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Pheochromocytoma

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https://www.readbyqxmd.com/read/29786512/-kidney-full-of-stones-and-an-adrenal-gland-not-quite-normal
#1
S Regnier Le Coz, D Drui
A 31-year-old patient was followed for cystinuria, justifying CT scans. In 2006, a tissue mass of 3cm of the right adrenal gland, homogeneous, measured at 3.5cm in 2007 was noted. Blood pressure was 90/61mmHg, without orthostatic hypotension. During the clinical interview, no discomfort, sweat attack, headache, or palpitation was reported by the patient. Hormonal assays did not favor a primary hyperaldosteronism, or a hypercortisolism. Adrenal androgens were normal. 24h normetanephrines urinary excretion and metanephrines to creatinine ratio were elevated...
May 18, 2018: Annales de Cardiologie et D'angéiologie
https://www.readbyqxmd.com/read/29785166/takotsubo-syndrome-induced-by-malignant-pheochromocytoma-in-a-patient-with-type-2-papillary-renal-cell-carcinoma-a-case-report
#2
Anca Diana Demea, Dan-Grigore Dunca, Roxana Adina Radu, Lucia Agoşton-Coldea
The clinical presentation of the Takotsubo syndrome mimics an acute coronary syndrome with chest pain, ischemia-like ECG changes, mild to moderate myocardial enzyme elevation, and apical ballooning on echocardiography and ventriculography. On coronary angiography, epicardial coronary arteries are either normal or exhibit minimal atherosclerotic changes. Primary Takotsubo syndrome usually occurs in postmenopausal women in whom symptoms are triggered by emotional or physical stress, associated with catecholamine surges...
2018: Clujul Medical (1957)
https://www.readbyqxmd.com/read/29783778/anti-tumorigenic-and-anti-metastatic-activity-of-the-sponge-derived-marine-drugs-aeroplysinin-1-and-isofistularin-3-against-pheochromocytoma-in-vitro
#3
Nicole Bechmann, Hermann Ehrlich, Graeme Eisenhofer, Andre Ehrlich, Stephan Meschke, Christian G Ziegler, Stefan R Bornstein
Over 10% of pheochromocytoma and paraganglioma (PPGL) patients have malignant disease at their first presentation in the clinic. Development of malignancy and the underlying molecular pathways in PPGLs are poorly understood and efficient treatment strategies are missing. Marine sponges provide a natural source of promising anti-tumorigenic and anti-metastatic agents. We evaluate the anti-tumorigenic and anti-metastatic potential of Aeroplysinin-1 and Isofistularin-3, two secondary metabolites isolated from the marine sponge Aplysina aerophoba , on pheochromocytoma cells...
May 20, 2018: Marine Drugs
https://www.readbyqxmd.com/read/29779869/genotypic-characteristics-and-their-association-with-phenotypic-characteristics-of-hereditary-medullary-thyroid-carcinoma-in-korea
#4
Kyong Yeun Jung, Seok-Mo Kim, Min Joo Kim, Sun Wook Cho, Bup-Woo Kim, Yong Sang Lee, Jong Ju Jeong, Kee-Hyun Nam, Woong Youn Chung, Kyu Eun Lee, Eun-Jae Chung, Hyo Jeong Kim, Do Joon Park, Myung-Whun Sung, Cheong Soo Park, Bo Young Cho, Young Joo Park, Hang-Seok Chang
BACKGROUND: Hereditary medullary thyroid carcinoma can present as a part of multiple endocrine neoplasia syndrome by rearranged during transfection gene mutation. We evaluated the prevalence of rearranged during transfection gene mutation in patients who have medullary thyroid carcinoma and the correlations of genotype with medullary thyroid carcinoma, pheochromocytoma, and hyperparathyroidism according to the revised American Thyroid Association risk level. METHODS: A total of 331 patients were diagnosed with medullary thyroid carcinoma, 172 of whom were tested for the rearranged during transfection germline mutation by sequencing of exon 8, 10, 11, and 13-16...
May 17, 2018: Surgery
https://www.readbyqxmd.com/read/29779206/old-new-and-emerging-immunohistochemical-markers-in-pheochromocytoma-and-paraganglioma
#5
REVIEW
Veronica K Y Cheung, Anthony J Gill, Angela Chou
The evolution of genetic research over the past two decades has greatly improved the understanding of pheochromocytomas and paragangliomas. It is now accepted that more than one third of pheochromocytoma and paragangliomas arise in the context of syndromic disease, usually hereditary. The genetic profile of these tumors also has important prognostic implications which may help guide treatment. Accompanying the changing molecular landscape is the development of new immunohistochemical markers. Initially used in assisting with diagnosis, immunohistochemical markers have now become an important adjunct to screening programs for inherited conditions and subsequently as prognostic markers...
