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Ganglioneuromas

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https://www.readbyqxmd.com/read/28760150/bilateral-pheochromocytoma-with-ganglioneuroma-component-associated-with-multiple-neuroendocrine-neoplasia-type-2a-a-case-report
#1
Boubacar Efared, Gabrielle Atsame-Ebang, Soufiane Tahirou, Khalid Mazaz, Nawal Hammas, Hinde El Fatemi, Laila Chbani
BACKGROUND: Composite pheochromocytoma/paragangliomas are very rare tumors composed of ordinary pheochromocytoma paragangliomas associated with neurogenic tumors. Several hereditary susceptibility disorders are known to be associated with pheochromocytoma/paragangliomas such as multiple endocrine neoplasia type 2 (2A or B). To the best of our knowledge, only four cases of composite pheochromocytoma/paragangliomas associated with multiple endocrine neoplasia type 2 have been reported. CASE PRESENTATION: A 40-year-old Arabic woman presented with headache, palpitations, paroxysmal hypertension, and weight loss, which she had had for the last 3 years...
August 1, 2017: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/28752484/composite-pheochromocytoma-paraganglioma-ganglioneuroma-a-clinicopathologic-study-of-eight-cases-with-analysis-of-succinate-dehydrogenase
#2
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/28748502/case-report-robot-assisted-laparoscopic-excision-of-giant-ganglioneuroma-of-the-adrenal-gland
#3
Priyank Shah, N Ragavan, Seetharam Bhat, Kunal Dholakiya
No abstract text is available yet for this article.
July 26, 2017: Journal of Robotic Surgery
https://www.readbyqxmd.com/read/28738920/excision-of-a-presacral-ganglioneuroma-in-a-young-man
#4
Eileen A O'Halloran, Xianzhong Ding, Dana M Hayden, Theodore J Saclarides, Joshua M Eberhardt
No abstract text is available yet for this article.
July 1, 2017: American Surgeon
https://www.readbyqxmd.com/read/28733979/novel-clinical-and-molecular-findings-in-spanish-patients-with-nevoid-basal-cell-carcinoma-syndrome
#5
N Alonso, J Cañueto, S Ciria, E Bueno, I Palacios-Alvarez, M Alegre, C Badenas, A Barreiro, L Pena, C Maldonado, M V Nespeira-Jato, C Peña-Penabad, A Azon, M Gavrilova, I Ferrer, O Sanmartin, L Robles, A Hernandez, M Urioste, S Puig, L Puig, R Gonzalez-Sarmiento
Nevoid Basal Cell Carcinoma Syndrome (NBCCS) is an autosomal dominant disorder characterised by developmental alterations and multiple basal cell carcinomas. Mutations in PTCH1, a membrane receptor for Sonic Hedgehog, are associated with the development of the disease. Most of them produce a truncated protein which is unable to supress Smoothened protein and continuously activates the downstream pathway OBJECTIVES: We aimed to characterize 22 unrelated Spanish subjects with NBCCS, the largest cohort of Gorlin syndrome reported to date in Spain METHODS AND RESULTS: We reported for the first time two young patients with uterus didelphys and ganglioneuroma, within the context of NBCCS...
July 22, 2017: British Journal of Dermatology
https://www.readbyqxmd.com/read/28730220/verner-morrison-syndrome-literature-review
#6
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/28700440/de-novo-tumors-of-teratoma-ganglioneuroma-arising-from-a-mature-cystic-teratoma-of-the-ovary
#7
Shannon Coy, Emily Meserve, Ross Berkowitz, Michelle S Hirsch
Mature teratomas are the most common ovarian neoplasms, accounting for 40% to 50% of ovarian tumors, and are histologically defined by the presence of multiple lineages of mature differentiated cells derived from one or more of the 3 embryonic germ layers; ectoderm, mesoderm, and endoderm. Neuroectodermal and neural crest differentiation can be observed in mature teratomas, but it is uncommon to find secondary tumors that arise from the neural crest lineage. Herein we report the uncommon finding of a ganglioneuroma arising in a mature cystic teratoma in a 26-yr-old woman...
