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https://www.readbyqxmd.com/read/29330717/posterior-retroperitoneoscopic-resection-of-extra-adrenal-paraganglioma-located-in-the-aorto-caval-space
#1
Sang-Wook Kang, Emad Kandil, Min Jhi Kim, Kwang Soon Kim, Cho Rok Lee, Jong Ju Jeong, Kee-Hyun Nam, Woong Youn Chung, Cheong Soo Park
BACKGROUND: The posterior retroperitoneoscopic adrenalec tomy has several advantages compared with the transperitoneal approach such as a shorter and more direct route to the target organ, no breach of the intraperitoneal space, and no required retraction of the adjacent organs. It also is a safe procedure with a short learning curve.1-5 This report presents a challenging case of an extra-adrenal paraganglioma located in the aorto-caval space and managed using the retroperitoneal approach...
January 12, 2018: Annals of Surgical Oncology
https://www.readbyqxmd.com/read/29330195/dicer1-gene-mutations-in-endocrine-tumors
#2
Michael Solarski, Fabio Rotondo, William Foulkes, John R Priest, Luis V Syro, Henriett Butz, Michael Cusimano, Kalman Kovacs
In this review, the importance of the DICER1 gene in the function of endocrine cells is discussed. There is conclusive evidence that DICER1 mutations play a crucial role in the development, progression, cell proliferation, therapeutic responsiveness and behavior of several endocrine tumors. We review the literature of DICER1 gene mutations in thyroid, parathyroid, pituitary, pineal gland, endocrine pancreas, paragangliomas, medullary, adrenocortical, ovarian, and testicular tumors. Although significant progress has been made during the last few years, much more work is needed to fully understand the significance of DICER1 mutations...
January 12, 2018: Endocrine-related Cancer
https://www.readbyqxmd.com/read/29315604/trends-of-genetic-screening-in-patients-with-pheochromocytoma-and-paraganglioma-15-year-experience-in-a-high-volume-tertiary-referral-center
#3
Ammar Asban, Wouter P Kluijfhout, Frederick T Drake, Toni Beninato, Elizabeth Wang, Kate Chomsky-Higgins, Wen T Shen, Jessica E Gosnell, Insoo Suh, Quan-Yang Duh
BACKGROUND AND OBJECTIVES: Genetic testing for pheochromocytoma and paraganglioma allows for early detection of hereditary syndromes and enables close follow-up of high-risk patient. We investigated the trends in genetic testing among patients at a high-volume referral center and evaluated the prevalence of pheochromocytomas and paragangliomas. METHODS: We reviewed the charts of 129 patients who underwent adrenalectomy for pheochromocytoma and paraganglioma between January 2000 and July 2015...
January 8, 2018: Journal of Surgical Oncology
https://www.readbyqxmd.com/read/29315193/the-application-of-sigmoid-sinus-tunnel-packing-or-push-packing-of-the-inferior-petrous-sinus-in-the-microsurgical-management-of-jugular-paragangliomas
#4
Mi Jang, Hongyan Liu, Chunfu Dai
OBJECTIVE: To analyze the outcomes of microsurgically treated jugular paragangliomas with control of bleeding from the inferior petrous sinus. STUDY DESIGN: Retrospective patient review. SETTING: A single university hospital. PATIENTS: Forty-three patients with jugular paragangliomas were diagnosed in the past 7 years in our clinic. MAIN OUTCOME MEASURES: Surgical tumor control, intraoperative blood loss, intraoperative management of the facial nerve, and the preoperative and postoperative function of the lower central nerves...
February 2018: Otology & Neurotology
https://www.readbyqxmd.com/read/29313083/-neuroendocrine-neoplasms-of-the-head-and-neck
#5
REVIEW
B Konukiewitz, A Agaimy, W Weichert, G Klöppel
Common to all neuroendocrine neoplasms (NENs), irrespective of their site of origin, is the expression of synaptophysin and chromogranin A. NENs of the head and neck region derive either from epithelial or neural/neuroectodermal tissues. The epithelial-type NENs express cytokeratins and include the well-differentiated typical and atypical carcinoids (also called low- and intermediate-grade neuroendocrine carcinomas by WHO), the poorly differentiated high-grade neuroendocrine carcinomas of small and large cell type and the mixed neuroendocrine-nonneuroendocrine neoplasms...