May 19, 2018: Endocrine Pathology
https://www.readbyqxmd.com/read/29779047/over-diagnosis-of-potential-malignant-behavior-in-men-2a-associated-pheochromocytomas-using-the-pass-and-gapp-algorithms
#6
Adam Stenman, Jan Zedenius, Carl Christofer Juhlin
PURPOSE: Pheochromocytomas (PCCs) exhibit malignant potential, but current histological modalities for the proper detection of aggressive behavior are debated. The two most widespread algorithms are the "Pheochromocytoma of the Adrenal Gland Scaled Score" (PASS) and the "Grading System for Adrenal Pheochromocytoma and Paraganglioma" (GAPP), both which mostly rely on histological parameters to identify PCC patients at risk of disseminated disease. Since the algorithms are derived from studies using predominantly sporadic PCCs, little is known whether the PASS or GAPP scores can predict malignant potential in hereditary cases...
May 19, 2018: Langenbeck's Archives of Surgery
https://www.readbyqxmd.com/read/29768630/what-determines-mortality-in-malignant-pheochromocytoma-report-of-a-case-with-eighteen-year-survival-and-review-of-the-literature
#7
Matheus de Oliveira Andrade, Vinícius Santos da Cunha, Dayana Carla de Oliveira, Olívia Laquis de Moraes, Adriana Lofrano-Porto
Pheochromocytoma (PCC) is a tumor derived from adrenomedullary chromaffin cells. Prognosis of malignant PCC is generally poor due to local recurrence or metastasis. We aim to report a case of malignant PCC with 18-year survival and discuss which factors may be related to mortality and long-term survival in malignant pheochromocytoma. The patient, a 45-year-old man, reported sustained arterial hypertension with paroxysmal episodes of tachycardia, associated with head and neck burning sensation, and hand and foot tremors...
March 2018: Archives of Endocrinology and Metabolism
https://www.readbyqxmd.com/read/29768188/micropatterned-biodegradable-polyesters-clicked-with-cqaasikvav-promote-cell-alignment-directional-migration-and-neurite-outgrowth
#8
Deteng Zhang, Sai Wu, Jianyong Feng, Yiyuan Duan, Dongming Xing, Changyou Gao
The interplay of microstructures and biological cues is critical to regulate the behaviors of Schwann cells (SCs) in terms of cellular spatial arrangement and directional migration as well as neurite orientation for bridging the proximal and distal stumps of the injured peripheral nervous system. In this study, stripe micropatterns having ridges/grooves of width 20/20 and 20/40 μm were fabricated on the surface of maleimide-functionalized biodegradable poly(ester carbonate) (P(LLA-MTMC)) films by the polydimethylsiloxane mold-pressing method, respectively...
May 13, 2018: Acta Biomaterialia
https://www.readbyqxmd.com/read/29764675/extracorporeal-membrane-oxygenation-support-in-a-patient-with-pheochromocytoma-stress-myocardyopathy
#9
Luis Martin-Villen, Yael Corcia-Palomo, Sara Escalona-Rodriguez, Alvaro Roldan-Reina, Domingo Acosta-Delgado, Rafael Martin-Bermudez
No abstract text is available yet for this article.
May 12, 2018: Medicina Intensiva
https://www.readbyqxmd.com/read/29760304/metyrapone-responsive-ectopic-acth-secreting-pheochromocytoma-with-a-vicious-cycle-via-a-glucocorticoid-driven-positive-feedback-mechanism
#10
Minako Inoue, Ken Okamura, Chie Kitaoka, Fumio Kinoshita, Ryo Namitome, Udai Nakamura, Masaki Shiota, Kenichi Goto, Toshio Ohtsubo, Kiyoshi Matsumura, Yoshinao Oda, Masatoshi Eto, Takanari Kitazono
In ectopic ACTH-secreting pheochromocytoma, combined ACTH-driven hypercortisolemia and hyper-catecholaminemia are serious conditions, which can be fatal if not diagnosed and managed appropriately, especially when glucocorticoid-driven positive feedback is suggested with a high ACTH/cortisol ratio. A 46-year-old man presented with headache, rapid weight loss, hyperhidrosis, severe hypertension and hyperglycemia without typical Cushingoid appearance. Endocrinological examinations demonstrated elevated plasma and urine catecholamines, serum cortisol and plasma ACTH...
May 15, 2018: Endocrine Journal
https://www.readbyqxmd.com/read/29755915/unusual-long-survival-with-a-giant-invasive-pheochromocytoma-of-an-incompatible-patient
#11
Asli Nar
Pheochromocytomas (PHEOs) are rare neuroendocrine tumors and about 2-13% of PHEOs are malignant. Predicting malignancy in PHEO cases with invasion but without metastasis is still controversial in the literature. This study presents an unusual long survival with a giant invasive PHEO in an incompatible patient and a review of the literature. In 1989, a 23-year-old female patient was operated for a giant adrenal mass with a pathological final diagnosis of PHEO. Information to the patient's family was provided about the short life span of the patient in the postoperative period because the tumor could not be totally resected...
