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Pheochromocytoma and extra-adrenal

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https://www.readbyqxmd.com/read/27865588/pediatric-patients-with-pheochromocytoma-and-paraganglioma-should-have-routine-preoperative-genetic-testing-for-common-susceptibility-genes-in-addition-to-imaging-to-detect-extra-adrenal-and-metastatic-tumors
#1
Bruna Babic, Dhaval Patel, Rachel Aufforth, Yasmine Assadipour, Samira M Sadowski, Martha Quezado, Naris Nilubol, Tamara Prodanov, Karel Pacak, Electron Kebebew
BACKGROUND: Pediatric pheochromocytomas and paragangliomas are rare with limited data on the optimal management approach. The aim of this study was to determine the role of genetic testing and imaging to detect extra-adrenal and/or metastatic tumors in pediatric pheochromocytomas and paragangliomas. METHODS: We performed a retrospective study of 55 patients diagnosed at ≤21 years of age with pheochromocytomas and paragangliomas with analysis of data on genetic testing and multimodal imaging...
November 16, 2016: Surgery
https://www.readbyqxmd.com/read/27812903/expanding-the-horizon-of-robotic-surgery-to-large-pelvic-paraganglioma
#2
Santosh Kumar, M Pragatheeswarane, Aditya Prakash Sharma, Kshitij Bishnoi, Manjeet Kumar Sharma, Vikas Kumar Panwar, Sameer Sethi
INTRODUCTION: Paragangliomas are the most common extra-adrenal pheochromocytoma. Symptomatic and growing tumors demand surgical extirpation. Minimally invasive techniques allow surgeons to perform the procedure without wide exposure and mobilization of intra-abdominal organs. To our knowledge, we present the fourth case of robotic excision of a large retroperitoneal paraganglioma. MATERIALS AND METHODS: An 18-year-old unmarried female presented with occipital headache for past 3 years...
November 3, 2016: Journal of Robotic Surgery
https://www.readbyqxmd.com/read/27790975/unusual-case-of-pheochromocytoma-presenting-with-diabetic-ketoacidosis
#3
Yub Raj Sedhai, Kruthika Reddy, Dhruvan Patel, James A Lozada
Pheochromocytoma is a rare catecholamine-secreting tumour that arises from chromaffin cells in the adrenal medulla or extra-adrenal sympathetic ganglia. It classically presents with paroxysmal headaches, hypertension, palpitations and sweating related to catecholamine excess. Diabetes is reported to be present in approximately one-third of patients with pheochromocytoma; however, diabetic ketoacidosis is an extremely rare complication. We present a case of an African-American male aged 30 years who initially presented with diabetic ketoacidosis and hypertensive urgency whose blood pressure and glycaemic control improved remarkably following tumour excision...
October 19, 2016: BMJ Case Reports
https://www.readbyqxmd.com/read/27751309/paraganglioma-masquerading-as-acute-myocardial-infarction-and-cardiogenic-shock
#4
Tanveer Ahmad, Shankaragouda Patil, Ashwini Kumar Pasarad, Nandakumar Neralakere Maheshwarappa, Kolkebaile Sadanand Kishore
Paragangliomas, extra-adrenal pheochromocytomas, are rare catecholamine-secreting tumor. A 34-year-old lady admitted with diagnosis of ST elevation acute myocardial infarction with cardiogenic shock. Left ventricular function, severely depressed, returned to normal after initial stabilization. Coronary angiogram was normal. A para-aortic paraganglioma was diagnosed during the patient's work-up with biochemical studies, computed tomography of abdomen and functional radioisotopes imaging and was eventually surgically resected...
September 2016: Indian Heart Journal
https://www.readbyqxmd.com/read/27728072/adrenal-and-extra-adrenal-pheochromocytomas-presenting-as-ventricular-arrhythmia-a-report-of-three-cases
#5
G Mallikarjun Rao
No abstract text is available yet for this article.
January 2016: Journal of the Association of Physicians of India
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
#6
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...