July 11, 2017: International Journal of Gynecological Pathology
https://www.readbyqxmd.com/read/28693694/trigeminal-ganglioneuroma-in-the-middle-posterior-cranial-fossa-a-case-report
#8
Ting Wang, Lin Ma, Xin Lou, Bo Bu
No abstract text is available yet for this article.
June 10, 2017: Chinese Medical Sciences Journal, Chung-kuo i Hsüeh K'o Hsüeh Tsa Chih
https://www.readbyqxmd.com/read/28608056/surgical-management-and-outcomes-of-ganglioneuroma-and-ganglioneuroblastoma-intermixed
#9
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/28607627/retroperitoneal-ganglioneuroma-mimicking-a-kidney-tumor-case-report
#10
Agnieszka Dąbrowska-Thing, Wojciech Rogowski, Ryszard Pacho, Ewa Nawrocka-Laskus, Żaneta Nitek
BACKGROUND: Ganglioneuroma (GN) is a rare benign tumor arising from the neural crest cells. The reported incidence of GN is one per million population. As a primary retroperitoneal tumor, it constitutes only a small percentage of 0.72 to 1.6%. GN can arise de novo or as a result of maturation of a neuroblastoma either spontaneously or after chemotherapy. The most common location is the posterior paraspinal mediastinum, retroperitoneum, neck and adrenal gland. However, GN can potentially occur anywhere along the peripheral autonomic ganglion sites...
2017: Polish Journal of Radiology
https://www.readbyqxmd.com/read/28578246/appendiceal-ganglioneuroma-in-neurofibromatosis-type-2
#11
Yonah B Esterson, Aryeh Y Esterson, Gregory M Grimaldi, John S Pellerito, Robin J Warshawsky
Here we report the case of a 30-year-old woman with a history of neurofibromatosis type 2 (NF2) who presented with an incidentally discovered pelvic mass on an ultrasound. The identity of the mass remained indeterminate following a CT and MRI, although appendiceal pathology was suspected. An elective laparoscopy and appendectomy were performed without complication and pathology on the specimen demonstrated an appendiceal ganglioneuroma. To our knowledge, there has been no prior documented case of an appendiceal ganglioneuroma in a patient with NF2...
May 27, 2017: Clinical Imaging
https://www.readbyqxmd.com/read/28550366/minimally-invasive-resection-of-adrenal-masses-in-infants-and-children-results-of-a-european-multi-center-survey
#12
Francesco Fascetti-Leon, Giovanni Scotton, Luca Pio, Raimundo Beltrà, Paolo Caione, Ciro Esposito, Girolamo Mattioli, Amulya K Saxena, Sabine Sarnaki, 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)...
May 26, 2017: Surgical Endoscopy
https://www.readbyqxmd.com/read/28537695/robotic-assisted-laparoscopic-excision-of-a-retroperitoneal-ganglioneuroma
#13
Lucas Medeiros Burttet, Fernando Jahn da Silva Abreu, Gabrielle Aguiar Varaschin, Brasil Silva, Milton Berger
INTRODUCTION: Ganglioneuromas are rare benign neoplasms of the sympathetic nervous system. We describe the case of an incidentally found ganglioneuroma in a woman. To our knowledge this is the first described case of robotic excision of a retroperitoneal ganglioneuroma. CASE: A 41-year-old female had an incidental retroperitoneal mass found during a routine US. CT scan and MRI showed an 8.3cm homogeneous mass, adjacent to left kidney upper pole, with peripheral contrast enhancement...
