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https://www.readbyqxmd.com/read/29678284/aberrant-g-protein-coupled-hormone-receptor-in-adrenal-diseases
#1
REVIEW
Matthieu St-Jean, Nada El Ghorayeb, Isabelle Bourdeau, André Lacroix
The regulation of cortisol or aldosterone production when ACTH of pituitary origin or the renin-angiotensin systems are suppressed in primary adrenal Cushing's syndrome or in primary aldosteronism is exerted by diverse genetic and molecular mechanisms. In addition to recently identified mutations in various genes implicated in the cyclic AMP or ion channel pathways, steroidogenesis is not really autonomous as it is frequently regulated by the aberrant adrenocortical expression of diverse hormone receptors, particularly G-protein coupled hormone receptors (GPCR) which can substitute for the normal function of ACTH or angiotensin-II...
April 2018: Best Practice & Research. Clinical Endocrinology & Metabolism
https://www.readbyqxmd.com/read/29675278/adrenocorticotropic-hormone-secreting-pheochromocytoma-underlying-glucocorticoid-induced-pheochromocytoma-crisis
#2
Gil A Geva, David J Gross, Haggi Mazeh, Karine Atlan, Iddo Z Ben-Dov, Matan Fischer
Context: Pheochromocytomas are hormone secreting tumors of the medulla of the adrenal glands found in 0.1-0.5% of patients with hypertension. The vast majority of pheochromocytomas secrete catecholamines, but they have been occasionally shown to also secrete interleukins, calcitonin, testosterone, and in rare cases adrenocorticotropic hormone. Pheochromocytoma crisis is a life threatening event in which high levels of catecholamines cause a systemic reaction leading to organ failure. Case Description: A 70-year-old man was admitted with acute myocardial ischemia following glucocorticoid administration as part of an endocrine workup for an adrenal mass...
2018: Case Reports in Endocrinology
https://www.readbyqxmd.com/read/29671009/interobserver-agreement-in-distinguishing-large-adrenal-adenomas-and-adrenocortical-carcinomas-on-computed-tomography
#3
Aaron J Thomas, Mouhammed A Habra, Priya R Bhosale, Aliya A Qayyum, Kareem Ahmed, Rafael Vicens, Khaled M Elsayes
PURPOSE: Large adrenal masses pose a diagnostic dilemma. The purpose of this study was twofold: first, to assess the degree of interobserver agreement in evaluating the morphology of pathologically proven adrenal adenomas and adrenocortical carcinomas larger than 4 cm in diameter; and second, to identify morphologic characteristics that correlated with the pathologic diagnosis. MATERIALS AND METHODS: For this blinded, retrospective study, we collected cases of 25 adrenal adenomas and 33 adrenocortical carcinomas measuring larger than 4 cm...
April 19, 2018: Abdominal Radiology
https://www.readbyqxmd.com/read/29669941/activating-prkacb-somatic-mutation-in-cortisol-producing-adenomas
#4
Stéphanie Espiard, Matthias J Knape, Kerstin Bathon, Guillaume Assié, Marthe Rizk-Rabin, Simon Faillot, Windy Luscap-Rondof, Daniel Abid, Laurence Guignat, Davide Calebiro, Friedrich W Herberg, Constantine A Stratakis, Jérôme Bertherat
Mutations in the gene encoding the protein kinase A (PKA) catalytic subunit α have been found to be responsible for cortisol-producing adenomas (CPAs). In this study, we identified by whole-exome sequencing the somatic mutation p.S54L in the PRKACB gene, encoding the catalytic subunit β (Cβ) of PKA, in a CPA from a patient with severe Cushing syndrome. Bioluminescence resonance energy transfer and surface plasmon resonance assays revealed that the mutation hampers formation of type I holoenzymes and that these holoenzymes were highly sensitive to cAMP...
April 19, 2018: JCI Insight
https://www.readbyqxmd.com/read/29662268/a-pediatric-case-of-pheochromocytoma-without-apparent-hypertension-associated-with-von-hippel-lindau-disease
#5
Junko Igaki, Akira Nishi, Takeshi Sato, Tomonobu Hasegawa
Pheochromocytomas are catecholamine-secreting tumors. These tumors are rare in children, and they may be associated with hereditary syndromes such as von Hippel-Lindau (VHL) disease. Most pediatric patients with pheochromocytoma present with sustained hypertension, while 10% to 69% of adult patients are asymptomatic. Herein, we present the case of a 12-yr-old Japanese girl with pheochromocytoma due to a germline mutation in the VHL (Arg161Gln). The only complaint was loss of weight. Pyrexia, anemia, and increases in C-reactive protein (CRP) and ferritin were observed...