October 5, 2016: Asian Journal of Endoscopic Surgery
https://www.readbyqxmd.com/read/27695589/ectopic-cushing-s-syndrome-due-to-retroperitoneal-acth-producing-paragangliomas
#7
Fan Chen, Xiangyu Wang, Yang Wang, Hui Meng, Xinguo Hou, Yaofeng Zhu, Wei Gao, Xuewen Jiang, Shouzhen Chen, Zhaocun Zhang, Zhichuan Zou, Tianyi He, Yue Yang, Kejia Zhu, Yong Wang, Yaxiao Liu, Jianfeng Cui, Benkang Shi, Gang Yin
Extra-adrenal pheochromocytomas, or paragangliomas, are rare tumours that derive from extra-adrenal chromaffin cells. Cushing's syndrome (CS) caused by paragangliomas is extremely rare. We report a 53-year-old man with hypertension, diabetes, and symptoms of hypokalemia. Computer tomography (CT) revealed two retroperitoneal masses and bilateral adrenal hyperplasia. Together with the laboratory examinations, ectopic CS caused by multiple paragangliomas was highly suspected. The patient underwent resections of retroperitoneal tumours, left kidney, and left adrenal; postoperative histopathology confirmed two paragangliomas that were both positively stained for adrenocorticotropic hormone (ACTH)...
September 2016: Canadian Urological Association Journal, Journal de L'Association des Urologues du Canada
https://www.readbyqxmd.com/read/27683685/malignant-paraganglioma-a-rare-presentation-with-foot-drop-a-case-report
#8
Sheikh Asad, Jens Peters-Willke, Louise Nott
Paragangliomas (or extra-adrenal paragangliomas) are rare neuroendocrine tumors that arise from the extra-adrenal autonomic paraganglia, small organs consisting mainly of neuroendocrine cells that are derived from the embryonic neural crest and have the ability to secrete catecholamines. This is in contrast to pheochromocytomas, where tumors occur in the adrenal gland. Malignant paraganglioma is a very rare presentation. We present a case report of a 56-year-old woman with acute foot drop with no previous significant background history...
December 2015: Journal of Spine Surgery (Hong Kong)
https://www.readbyqxmd.com/read/27592061/-hypertension-etiological-work-up-hormonological-assessment-always-before-imaging
#9
Bernard Chamontin, Florent Seguro, Nicolas Touront, Béatrice Bouhanick
The purpose is to consider the practical management of etiological work up in hypertension, beyond national or international recommendations, leading to consider the prior practice of hormonal assays or renal, renovascular or adrenal imaging. The ease of access to imaging, difficulties to meet the requirements to obtain reliable hormonal assays explain the use of first-line imaging in clinical practice. The renal and adrenal CT angiography provides diagnostic orientation without allowing a formal conclusion...
August 31, 2016: La Presse Médicale
https://www.readbyqxmd.com/read/27521779/an-unusual-case-of-asymptomatic-non-urothelial-bladder-tumour
#10
Daniel Ching, Eleni Anastasiadis, Sarb Sandhu
INTRODUCTION: Non-urothelial tumours are rare and account for less than 5% of all bladder tumours. Bladder paragangliomas also known as extra-adrenal pheochromocytomas are of the non-urothelial subgroup. We present an unusual case of asymptomatic bladder paraganglioma. CASE REPORT: A 77year old lady presented with acute abdominal pain was found to have an incidental enhancing nodule in the bladder. During cystoscopy and transurethral resection the patient experienced significant fluctuations in blood pressure that required anaesthetic adjustments...