March 13, 2018: Curēus
https://www.readbyqxmd.com/read/29755524/a-clinical-roadmap-to-investigate-the-genetic-basis-of-pediatric-pheochromocytoma-which-genes-should-physicians-think-about
#12
REVIEW
Bernardo Dias Pereira, Tiago Nunes da Silva, Ana Teresa Bernardo, Rui César, Henrique Vara Luiz, Karel Pacak, Luísa Mota-Vieira
Pheochromocytoma is very rare at a pediatric age, and when it is present, the probability of a causative genetic mutation is high. Due to high costs of genetic surveys and an increasing number of genes associated with pheochromocytoma, a sequential genetic analysis driven by clinical and biochemical phenotypes is advised. The published literature regarding the genetic landscape of pediatric pheochromocytoma is scarce, which may hinder the establishment of genotype-phenotype correlations and the selection of appropriate genetic testing at this population...
2018: International Journal of Endocrinology
https://www.readbyqxmd.com/read/29753028/pituitary-somatolactotropes-evade-an-oncogenic-response-to-ras
#13
Allyson K Roof, Tammy Trudeau, Arthur Gutierrez-Hartmann
Distinct cell types have been shown to respond to activated Ras signaling in a cell-specific manner. In contrast to its pro-tumorigenic role in some human epithelial cancers, oncogenic Ras triggers differentiation of pheochromocytoma cells and medullary thyroid carcinoma cells. Furthermore, we have previously demonstrated that in pituitary somatolactotropes, activated Ras promotes differentiation and is not sufficient to drive tumorigenesis. These findings demonstrate that lactotrope cells have the ability to evade the tumorigenic fate that is often associated with persistent activation of Ras/ERK signaling, and suggest that there may be differential expression of inhibitory signaling molecules or negative cell cycle regulators that act as a brake to prevent the tumorigenic effects of sustained Ras signaling...
May 9, 2018: Molecular and Cellular Endocrinology
https://www.readbyqxmd.com/read/29749133/characteristics-of-adrenal-incidentalomas-in-a-large-prospective-computed-tomography-based-multicenter-study-the-coar-study-in-korea
#14
Seong Hee Ahn, Jae Hyeon Kim, Seung Hee Baek, Hyeonmok Kim, Yoon Young Cho, Sunghwan Suh, Beom Jun Kim, Seongbin Hong, Jung Min Koh, Seung Hun Lee, Kee Ho Song
PURPOSE: Previous studies on adrenal incidentalomas (AIs) are limited by their retrospective design, small numbers of patients, Western populations, or use of an outdated imaging technique. We investigated the characteristics of AIs in Korean patients and compared them with those reported in the largest retrospective study in Italy to discover the effects of improved imaging techniques and ethnicity differences. MATERIALS AND METHODS: This was a prospective, multicenter, cross-sectional observational study including 1005 Korean patients...
June 2018: Yonsei Medical Journal
https://www.readbyqxmd.com/read/29748231/management-of-endocrine-disease-differential-diagnosis-investigation-and-therapy-of-bilateral-adrenal-incidentalomas
#15
Isabelle Bourdeau, Nada El Ghorayeb, Nadia Gagnon, Andre Lacroix
The investigation and management of unilateral adrenal incidentalomas have been extensively considered in the last decades. While bilateral adrenal incidentalomas represent about 15 percent of adrenal incidentalomas, they have been less frequently discussed. The differential diagnosis of bilateral incidentalomas includes metastasis, primary bilateral macronodular adrenal hyperplasia (BMAH) and bilateral cortical adenomas. Les frequent etiologies are bilateral pheochromocytomas, congenital adrenal hyperplasia, Cushing's disease or ectopic ACTH secretion with secondary bilateral adrenal hyperplasia, primary malignancies, myelolipomas, infections or hemorrhage...
May 10, 2018: European Journal of Endocrinology
https://www.readbyqxmd.com/read/29747617/pheochromocytoma-as-a-rare-cause-of-hypertension-in-a-46-x-i-x-q10-turner-syndrome-a-case-report-and-literature-review
#16
Ji Yeon Shin, Bo Hyun Kim, Young Keum Kim, Tae Hwa Kim, Eun Heui Kim, Min Jin Lee, Jong Ho Kim, Yun Kyung Jeon, Sang Soo Kim, In Joo Kim
BACKGROUND: Cardiovascular disease (CVD) presents the most serious health problems and contributes to the increased mortality in young women with Turner syndrome. Arterial hypertension in Turner syndrome patients is significantly more prevalent than that in a general age-matched control group. The aetiology of hypertension in Turner syndrome varies, even in the absence of cardiac anomalies and obvious structural renal abnormalities. Pheochromocytoma is an extremely rare cause among various etiologies for hypertension in patients with Turner syndrome...