2018: Clinical Pediatric Endocrinology: Case Reports and Clinical Investigations: Official Journal of the Japanese Society for Pediatric Endocrinology
https://www.readbyqxmd.com/read/29661460/partial-cystectomy-of-paraganglioma-of-the-urinary-bladder-before-living-kidney-transplantation-case-report
#6
K Kido, S Hatakeyama, I Hamano, H Yamamoto, A Imai, T Yoneyama, Y Hashimoto, T Koie, T Fujita, R Murakami, H Tomita, T Suzuki, S Narumi, C Ohyama
BACKGROUND: Paraganglioma (extra-adrenal pheochromocytoma) of the bladder is a very rare disease, accounting for 0.06% of all bladder tumors. Optimal management of bladder paraganglioma before kidney transplantation is unknown. We report a case of partial cystectomy for urinary bladder paraganglioma before living kidney transplantation. CASE PRESENTATION: A 59-year-old man with a 27-year history of hemodialysis was referred to our department for further examination of a bladder tumor detected during pre-transplantation testing...
April 2018: Transplantation Proceedings
https://www.readbyqxmd.com/read/29660928/adrenal-schwannoma-ct-mr-manifestations-and-pathological-correlation
#7
Wei Tang, Xiang-Rong Yu, Liang-Ping Zhou, Hong-Bo Gao, Qi-Feng Wang, Wei-Jun Peng
OBJECTIVES: This study aimed to describe the computed tomography (CT), magnetic resonance imaging (MRI) imaging features of adrenal schwannoma and to correlate imaging findings with histopathologic findings. METHODS: The findings from multiphase CT or MRI examinations of seventeen patients with histopathologically confirmed adrenal schwannoma were reviewed. The imaging criteria included shape, size, margin, attenuation, signal intensity, secondary degeneration, and internal mass enhancement pattern...
2018: Clinical Hemorheology and Microcirculation
https://www.readbyqxmd.com/read/29659392/pedunculated-hepatocellular-carcinoma-mimicking-right-adrenal-tumor-on-fdg-pet-ct
#8
Aisheng Dong, Xinping Zhong, Yang Wang
Pedunculated hepatocellular carcinoma is an unusual form of hepatocellular carcinoma protruding from the liver with or without a pedicle. We present a case of pedunculated hepatocellular carcinoma misdiagnosed as right adrenal tumor on MRI and FDG PET/CT. Intraoperative exploration revealed the mass was attached to the liver, but the right adrenal gland was intact. Laparoscopic biopsy revealed poorly differentiated hepatocellular carcinoma. This case indicates it may be difficult to diagnose pedunculated hepatocellular carcinoma because it is hard to define its origin on imaging...
April 13, 2018: Clinical Nuclear Medicine
https://www.readbyqxmd.com/read/29653114/adrenal-androgen-measurement-for-assessing-the-selectivity-of-adrenal-venous-sampling-in-primary-aldosteronism
#9
Hongyun Li, Xuebin Zhang, Shanmei Shen, Yan Zhang, Wenjing Zhang, Wenhuan Feng, Ping Li, Dalong Zhu
Adrenal venous sampling (AVS) is the reference standard for primary aldosteronism (PA) subtyping. Cortisol is widely used to assess the success of selective AVS, but it is not always reliable. The aim of the present study was to investigate the usefulness of adrenal androgens, compared to cortisol, in assessing the selectivity of AVS. We consecutively recruited 37 patients with PA undergoing AVS. AVS procedures were performed with the bilateral simultaneous technique without cosyntropin stimulation. We collected two baseline blood samples from 10 patients with a 15-min interval between time -15 (t-15) and time 0 (t0) to measure the variability in the levels of the tested hormones over time...
April 10, 2018: Steroids
https://www.readbyqxmd.com/read/29651813/contralateral-adrenal-width-predicts-the-duration-of-prolonged-post-surgical-steroid-replacement-for-subclinical-cushing-syndrome
#10
Masahiro Sugiura, Yusuke Imamura, Koji Kawamura, Satoshi Yamamoto, Tomokazu Sazuka, Kazuyoshi Nakamura, Shinichi Sakamoto, Hidekazu Nagano, Hisashi Koide, Tomoaki Tanaka, Takashi Imamoto, Akira Komiya, Tomohiko Ichikawa
OBJECTIVES: To identify pre-treatment factors affecting the duration of post-surgical steroid replacement in patients undergoing adrenalectomy for subclinical Cushing syndrome. METHODS: The present retrospective analysis included 64 patients who underwent unilateral laparoscopic adrenalectomy for subclinical Cushing syndrome. Adrenal tumor and contralateral adrenal sizes together with various clinical factors were studied in association with the duration of post-surgical steroid replacement...