2016: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/27508677/-op-lb01-04-high-prevalence-of-cardiac-electric-abnormalities-in-patients-with-pheochromocytomas-and-secreting-paragangliomas
#11
E Berra, M Gonzales, A Bersali, A Persu
OBJECTIVE: Pheochromocytomas and paragangliomas (PPGLs) are catecholamine-producing tumours of chromaffin cells of the adrenal medulla and extra-adrenal paraganglia. Besides hypertension, they can be at the origin of a wide range of cardiovascular manifestations, including cardiac electric abnormalities such as long QT interval, ventricular tachycardia and torsades de pointe. However, knowledge about PPGL-related arrhythmias is scarce and mostly derived from case reports. The aim of our study was to evaluate the prevalence, nature and reversibility of cardiac electric changes and arrhythmias in patients with PPGLs...
September 2016: Journal of Hypertension
https://www.readbyqxmd.com/read/27446377/pheochromocytoma-of-the-pancreas-a-report-of-three-cases-and-a-literature-review
#12
Min Yang, Hui Ding, Min Cai, Yan-An He, Yu Cai, Yong Zeng, Bo-Le Tian
Pheochromocytoma is primarily derived from the adrenal medulla. The majority of extra-adrenal pheochromocytoma cases occur in the superior para-aortic region and para-adrenal area. However, pheochromocytoma originating from the pancreas is rare. The present study reports the cases of three patients who had no history of hypertension but were post-operatively diagnosed with pheochromocytoma located in the pancreas. Of the three patients, two were admitted to hospital due to abdominal pain, and imaging examinations revealed a soft-tissue lesion in the head of pancreas...
August 2016: Oncology Letters
https://www.readbyqxmd.com/read/27428777/paraganglioma-pituitary-adenoma-and-osteosarcoma-in-a-dog
#13
Cecilia Robat, Ruth Houseright, Joanne Murphey, Saundra Sample, Marie Pinkerton
An 11-year-old neutered male Alaskan Malamute mixed-breed dog was presented with a complaint of polyuria/polydipsia (PU/PD), weight loss, tachypnea, regurgitation, and a previous history of nontreated osteosarcoma of the right distal radius, diagnosed 21 months prior. On physical examination, an abdominal mass was palpated. The abdominal mass was aspirated and cytologically diagnosed as a neuroendocrine tumor, suspected to be a pheochromocytoma. Laboratory examination revealed a mild anemia and thrombocytopenia, markedly elevated ATP and ALP activities, and moderate hypercalcemia...
September 2016: Veterinary Clinical Pathology
https://www.readbyqxmd.com/read/27390021/management-of-adrenal-incidentalomas-european-society-of-endocrinology-clinical-practice-guideline-in-collaboration-with-the-european-network-for-the-study-of-adrenal-tumors
#14
Martin Fassnacht, Wiebke Arlt, Irina Bancos, Henning Dralle, John Newell-Price, Anju Sahdev, Antoine Tabarin, Massimo Terzolo, Stylianos Tsagarakis, Olaf M Dekkers
: By definition, an adrenal incidentaloma is an asymptomatic adrenal mass detected on imaging not performed for suspected adrenal disease. In most cases, adrenal incidentalomas are nonfunctioning adrenocortical adenomas, but may also represent conditions requiring therapeutic intervention (e.g. adrenocortical carcinoma, pheochromocytoma, hormone-producing adenoma or metastasis). The purpose of this guideline is to provide clinicians with best possible evidence-based recommendations for clinical management of patients with adrenal incidentalomas based on the GRADE (Grading of Recommendations Assessment, Development and Evaluation) system...
August 2016: European Journal of Endocrinology
https://www.readbyqxmd.com/read/27382822/anesthetic-management-in-unexpected-extra-adrenal-pheocromocytoma-presenting-with-thoracic-spinal-cord-compression
#15
Amr El Kouny, Mohammed Al Harbi, Rashid Muhammad Arif, Nazar Ilyas, El Abbasy Omar Hamed, Maqsood Memon, Ali Nawaz, Vassilios Dimitriou
A 52 yearold female presented with a thoracic paravertebral tumour causing spinal nerve root compression and lower limbs neurologic symptoms. The patient was scheduled to undergo thoracic decompression laminectomy and instrumentation. Markedly severe hemodynamic fluctuations happened during the manipulation of the tumor and continued after the tumor was removed. After multimodal antihypertensive therapy the vital signs were adequately managed and the surgery was successfully performed without complications...
