern of GATA3 in adrenal lesions is not well established. We aim to describe the expression of GATA3 in adrenal tumors and determine if there is differential staining between pheochromocytoma and adrenal cortical carcinoma. METHODS AND RESULTS: A retrospective search was performed to identify 74 adrenal lesions which were immunohistochemically tested for GATA3 expression...
April 4, 2017: Histopathology
https://www.readbyqxmd.com/read/28302232/giant-ganglioneuroma-in-a-5-year-child
#4
Fazal Wahab Khan, Saulat Hasnain Fatimi, Hamza Abdur Rahim Khan
We report a 5-year boy presenting with pain in the lower chest and upper abdomen. On evaluation with computed tomography scan of the chest, he was found to have a large 16 x 14 cm posterior mediastinal mass compressing the inferior vena cava and liver, and shifting the heart to the opposite side. Tumor was approached via right postero-lateral thoracotomy and dissected off from esophagus, heart, inferior vena cava and lungs. The entire tumor was resected without any complications. Postoperative course was unremarkable...
March 2017: Journal of the College of Physicians and Surgeons—Pakistan: JCPSP
https://www.readbyqxmd.com/read/28291514/synchronous-ganglioneuroma-and-schwannoma-of-the-vagal-inferior-ganglion
#5
Shaolong Yang, Danhui Zhao, Jie Wei, Peifeng Li
Neurogenic neoplasms resulting from autonomic nerves are considerably rare. In this paper, we report a case of a 41-year-old woman with composite tumor of synchronous ganglioneuroma and schwannoma in the vagal inferior ganglion. Ultrasonography and computed tomography showed a well-defined mass, which extruded from the internal and external carotid arteries. Two tumors were closely attached but with an evident boundary. The small tumor was composed of spindle cells and numerous mature ganglion cells, and the large one consisted entirely of differentiated neoplastic Schwann cells...
March 14, 2017: Clinical Neuropathology
https://www.readbyqxmd.com/read/28285811/-composite-pheochromocytoma-a-rare-adrenal-tumor
#6
Gwladys Robinet, Nathalie Rioux-Leclercq, Andréa Manunta, Romain Mathieu, Frédérique Tissier, Benoit Peyronnet, Solène-Florence Kammerer-Jacquet
INTRODUCTION: Composite pheochromocytoma is a rare tumor of the adrenal medulla composed of pheochromocytoma and neuroblastic tumor. We report the case of a composite pheochromocytoma detected in a patient with neurofibromatosis type 1. CASE REPORT: A 61-year-old male patient presented occasional sweats with palpitation and moderate high blood pressure. Urinary catecholamine level was increased. CT scan showed a heterogeneous tumor limited to the adrenal gland. Histologically, the tumor showed two components: pheochromocytoma and ganglioneuroma and was diagnosed as a composite pheochromocytoma...
April 2017: Annales de Pathologie
https://www.readbyqxmd.com/read/28246490/cystic-adrenal-lesions-focus-on-pediatric-population-a-review
#7
REVIEW
Mara Carsote, Adina Ghemigian, Dana Terzea, Ancuta Augustina Gheorghisan-Galateanu, Ana Valea
BACKGROUND AND AIM: The cysts may potentially affect any organ; adrenals cysts are rare. This is a review of the literature regarding adrenal cysts, focusing on children and young adults. GENERAL DATA: Three major types have been described: pure cysts (endothelial, epithelial, and hemorrhagic or pseudocyst), parasitic (as hydatid) cysts and cystic part of a tumour (most frequent are neuroblastoma, ganglioneuroma, pheocromocytoma, and teratoma). The complications are: bleeding, local pressure effects; infection; rupture (including post-traumatic); arterial hypertension due to renal vessels compression...
2017: Clujul Medical (1957)
https://www.readbyqxmd.com/read/28044305/clinicopathological-analysis-of-mediastinal-masses-a-mixed-bag-of-non-neoplastic-and-neoplastic-etiologies
#8
Preeti Sharma, Vidya Jha, Naveen Kumar, Rohit Kumar, Ashish Mandal
OBJECTIVE: The mediastinum is the central portion of the thoracic cavity, housing numerous organs and harbouring a mixed bag of non-neoplastic and neoplastic lesions. Accurate diagnosis is essential owing to the widely variable therapeutic and prognostic implications. MATERIAL AND METHOD: Cases of mediastinal masses were retrospectively reviewed from January 2011 till January 2016. Clinico-radiological records of these cases were retrieved. Fine needle aspiration cytology (FNAC) was performed wherever feasible...