January 8, 2018: Der Pathologe
https://www.readbyqxmd.com/read/29305799/prevention-of-serious-skeletal-related-events-by-interventional-radiology-techniques-in-patients-with-malignant-paraganglioma-and-pheochromocytoma
#6
Guillaume Gravel, Sophie Leboulleux, Lambros Tselikas, Flora Fassio, Mohamed Berraf, Amandine Berdelou, Bakar Ba, Segolene Hescot, Julien Hadoux, Martin Schlumberger, Abir Al Ghuzlan, France Nguyen, Mathieu Faron, Thierry de Baere, Eric Baudin, Frederic Deschamps
PURPOSE: Bone metastases (BM) and skeletal-related events (SRE) are frequent in patients with malignant pheochromocytoma and paraganglioma (PPM) and the best modality of prevention unknown. The role of interventional radiology (IR) techniques for the prevention of SRE in the multidisciplinary management of malignant PPM has not been evaluated in that setting. METHODS: Single referral center retrospective review of all patients with malignant PPM with BM from 2000 to 2016...
January 5, 2018: Endocrine
https://www.readbyqxmd.com/read/29305721/paragangliomas-arise-through-an-autonomous-vasculo-angio-neurogenic-program-inhibited-by-imatinib
#7
Fabio Verginelli, Silvia Perconti, Simone Vespa, Francesca Schiavi, Sampath Chandra Prasat, Paola Lanuti, Alessandro Cama, Lorenzo Tramontana, Diana Liberata Esposito, Simone Guarnieri, Artenca Sheu, Mattia Russel Pantalone, Rosalba Florio, Annalisa Morgano, Cosmo Rossi, Giuseppina Bologna, Marco Marchisio, Andrea D'Argenio, Elisa Taschin, Rosa Visone, Giuseppe Opocher, Angelo Veronese, Carlo T Paties, Vinalogu K Rajasekhar, Cecilia Söderberg-Nauclér, Mario Sanna, Lavinia Vittoria Lotti, Renato Mariani-Costantini
Tumours can be viewed as aberrant tissues or organs sustained by tumorigenic stem-like cells that engage into dysregulated histo/organogenetic processes. Paragangliomas, prototypical organoid tumours constituted by dysmorphic variants of the vascular and neural tissues found in normal paraganglia, provide a model to test this hypothesis. To understand the origin of paragangliomas, we built a biobank comprising 77 cases, 18 primary cultures, 4 derived cell lines, 80 patient-derived xenografts and 11 cell-derived xenografts...
January 5, 2018: Acta Neuropathologica
https://www.readbyqxmd.com/read/29301496/mr-imaging-features-of-benign-retroperitoneal-paragangliomas-and-schwannomas
#8
Yanguang Shen, Yan Zhong, Haiyi Wang, Lu Ma, Yingwei Wang, Kun Zhang, Zhonghua Sun, Huiyi Ye
BACKGROUND: To determine whether MRI feature analysis can differentiate benign retroperitoneal extra-adrenal paragangliomas and schwannomas. METHODS: The MRI features of 50 patients with confirmed benign retroperitoneal extra-adrenal paragangliomas and schwannomas were retrospectively reviewed by two radiologists blinded to the histopathologic diagnosis. These features were compared between two types of tumours by use of the Mann-Whitney test and binary logistic regression...
January 4, 2018: BMC Neurology
https://www.readbyqxmd.com/read/29299512/head-and-neck-paragangliomas-a-two-decade-institutional-experience-and-algorithm-for-management
#9
Joshua D Smith, Rachel N Harvey, Owen A Darr, Mark E Prince, Carol R Bradford, Gregory T Wolf, Tobias Else, Gregory J Basura
Objectives: Paragangliomas of the head and neck and cranial base are typically benign, slow-growing tumors arising within the jugular foramen, middle ear, carotid bifurcation, or vagus nerve proper. The objective of this study was to provide a comprehensive characterization of our institutional experience with clinical management of these tumors and posit an algorithm for diagnostic evaluation and treatment. Methods: This was a retrospective cohort study of patients undergoing treatment for paragangliomas of the head and neck and cranial base at our institution from 2000-2017...
December 2017: Laryngoscope Investigative Otolaryngology
https://www.readbyqxmd.com/read/29290904/primary-extradural-paraganglioma-of-the-thoracic-spine-a-case-report
#10
Alexander Reddy, Fanny Morón
Paragangliomas are rare, mostly benign neuroendocrine tumors arising from autonomic paraganglia. Spinal paragangliomas are uncommon, and among these, paragangliomas of the thoracic spine are distinctly unusual. We present the case of a primary paraganglioma of the extradural thoracic spine in a 34-year-old woman.