May 23, 2017: International Braz J Urol: Official Journal of the Brazilian Society of Urology
https://www.readbyqxmd.com/read/28532923/radiation-induced-schwannomas-and-neurofibromas-a-systematic-review
#14
REVIEW
Ryuya Yamanaka, Azusa Hayano
OBJECTIVE: Radiation-induced benign peripheral nerve sheath tumors are uncommon late complications of irradiation. We conducted the largest systematic review of individual patient data. METHODS: We performed a systematic search of PubMed databases and compiled a comprehensive literature review. Kaplan-Meier analysis was used to investigate survival, and statistical significance was assessed with a log-rank test. RESULTS: We analyzed 40 cases of radiation-induced benign peripheral nerve sheath tumors...
August 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28512424/optical-coherence-tomography-angiography-of-retinal-microvascular-changes-overlying-choroidal-nodules-in-neurofibromatosis-type-1
#15
Catherine Cassiman, Ingele Casteels, Peter Stalmans, Eric Legius, Julie Jacob
PURPOSE: To report 3 cases of neurofibromatosis type 1 (NF1) with choroidal nodules associated with retinal microvascular changes imaged with optical coherence tomography angiography (OCTA). METHODS: Small case series in 3 NF1 patients. OCTA examinations were performed by a trained examiner (J.J.) after pupillary dilation. A standard scan, centered over the macula measuring 6 × 6 mm and 3 × 3 mm was obtained according to the findings on standard color photography...
January 2017: Case Reports in Ophthalmology
https://www.readbyqxmd.com/read/28511265/diagnostic-value-of-diffusion-weighted-mri-for-tumor-characterization-differentiation-and-monitoring-in-pediatric-patients-with-neuroblastic-tumors
#16
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
#17
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/28506406/adrenal-ganglioneuroma-the-padua-endocrine-surgery-unit-experience
#18
Maurizio Iacobone, Francesca Torresan, Marilisa Citton, Donatella Schiavone, Giovanni Viel, Gennaro Favia
BACKGROUND: Adrenal ganglioneuroma (AGN) is a rare tumor that originates from the gangliar cells of the sympathetic nervous system. It represents less than 5% of all adrenal masses. AGN occurs as a large mass, with benign behavior and no relevant symptoms and hormonal secretion, but it is often misdiagnosed because the preoperative radiological diagnosis is generally challenging. The aim of the present paper is to report the experience of a tertiary referral academic center regarding the management of AGN and review the relevant literature...
May 2017: International Journal of Surgery
https://www.readbyqxmd.com/read/28490166/giant-ganglioneuroma-of-thoracic-spine-a-case-report-and-review-of-literature
#19
Yong Huang, Lidi Liu, Qiao Li, Shaokun Zhang
Ganglioneuroma (GN) is a rare benign tumor of neural crest origin usually found in the abdomen, but may occasionally present at uncommon sites including the cervical, lumbar, or sacral spine. However, GNs of thoracic spine are extremely rare. In this report, we describe a 12-year-old girl with giant GN in the thoracic spine, who underwent successful resection (T1-4 level) of the tumor. Histopathological examination confirmed the diagnosis. GN should be considered in the differential diagnosis of any paraspinal mass...
May 2017: Journal of Korean Neurosurgical Society
https://www.readbyqxmd.com/read/28481069/-laparoscopic-adrenalectomy-advantages-of-the-minimally-invasive-approach
#20
P Rodríguez Iglesias, J Gómez-Chacón Villalba, L Rodríguez Caraballo, P Ortolá Fortes, J Cortés Sáez, A Marco Macián, J J Vila Carbó
AIM: To describe our experience as a tertiary center on the use of laparoscopic adrenal surgery in children. MATERIAL AND METHODS: A descriptive, retrospective study of patients with pathologic adrenal masses undergoing laparoscopic adrenal surgery, between 2012 and 2015. Epidemiological variables, surgical technique, complications and follow-up were studied. RESULTS: Nine patients were studied with a median age of 62 months (5-184). In 3 patients (33...
October 10, 2016: Cirugía Pediátrica: Organo Oficial de la Sociedad Española de Cirugía Pediátrica
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