2017: Türk Patoloji Dergisi
https://www.readbyqxmd.com/read/28043883/trigeminal-ganglioneuroma-a-rare-case-of-trigeminal-neuralgia-caused-by-cerebellopontine-angle-tumor
#9
Nadeem Khan, Alex Michael, Ali Choucair, Esther Bit-Ivan
BACKGROUND: Intracranial ganglioneuromas are very rare benign tumors of neural crest origin and generally arise from the peripheral nervous system or adrenal glands. Very few cases of intracranial ganglioneuroma arising from the trigeminal nerve have been reported in the literature, all in East Asia. CASE DESCRIPTION: A 52-year-old male presented to his primary care physician for evaluation of right facial and periorbital pain for over 18 months. He also reported a two-week history of diplopia with right lateral gaze, which resolved spontaneously...
March 2017: World Neurosurgery
https://www.readbyqxmd.com/read/27994400/in-vivo-confocal-microscopic-architecture-of-corneal-nerves-in-a-case-of-multiple-endocrine-neoplasia-type-2b
#10
Chintan Malhotra, Arun Kumar Jain, Bikram Thapa, Sabin Sahu
A detailed ocular examination and in vivo confocal microscopy (IVCM) using the Heidelberg retinal tomograph 3 with Rostock cornea module were performed in a patient with multiple endocrine neoplasia (MEN) 2b syndrome. Ocular findings included ptosis secondary to thickening of the lid margins, subconjunctival and perilimbal neuromas, and prominent corneal nerves extending up to the pupillary area. IVCM demonstrated structural alterations of both the main nerve trunks and the smaller branches. The main nerve trunks were grossly thickened while the smaller fibers were present in an intertwining manner in the anterior stroma...
October 2016: Middle East African Journal of Ophthalmology
https://www.readbyqxmd.com/read/27988912/clinical-characteristics-of-adrenal-tumors-in-children-a-retrospective-review-of-a-15-year-single-center-experience
#11
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/27978508/targeted-next-generation-sequencing-of-pediatric-neuro-oncology-patients-improves-diagnosis-identifies-pathogenic-germline-mutations-and-directs-targeted-therapy
#12
Cassie N Kline, Nancy M Joseph, James P Grenert, Jessica van Ziffle, Eric Talevich, Courtney Onodera, Mariam Aboian, Soonmee Cha, David R Raleigh, Steve Braunstein, Joseph Torkildson, David Samuel, Michelle Bloomer, Alejandra G de Alba Campomanes, Anuradha Banerjee, Nicholas Butowski, Corey Raffel, Tarik Tihan, Andrew W Bollen, Joanna J Phillips, W Michael Korn, Iwei Yeh, Boris C Bastian, Nalin Gupta, Sabine Mueller, Arie Perry, Theodore Nicolaides, David A Solomon
BACKGROUND: Molecular profiling is revolutionizing cancer diagnostics and leading to personalized therapeutic approaches. Herein we describe our clinical experience performing targeted sequencing for 31 pediatric neuro-oncology patients. METHODS: We sequenced 510 cancer-associated genes from tumor and peripheral blood to identify germline and somatic mutations, structural variants, and copy number changes. RESULTS: Genomic profiling was performed on 31 patients with tumors including 11 high-grade gliomas, 8 medulloblastomas, 6 low-grade gliomas, 1 embryonal tumor with multilayered rosettes, 1 pineoblastoma, 1 uveal ganglioneuroma, 1 choroid plexus carcinoma, 1 chordoma, and 1 high-grade neuroepithelial tumor...
November 14, 2016: Neuro-oncology
https://www.readbyqxmd.com/read/27916824/graphene-oxide-nanoribbons-induce-autophagic-vacuoles-in-neuroblastoma-cell-lines
#13
Emanuela Mari, Stefania Mardente, Emanuela Morgante, Marco Tafani, Emanuela Lococo, Flavia Fico, Federica Valentini, Alessandra Zicari
Since graphene nanoparticles are attracting increasing interest in relation to medical applications, it is important to understand their potential effects on humans. In the present study, we prepared graphene oxide (GO) nanoribbons by oxidative unzipping of single-wall carbon nanotubes (SWCNTs) and analyzed their toxicity in two human neuroblastoma cell lines. Neuroblastoma is the most common solid neoplasia in children. The hallmark of these tumors is the high number of different clinical variables, ranging from highly metastatic, rapid progression and resistance to therapy to spontaneous regression or change into benign ganglioneuromas...
November 29, 2016: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/27888390/what-do-we-need-to-know-about-colonic-polypoid-ganglioneuroma-a-case-report-and-a-comprehensive-review
#14
Mohamed Abdelfatah, George Sangah, Glenn Harvin
No abstract text is available yet for this article.
November 26, 2016: Journal of Gastrointestinal Cancer
https://www.readbyqxmd.com/read/27876089/rapid-onset-obesity-hypoventilation-hypothalamic-dysfunction-autonomic-dysregulation-and-neuroendocrine-tumor-syndrome-with-a-homogenous-enlargement-of-the-pituitary-gland-a-case-report
#15
Lama Aljabban, Lina Kassab, Nour Alhuda Bakoura, Mohammad Fayez Alsalka, Ismaeil Maksoud
BACKGROUND: Rapid-onset obesity with hypoventilation, hypothalamic dysfunction, and autonomic dysregulation syndrome is a rare pediatric disorder with a variable sequence of clinical presentations, undefined etiology, and high risk of mortality. Our patient presented an unusual course of the disease accompanied by a homogenous mild enlargement of her pituitary gland with an intact pituitary-endocrine axis which, to the best of our knowledge, represents a new finding in rapid-onset obesity with hypoventilation, hypothalamic dysfunction, and autonomic dysregulation syndrome...