December 2017: Journal of Radiology Case Reports
https://www.readbyqxmd.com/read/29288372/robotic-assisted-laparoscopic-surgery-for-pediatric-tumors-a-bicenter-experience
#11
P Meignan, Q Ballouhey, J Lejeune, K Braik, B Longis, A R Cook, H Lardy, L Fourcade, Aurélien Binet
Mini-invasive surgery is more and more integrated in pediatric surgery. The robotic-assisted surgery brought new advantages from which the patient and the surgeon could benefit compared to laparoscopy. Its use in oncological surgery is still controversial. 12 robotic-assisted tumor resections with the da Vinci Surgical Robot (Intuitive Surgical, Sunnyvale, CA) were attempted in 11 children (mean age 7.65 years; age range 0.75-16.75 years; mean weight 30.3 kg; weight range 8.6-62 kg) in two centers. Mean total operative time was 145 min (range 72-263 min)...
December 29, 2017: Journal of Robotic Surgery
https://www.readbyqxmd.com/read/29282712/homozygous-tmem127-mutations-in-two-patients-with-bilateral-pheochromocytomas
#12
K Eijkelenkamp, M J W Olderode-Berends, R B van der Luijt, M Robledo, M van Dooren, R A Feelders, J de Vries, M N Kerstens, T P Links, A N A van der Horst-Schrivers
Pheochromocytoma (PCC) and paraganglioma (PGL) are rare neuroendocrine tumors that are hereditary in up to 50% of patients. The gene encoding transmembrane-protein-127 (TMEM127) is one of the PCC/PGL susceptibility genes with an autosomal dominant inheritance pattern. Here we report two patients with bilateral PCC who both harbored a homozygous TMEM127-mutation. In a 31-year old mentally retarded patient the homozygous c.410-2A>G mutation was discovered during an update of DNA-analysis. A 26-year old mentally retarded patient was found to have a homozygous c...
December 28, 2017: Clinical Genetics
https://www.readbyqxmd.com/read/29282558/synchronous-bilateral-pheochromocytomas-and-paraganglioma-with-novel-germline-mutation-in-max-a-case-report
#13
Masahiro Shibata, Takahiro Inaishi, Noriyuki Miyajima, Yayoi Adachi, Yuko Takano, Kenichi Nakanishi, Dai Takeuchi, Sumiyo Noda, Yuichi Aita, Kazuhiro Takekoshi, Yasuhiro Kodera, Toyone Kikumori
BACKGROUND: Recent advance of genetic testing has contributed to the diagnosis of hereditary pheochromocytoma and paraganglioma (PPGL). The clinical characteristics of hereditary PPGL are varying among the types of mutational genes. It is still difficult to specify the pathognomonic symptoms in the case of rare genetic mutations. Here, we report the case of synchronous bilateral pheochromocytomas and paraganglioma with novel MYC associated factor X (MAX) gene mutation. CASE PRESENTATION: A 24-year-old female had hyperhidrosis and hypertension...
December 28, 2017: Surgical Case Reports
https://www.readbyqxmd.com/read/29273559/high-specificity-of-spot-urinary-free-metanephrines-in-diagnosis-and-prognosis-of-pheochromocytomas-and-paragangliomas-by-hplc-with-electrochemical-detection
#14
Ming Zuo, Qianna Zhen, Xiaoqing Zhang, Wenbi Zou, Xiangchun Yang, Gang Tian, Zhenghu Shi, Qifu Li, Min Ding
BACKGROUND: The metanephrines (MNs) in plasma and urine were proposed as biomarkers for the diagnosis of pheochromocytomas and paragangliomas (PPGLs). However, plasma free MNs and 24h urinary fractionated MNs were not satisfactory enough in specificity for the diagnosis of PPGLs. Moreover, the collection of 24h urine was inconvenient. This work examined the diagnostic and prognostic efficiency of free MNs in spot urine for PPGLs. METHODS: We measured free MNs concentration in spot urine and plasma of 28 PPGLs patients and 155 control subjects by HPLC with electrochemical detection...