May 10, 2018: BMC Endocrine Disorders
https://www.readbyqxmd.com/read/29742697/giant-malignant-pheochromocytoma-in-an-elderly-patient-a-case-report
#17
Chengquan Ma, Erlin Sun, Bingxin Lu
RATIONALE: Malignant pheochromocytoma is a rare disease and surgical resection is the only curative treatment. PATIENT CONCERNS: An 81-year-old man of Chinese ethnicity was found to have a giant retroperitoneal tumor. DIAGNOSES: B-scan ultrasonography and CT scan presented a mass above the left kidney, measuring 13.5 × 10 .6 × 9.8 cm. Subsequent analysis of 24-h urinary catecholamines and vanillylmandelic acid, as well as of blood catecholamines and blood cortisol, showed no elevated levels...
May 2018: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29740169/gain-of-function-mutations-in-dnmt3a-in-patients-with-paraganglioma
#18
Laura Remacha, Maria Currás-Freixes, Raúl Torres-Ruiz, Francesca Schiavi, Rafael Torres-Pérez, Bruna Calsina, Rocío Letón, Iñaki Comino-Méndez, Juan M Roldán-Romero, Cristina Montero-Conde, María Santos, Lucía Inglada Pérez, Guillermo Pita, María R Alonso, Emiliano Honrado, Susana Pedrinaci, Benedicto Crespo-Facorro, Antonio Percesepe, Maurizio Falcioni, Sandra Rodríguez-Perales, Esther Korpershoek, Santiago Ramón-Maiques, Giuseppe Opocher, Cristina Rodríguez-Antona, Mercedes Robledo, Alberto Cascón
PURPOSE: The high percentage of patients carrying germline mutations makes pheochromocytomas/paragangliomas the most heritable of all tumors. However, there are still cases unexplained by mutations in the known genes. We aimed to identify the genetic cause of disease in patients strongly suspected of having hereditary tumors. METHODS: Whole-exome sequencing was applied to the germlines of a parent-proband trio. Genome-wide methylome analysis, RNA-seq, CRISPR/Cas9 gene editing, and targeted sequencing were also performed...
May 8, 2018: Genetics in Medicine: Official Journal of the American College of Medical Genetics
https://www.readbyqxmd.com/read/29730012/evaluation-of-chemical-property-cytotoxicity-and-antioxidant-activity-in-vitro-and-in-vivo-of-polysaccharides-from-fuzhuan-brick-teas
#19
Guijie Chen, Mingjia Wang, Minhao Xie, Peng Wan, Dan Chen, Bing Hu, Hong Ye, Xiaoxiong Zeng, Zhonghua Liu
Fuzhuan brick tea (FBT) possesses various health-promoting functions. However, the available information regarding biological activity of polysaccharides from FBT (FBTPS) is still limited. In this work, the chemical property, cytotoxicity and antioxidant activity in vitro and in vivo of FBTPS were evaluated. It was found that FBTPSs were typical acidic heteropolysaccharides mainly composed of Man, Rha, GalA, Glc, Gal and Ara with little molar content of Rib and GlcA. FBTPS showed little toxicity to human hepatic epithelial (L-02) cell...
May 3, 2018: International Journal of Biological Macromolecules
https://www.readbyqxmd.com/read/29720885/successful-second-line-metronomic-temozolomide-in-metastatic-paraganglioma-case-reports-and-review-of-the-literature
#20
Isabel Tena, Garima Gupta, Marcos Tajahuerce, Marta Benavent, Manuel Cifrián, Alejandro Falcon, María Fonfria, Maribel Del Olmo, Rosa Reboll, Antonio Conde, Francisca Moreno, Julia Balaguer, Adela Cañete, Rosana Palasí, Pilar Bello, Alfredo Marco, José Luis Ponce, Juan Francisco Merino, Antonio Llombart, Alfredo Sanchez, Karel Pacak
Metastatic pheochromocytoma and paraganglioma (mPHEO/PGL) are frequently associated with succinate dehydrogenase B ( SDHB ) mutations. Cyclophosphamide-dacarbazine-vincristine (CVD) regimen is recommended as standard chemotherapy for advanced mPHEO/PGL. There is limited evidence to support the role of metronomic schemes (MS) of chemotherapy in mPHEO/PGL treatment. We report 2 patients with SDHB -related mPGL who received a regimen consisting of MS temozolomide (TMZ) and high-dose lanreotide after progression on both CVD chemotherapy and high-dose lanreotide...
2018: Clinical Medicine Insights. Oncology
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