November 22, 2016: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/27873762/ganglioneuroma-of-the-pancreas-in-a-4-year-old-girl
#16
REVIEW
Naruhiko Ikoma, Juan A Santamaria-Barria, Curtis Wray, KuoJen Tsao
Ganglioneuroma (GN) is the uncommon, benign representative of the peripheral neuroblastic tumours (PNTs), which arise from primitive sympathetic ganglion cells. PNTs comprise one of the most common groups of neoplastic diseases in infants and children, but its occurrence in the pancreas is rare. We report a 4-year-old girl with GN of the pancreas requiring pancreaticoduodenectomy as a definitive therapy and with a great outcome, and we review the published literature.
November 14, 2016: BMJ Case Reports
https://www.readbyqxmd.com/read/27862333/genetic-variants-in-the-promoter-region-of-the-calcium-sensing-receptor-gene-are-associated-with-its-down-regulation-in-neuroblastic-tumors
#17
Laia Masvidal, Raquel Iniesta, Marta García, Carla Casalà, Cinzia Lavarino, Jaume Mora, Carmen de Torres
We have previously reported that the calcium-sensing receptor (CaSR) is expressed in benign, differentiated neuroblastic tumors, and epigenetically silenced in undifferentiated, malignant cases. Furthermore, cinacalcet, an allosteric activator of the CaSR, reduces neuroblastoma tumor growth in preclinical models. However, to identify patients that might benefit from this treatment, a complete understanding of mechanisms governing CaSR expression in these tumors would be required. We have now analyzed two polymorphisms in the promoter region of the CASR gene (rs7652589 and rs1501899) by allelic discrimination in neuroblastoma patients and cell lines...
November 15, 2016: Molecular Carcinogenesis
https://www.readbyqxmd.com/read/27813619/extradural-ganglioneuroma-with-t1-t2-involvement-mimicking-spondylodiscitis-a-case-report-and-a-review-of-the-literature
#18
REVIEW
Mehdi Mogharrabi, Ramin Azarhoush, Hamid Javadi, Elahe Pirayesh, Majid Assadi
Ganglioneuroma (GN) is a rare benign neural tumor, usually derived from the ganglia of the sympathetic system. This report describes a 36-year-old man who presented with back pain and local tenderness that closely mimicked the clinical and ima-ging findings of spondylodiskitis. However, histologic examination made the diagnosis of GN. To our knowledge, this is the first report presenting the pattern of a GN as a differential diagnosis of spondylodiskitis.
2016: Nuclear Medicine Review. Central & Eastern Europe
https://www.readbyqxmd.com/read/27704704/composite-paraganglioma-ganglioneuroma-concomitant-with-adrenal-metastasis-of-medullary-thyroid-carcinoma-in-a-patient-with-multiple-endocrine-neoplasia-type-2b-a-case-report
#19
Mutsushi Yamasaki, Yoshiyasu Sato, Takeo Nomura, Fuminori Sato, Shinya Uchino, Hiromitsu Mimata
Multiple endocrine neoplasia type 2 (MEN2) is an autosomal-dominant cancer syndrome with major components of medullary thyroid carcinoma, pheochromocytoma, and hyperparathyroidism. MEN2B is the most aggressive and rarest of the MEN2 variants. Pheochromocytoma in MEN2 is virtually always located in the adrenal medulla, but MEN2-associated extra-adrenal pheochromocytomas (paraganglioma) are rare. A 59-year-old man who has been diagnosed with MEN2B consulted our hospital for surgical treatment of a 10-mm left adrenal mass and a 30-mm retroperitoneal mass...
February 2017: Asian Journal of Endoscopic Surgery
https://www.readbyqxmd.com/read/27668117/organ-preservation-in-a-case-of-retroperitoneal-ganglioneuroma-a-case-report-and-review-of-literature
#20
Santosh Kumar, Shivanshu Singh, Abhishek Chandna
The retroperitoneum is a closed space harbouring vital organs including the great vessels, kidneys and adrenal glands, ureters, and the ascending and descending colon. Surgical management of retroperitoneal pathologies may need multiorgan resection in order to achieve complete surgical resection while preservation of surrounding organs should be attempted, especially in case of benign tumors. We present a case of 15-year-old girl with an 11 × 6 × 5 cm retroperitoneal ganglioneuroma displacing the right kidney, renal vein, and ureter and abutting the IVC which was excised in toto preserving the right kidney and ureter with careful dissection around the great vessels...
2016: Case Reports in Surgery
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