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Pheochromocytoma and extra-adrenal

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https://www.readbyqxmd.com/read/28197344/from-bad-to-worse-paraganglioma-diagnosis-during-induction-of-labor-for-coexisting-preeclampsia
#1
Sasima Dusitkasem, Blair H Herndon, Dalton Paluzzi, Joseph Kuhn, Robert H Small, John C Coffman
Pheochromocytomas and extra-adrenal paragangliomas are catecholamine-secreting tumors that rarely occur in pregnancy. The diagnosis of these tumors in pregnancy can be challenging given that many of the signs and symptoms are commonly attributed to preeclampsia or other more common diagnoses. Early diagnosis and appropriate management are essential in optimizing maternal and fetal outcomes. We report a rare case of a catecholamine-secreting tumor in which diagnosis occurring at the time labor was being induced for concomitant preeclampsia with severe features...
2017: Case Reports in Anesthesiology
https://www.readbyqxmd.com/read/28187001/molecular-markers-of-paragangliomas-pheochromocytomas
#2
REVIEW
Svetlana O Zhikrivetskaya, Anastasiya V Snezhkina, Andrew R Zaretsky, Boris Y Alekseev, Anatoly V Pokrovsky, Alexander L Golovyuk, Nataliya V Melnikova, Oleg A Stepanov, Dmitry V Kalinin, Alexey A Moskalev, George S Krasnov, Alexey A Dmitriev, Anna V Kudryavtseva
Paragangliomas/pheochromocytomas comprise rare tumors that arise from the extra-adrenal paraganglia, with an incidence of about 2 to 8 per million people each year. Approximately 40% of cases are due to genetic mutations in at least one out of more than 30 causative genes. About 25-30% of pheochromocytomas/paragangliomas develop under the conditions of a hereditary tumor syndrome a third of which are caused by mutations in the VHL gene. Together, the gene mutations in this disorder have implicated multiple processes including signaling pathways, translation initiation, hypoxia regulation, protein synthesis, differentiation, survival, proliferation, and cell growth...
February 8, 2017: Oncotarget
https://www.readbyqxmd.com/read/28181818/recommendations-for-the-management-of-adrenal-incidentalomas-what-is-pertinent-for-radiologists
#3
Anju Sahdev
Adrenal incidentalomas are unsuspected, asymptomatic adrenal masses detected on imaging. Most are non-functioning benign adrenocortical adenomas, but can represent other benign lesions or lesions requiring therapeutic intervention including adrenocortical carcinoma, pheochromocytoma, hormone-producing adenoma or metastasis. This review summarises and highlights radiological recommendations within the recently issued guidelines for the management of adrenal incidentalomas from European Society of Endocrinology Clinical Practice in collaboration with European Network for Study of Adrenal Tumours...
February 9, 2017: British Journal of Radiology
https://www.readbyqxmd.com/read/28058099/adenomatoid-tumor-of-the-adrenal-gland-in-young-woman-from-clinical-and-radiological-to-pathological-study
#4
Brankica Krstevska, Sasha Jovanovska Mishevska, Rubens Jovanovic
Adenomatoid tumors are neoplasms of mesothelial origin, usually occurring in the male and female genital tracts. Extragenital localization sites such as adrenal glands are rare but have been reported. When found in the adrenals, they represent great clinical, radiological and pathological diagnostic challenge, with wide range of differential diagnoses to be considered. We present a case of a 30 years old female, with incidental ultrasound finding of unilateral tumor in the right adrenal gland. Multi slices CT scan was of value in localizing this tumor, but not in the precise diagnosis...
November 17, 2016: Rare Tumors
https://www.readbyqxmd.com/read/28011503/surgical-resection-of-giant-pheochromocytomas-arising-behind-the-retrohepatic-inferior-vena-cava
#5
Yuji Soejima, Tomoharu Yoshizumi, Toru Ikegami, Norifumi Harimoto, Shinji Ito, Noboru Harada, Takashi Motomura, Akihisa Nagatsu, Tetsuo Ikeda, Yoshihiko Maehara
BACKGROUND: Surgical resection of retrocaval giant pheochromocytomas (PCs) and of extra-adrenal paragangliomas (EAPs) is a technically challenging procedure but data on these procedures are scarce. The purpose of this study was to present and evaluate our surgical results for such tumors. PATIENTS AND METHODS: We retrospectively analyzed four consecutive patients who had surgery for giant retro-caval PCs and EAPs in terms of surgical technique, resectability of the tumor, requirement for bypass, and postoperative complications...
2017: Anticancer Research
https://www.readbyqxmd.com/read/27999715/secretory-paraspinal-paraganglioma-of-thoracolumar-spine-case-report-and-review-of-literature
#6
Saad Akhtar, Sidra Sattar, Ehsan Bari, Naila Kayani, Sarosh Moeen
BACKGROUND: Pheochromocytomas are catecholamine secreting tumors of the adrenal medulla chromaffin cells, however, when present extra-adrenally they are called paragangliomas. Paragangliomas rarely produce catecholamine in excess, which is evident by clinical symptoms, urine, and blood biochemistry. Total resection of these tumors can lead to complete clinical and biochemical resolution. This case report presents the clinical features, radiological findings, and neurological outcome in a middle-aged female with a secretory paraganglioma...
2016: Surgical Neurology International
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
#7
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...
January 2017: Surgery
https://www.readbyqxmd.com/read/27812903/expanding-the-horizon-of-robotic-surgery-to-large-pelvic-paraganglioma
#8
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
#9
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
#10
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
#11
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
#12
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/27695589/ectopic-cushing-s-syndrome-due-to-retroperitoneal-acth-producing-paragangliomas
#13
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
#14
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
#15
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...
October 2016: La Presse Médicale
https://www.readbyqxmd.com/read/27521779/an-unusual-case-of-asymptomatic-non-urothelial-bladder-tumour
#16
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
#17
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
#18
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
#19
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
#20
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
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