April 12, 2018: International Journal of Urology: Official Journal of the Japanese Urological Association
https://www.readbyqxmd.com/read/29649281/classification-for-long-term-survival-in-oligometastatic-patients-treated-with-ablative-radiotherapy-a-multi-institutional-pooled-analysis
#11
Julian C Hong, Diandra N Ayala-Peacock, Jason Lee, A William Blackstock, Paul Okunieff, Max W Sung, Ralph R Weichselbaum, Johnny Kao, James J Urbanic, Michael T Milano, Steven J Chmura, Joseph K Salama
BACKGROUND: Radiotherapy is increasingly used to treat oligometastatic patients. We sought to identify prognostic criteria in oligometastatic patients undergoing definitive hypofractionated image-guided radiotherapy (HIGRT). METHODS: Exclusively extracranial oligometastatic patients treated with HIGRT were pooled. Characteristics including age, sex, primary tumor type, interval to metastatic diagnosis, number of treated metastases and organs, metastatic site, prior systemic therapy for primary tumor treatment, prior definitive metastasis-directed therapy, and systemic therapy for metastasis associated with overall survival (OS), progression-free survival (PFS), and treated metastasis control (TMC) were assessed by the Cox proportional hazards method...
2018: PloS One
https://www.readbyqxmd.com/read/29645065/-131-i-mibg-therapy-of-malignant-pheochromocytoma-and-paraganglioma-tumors-a-single-center-study
#12
Agnieszka Kotecka-Blicharz, Kornelia Hasse-Lazar, Daria Handkiewicz-Junak, Tomasz Gawlik, Agnieszka Pawlaczek, Małgorzata Oczko-Wojciechowska, Barbara Michalik, Sylwia Szpak-Ulczok, Jolanta Krajewska, Beata Jurecka-Lubieniecka, Barbara Jarząb
INTRODUCTION: Pheochromocytomas and paragangliomas are rare tumors deriving from chromaffin cells of adrenal medulla or paraganglia. They are usually benign but 10-35% of them present malignant behavior. The aim of the study was to evaluate the efficacy and safety of 131-I MIBG therapy in malignant pheochromocytoma /paraganglioma patients (MPPGL). MATERIAL AND METHODS: 18 patients (7 women and 11 men) were included in this study. Between 2002 and 2016 they underwent 131-I MIBG therapy because of MPPGL and their medical data were analyzed retrospectively...
April 12, 2018: Endokrynologia Polska
https://www.readbyqxmd.com/read/29644077/unusual-presentation-of-pheochromocytoma-thirteen-years-of-anxiety-requiring-psychiatric-treatment
#13
Catherine Alguire, Jessica Chbat, Isabelle Forest, Ariane Godbout, Isabelle Bourdeau
Pheochromocytoma is a rare tumor of the adrenal gland. It often presents with the classic triad of headache, palpitations and generalized sweating. Although not described as a typical symptom of pheochromocytoma, anxiety is the fourth most common symptom reported by patients suffering of pheochromocytoma. We report the case of a 64 year old man who had severe anxiety and panic disorder as presenting symptoms of pheochromocytoma. After 13 years of psychiatric follow-up, the patient was diagnosed with malignant pheochromocytoma...
2018: Endocrinology, Diabetes & Metabolism Case Reports
https://www.readbyqxmd.com/read/29644041/paraganglioma-or-pheochromocytoma-a-peculiar-diagnosis
#14
Ricardo Rodrigues Marques, Carlos Tavares Bello, Ana Alves Rafael, Luís Viana Fernandes
Paragangliomas and pheochromocytomas are rare catecholamine secreting neoplasms that arise in the extra-adrenal autonomic paraganglia and adrenal medulla, respectively. Although typically presenting with paroxysms of headaches, palpitations, diaphoresis and hypertension, a broad spectrum of clinical manifestations may occur. Diagnosis relies on biochemical studies followed by adequate imaging investigation. Cross sectional morphological and functional imaging modalities have improved diagnostic accuracy and are crucial in the surgical planning...
April 2018: Journal of Surgical Case Reports
https://www.readbyqxmd.com/read/29644036/pulmonary-paraganglioma-in-a-10-year-old-a-case-report-and-review-of-the-literature
#15
Tracy R Geoffrion, Linde DeKeyzer, Gopi Shah, Suja J Nair, Timothy J Pirolli, Kemp Kernstine
Paraganglioma is a rare extra-adrenal tumor of the paraganglia often found in association with sympathetic and parasympathetic nerves. The case presented is of a 10-year-old boy with hemoptysis who was found to have an obstructive bronchial mass. He underwent surgical resection and biopsy confirmed primary pulmonary paraganglioma. He was subsequently found to have an associated genetic syndrome. This is the first case report describing a primary pulmonary paraganglioma in a child.