February 2016: Middle East Journal of Anesthesiology
https://www.readbyqxmd.com/read/27257146/diagnosis-of-endocrine-disease-the-diagnostic-performance-of-adrenal-biopsy-a-systematic-review-and-meta-analysis
#16
REVIEW
Irina Bancos, Shrikant Tamhane, Muhammad Shah, Danae A Delivanis, Fares Alahdab, Wiebke Arlt, Martin Fassnacht, M Hassan Murad
OBJECTIVE: To perform a systematic review of published literature on adrenal biopsy and to assess its performance in diagnosing adrenal malignancy. METHODS: Medline In-Process and Other Non-Indexed Citations, MEDLINE, EMBASE, and Cochrane Central Register of Controlled Trial were searched from inception to February 2016. Reviewers extracted data and assessed methodological quality in duplicate. RESULTS: We included 32 observational studies reporting on 2174 patients (39...
August 2016: European Journal of Endocrinology
https://www.readbyqxmd.com/read/27248805/procedural-and-clinical-outcomes-of-percutaneous-adrenal-biopsy-in-a-high-risk-population-for-adrenal-malignancy
#17
Danae A Delivanis, Dana Erickson, Thomas D Atwell, Neena Natt, Spyridoula Maraka, Schmit D Grant, Patrick W Eiken, Mark A Nathan, William F Young, Irina Bancos
OBJECTIVE: The role of percutaneous adrenal biopsy in a high risk population for adrenal malignancy has not been fully investigated. Our aim was to describe the clinical presentation leading to the adrenal biopsy and evaluate the diagnostic performance, complications and non-diagnostic rate of adrenal biopsy. DESIGN: Single center, retrospective cohort study. PATIENTS AND MEASUREMENTS: Medical records of patients who underwent adrenal biopsy between 1994 and 2014 were reviewed...
June 1, 2016: Clinical Endocrinology
https://www.readbyqxmd.com/read/27215711/traumatic-rupture-of-extra-adrenal-pheochromocytoma
#18
Ali Bohlok, Raed Chehabeddine, Achraf Safa, Malek Tabbara
No abstract text is available yet for this article.
May 2016: American Surgeon
https://www.readbyqxmd.com/read/27172533/retroperitoneal-functioning-paraganglioma-a-rare-case-of-secondary-diabetes
#19
Radu Mihail Mirica, Octav Ginghina, Gabriela Zugravu, Razvan Iosifescu, Mihai Ionescu, Anca Ichiman, Adrian Rosca, Alexandra Mirica, Nicolae Iordache, Rubin Munteanu
INTRODUCTION: Paragangliomas are rare neuroendocrine tumors that arise from the extra-adrenal autonomic paraganglia, which can derive from either parasympathetic or sympathetic paraganglia and are closely related to pheochromocytomas. CASE REPORT: We present the case of a young male patient of 37 years old, who was admitted for hypertensive crisis and palpitations. His medical history included medically controlled type 2 diabetes mellitus, (diagnosed 10 months ago), Hepatitis A...
March 2016: Chirurgia
https://www.readbyqxmd.com/read/27130746/uncontrolled-hypertension-palpitation-and-sweating-in-young-female-a-rare-cause
#20
H V Sandeep, K S Sarat, L T Ng
Extra-adrenal /retroperitoneal paraganglioma is a rare cause of hypertension in young with increased incidence of metastasis as compared to adrenal pheochromocytoma. We present a case of a young female with history of headache, nausea/vomiting, palpitations, uncontrolled hypertension, heat intolerance and diaphoresis. The 24-hour urine catecholamine levels were elevated. Clinical diagnosis of pheochromocytoma was made and further evaluation with Computed Tomography (CT) scan of the adrenals revealed extradrenal para-aortic retroperitoneal mass in keeping with paraganglioma...
February 2016: Medical Journal of Malaysia
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