December 19, 2017: Clinica Chimica Acta; International Journal of Clinical Chemistry
https://www.readbyqxmd.com/read/29259765/case-report-managing-a-giant-high-grade-carotid-body-tumor-in-a-resource-limited-setting
#15
Sunil Munakomi, Samrita Chaudhary, Iype Cherian
Herein we report the management of a giant, high-grade and vascular carotid body tumor in a young woman. She presented with slowly progressive neck swelling. Vascular imaging revealed a left-sided, high-grade giant carotid body tumor (> 8cm).  The tumor was completely excised by caudocranial subadventitial dissection. Histology of the tumor revealed a characteristic Zellballen pattern of the lesion, suggestive of a paraganglioma. The patient made an uneventful recovery. We also discuss newer insights regarding the management of such highly vascular lesions...
2017: F1000Research
https://www.readbyqxmd.com/read/29240581/cutaneous-melanocytoma-with-crtc1-trim11-fusion-report-of-5-cases-resembling-clear-cell-sarcoma
#16
Lucie Cellier, Emilie Perron, Daniel Pissaloux, Marie Karanian, Veronique Haddad, Laurent Alberti, Arnaud de la Fouchardière
We report 5 cases of primary intradermal nodular unpigmented tumors with a melanocytic immunophenotype associated with a novel CRTC1-TRIM11 fusion. Clinically, the cutaneous nodules were slowly growing in 3 women and 2 men (25 to 82 y old, median, 28 y) with no specific topography. Lesion size ranged from 4 to 12 mm (median, 5 mm). The tumors were strictly located in the dermis with a nodular pattern. The cells were arranged in confluent nests and fascicules. Central fibronecrotic areas were present in 2 cases...
December 7, 2017: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/29239044/pathology-and-genetics-of-phaeochromocytoma-and-paraganglioma
#17
REVIEW
John Turchini, Veronica K Y Cheung, Arthur S Tischler, Ronald R De Krijger, Anthony J Gill
Phaeochromocytoma and paraganglioma (PHEO/PGL) are rare tumours with an estimated annual incidence of 3 per million. Advances in molecular understanding have led to the recognition that at least 30-40% arise in the setting of hereditary disease. Germline mutations in the succinate dehydrogenase genes SDHA, SDHB, SDHC, SDHD and SDHAF2 are the most prevalent of the more than 19 hereditary genetic abnormalities which have been reported. It is therefore recommended that, depending on local resources and availability, at least some degree of genetic testing should be offered to all PHEO/PGL patients, including those with clinically sporadic disease...
January 2018: Histopathology
https://www.readbyqxmd.com/read/29239034/succinate-dehydrogenase-sdh-deficient-neoplasia
#18
REVIEW
Anthony J Gill
The succinate dehydrogenase (SDH) complex is a key respiratory enzyme composed of four subunits: SDHA, SDHB, SDHC and SDHD. Remarkably, immunohistochemistry for SDHB becomes negative whenever there is bi-alleic inactivation of any component of SDH, which is very rare in the absence of syndromic disease. Therefore, loss of SDHB immunohistochemistry serves as a marker of syndromic disease, usually germline mutation of one of the SDH subunits. Tumours which show loss of SDHB expression are termed succinate dehydrogenase-deficient...
January 2018: Histopathology
https://www.readbyqxmd.com/read/29222838/results-of-a-systematic-literature-review-of-treatment-modalities-for-jugulotympanic-paraganglioma-stratified-per-fisch-class
#19
Thijs T G Jansen, Henri J L M Timmers, Henri A M Marres, Johannes H A M Kaanders, Henricus P M Kunst
OBJECTIVE: Key for successful jugulotympanic paraganglioma management is a personalized approach aiming for the best practice for each individual patient. To this end, a systematic review is performed, evaluating the local control- and complication rates for the different treatment modalities stratified by the broadly accepted Fisch classification. DESIGN: A systematic literature review according to the PRISMA statement was performed. A detailed overview of individual treatment outcomes per Fisch class is provided...
December 8, 2017: Clinical Otolaryngology
https://www.readbyqxmd.com/read/29218662/laparoscopic-removal-of-retroperitoneal-tumor-with-maneuver-of-hanging-inferior-vena-cava
#20
Sungho Kim, Ho-Seong Han, YoungRok Choi, Yoo-Seok Yoon, Jai Young Cho
BACKGROUND: Laparoscopic resection of retroperitoneal mass is challenging because of its location close to major vessels and a limited range of laparoscopic instruments [1-3]. We report a case of a huge retroperitoneal paraganglioma that was successfully excised laparoscopically using maneuver of hanging IVC METHOD: A 67-year-old female had abdominal mass detected during routine check-up. She had no symptoms associated mass. Hematologic, biochemical investigations, and hormone tests reveal normal results...
December 7, 2017: Surgical Endoscopy
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