April 2018: Journal of Surgical Case Reports
https://www.readbyqxmd.com/read/29643654/role-of-endoscopic-ultrasound-guided-fine-needle-aspiration-in-adrenal-lesions-analysis-of-32-patients
#16
Rakesh K Gupta, Kaushik Majumdar, Ravindra K Saran, Siddharth Srivastava, Puja Sakhuja, Vineeta V Batra
Objective: Endoscopic ultrasound-guided fine-needle aspiration cytology (EUS-FNAC) is a precise and safe technique that provides both radiological and pathological diagnosis with a better diagnostic yield and minimal adverse events. EUS-FNAC led to the remarkable increase in the detection rate of incidentaloma found during radiologic staging or follow-up in various malignancy or unrelated conditions. Aims: We did this preliminary study with an aim to evaluate the role of EUS-FNA in diagnosing and classifying adrenal lesions, clinical impact, and compare the outcome with the previously published literature...
April 2018: Journal of Cytology
https://www.readbyqxmd.com/read/29642543/comparative-genomics-and-transcriptome-profiling-in-primary-aldosteronism
#17
REVIEW
Elke Tatjana Aristizabal Prada, Isabella Castellano, Eva Sušnik, Yuhong Yang, Lucie S Meyer, Martina Tetti, Felix Beuschlein, Martin Reincke, Tracy A Williams
Primary aldosteronism is the most common form of endocrine hypertension with a prevalence of 6% in the general population with hypertension. The genetic basis of the four familial forms of primary aldosteronism (familial hyperaldosteronism FH types I-IV) and the majority of sporadic unilateral aldosterone-producing adenomas has now been resolved. Familial forms of hyperaldosteronism are, however, rare. The sporadic forms of the disease prevail and these are usually caused by either a unilateral aldosterone-producing adenoma or bilateral adrenal hyperplasia...
April 9, 2018: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/29629945/clonal-evolution-in-primary-diffuse-large-b-cell-lymphoma-of-the-central-nervous-system
#18
Julia Garcia-Reyero, Nerea Martinez Magunacelaya, Ainara Gonzalez Pereña, Sara Marcos Gonzalez, Nuria Teran-Villagra, Ainara Azueta, Ana Batlle, Sonia Gonzalez de Villambrosia, Jose Revert Arce, Santiago Montes-Moreno
Primary diffuse large B-cell lymphoma (DLBCL) of the central nervous system (CNS) is an aggressive subtype of DLBCL with characteristic clinicopathologic features. Relapse outside the CNS involving extranodal locations has been found in a fraction of cases (16%). Here we describe a case of DLBCL arising in the CNS that relapsed 18 months after the initial diagnosis in the testis and bilateral adrenal glands. Both tumors showed equivalent morphology, phenotype, cytogenetic features, and clonal relationship. Somatic mutation analysis by next generation sequencing demonstrated MYD88L265P mutation in both tumors and de novo CD79B Y196S mutation exclusive to the relapse...
April 7, 2018: Applied Immunohistochemistry & Molecular Morphology: AIMM
https://www.readbyqxmd.com/read/29627576/magnolol-abrogates-chronic-mild-stress-induced-depressive-like-behaviors-by-inhibiting-neuroinflammation-and-oxidative-stress-in-the-prefrontal-cortex-of-mice
#19
Jie Cheng, Shuqi Dong, Litao Yi, Di Geng, Qing Liu
Magnolol, the main constituent of Magnolia officinalis, has been shown to produce antidepressant-like effect in rodents. Growing evidence shows that neuroinflammation, oxidative stress and neuroendocrine contribute to the pathogenesis of major depression. Here, the aim of this present study was to determine whether magnolol affected these systems in mice exposed to chronic mild stress (CMS). The ameliorative effect of magnolol on depressive-like symptoms was investigated through behavioral tests, including the classical sucrose preference and forced swimming tests...
April 5, 2018: International Immunopharmacology
https://www.readbyqxmd.com/read/29626607/prevalence-clinical-characteristics-and-long-term-outcomes-of-classical-11-%C3%AE-hydroxylase-deficiency-11bohd-in-turkish-population-and-novel-mutations-in-cyp11b1-gene
#20
Firdevs Baş, Güven Toksoy, Berrin Ergun-Longmire, Zehra Oya Uyguner, Zehra Yavaş Abalı, Şükran Poyrazoğlu, Volkan Karaman, Şahin Avcı, Umut Altunoğlu, Ruveyde Bundak, Birsen Karaman, Seher Başaran, Feyza Darendeliler
Congenital adrenal hyperplasia (CAH) due to 11β-hydroxylase deficiency (11BOHD) is a rare autosomal recessive disorder and the second most common form of CAH. AIM: To investigate genotype-phenotype correlation and to evaluate clinical characteristics and long-term outcomes of patients with 11BOHD. METHODS: A total of 28 patients (n = 14, 46,XX; n = 14, 46,XY) with classical 11BOHD from 25 unrelated families were included in this study. Screening of CYP11B1 is performed by Sanger sequencing...
April 4, 2018: Journal of Steroid Biochemistry and Molecular Biology
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