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adrenocortical adenoma

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https://www.readbyqxmd.com/read/28199934/mixed-corticomedullary-adrenal-carcinoma-case-report-comparison-in-features-treatment-and-prognosis-with-the-other-two-reported-cases
#1
Mhd Belal Alsabek, Riad Alhmaidi, Bader Ghazzawi, Ghiath Hamed, Alhadi Alseoudi
INTRODUCTION: Adrenal corticomedullary adenoma was reviewed in many cases in PubMed Library, While the coincidence corticomedullary adrenal carcinoma in the same gland was just described in two cases in the medical literature. Our case is the third to be reported and was treated with surgery and adjuvant chemotherapy and followed for two years. PRESENTATION OF CASE: A 50-year-old man suffered from a mass effect in the left abdominal side. While the laboratory showed a mild elevation in the levels of both serum cortisol and 24h urine cortisol, radiological images were highly suggested an adrenal malignant tumor without metastasis...
January 31, 2017: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/28181818/recommendations-for-the-management-of-adrenal-incidentalomas-what-is-pertinent-for-radiologists
#2
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/28179451/cushing-s-syndrome-in-children-and-adolescents-a-danish-nationwide-population-based-cohort-study
#3
Johanne Marie Holst, Erzsébet Horváth-Puhó, Rb Jensen, Mariane Rix, Niels Thomas Hertel, Olaf Dekkers, Henrik Toft Sørensen, Anders Juul, Jens Otto L Jorgensen
OBJECTIVE: Cushing's syndrome (CS) affects all age groups, but epidemiologic data in young pa-tients are very limited. We therefore examined the incidence, prevalence, and hospital morbidity of CS in children and adolescents. DESIGN: In a nationwide cohort study we included all Danish citizens aged 0-20 years from 1977 to 2012. Data were obtained from the Danish National Patient Registry using the International Classi-fication of Diseases (ICD) codes and the Danish Civil Registration System...
February 8, 2017: European Journal of Endocrinology
https://www.readbyqxmd.com/read/28170340/adrenocortical-neoplasms-in-adulthood-and-childhood-distinct-presentation-review-of-the-clinical-pathological-and-imaging-characteristics
#4
Andréa Farias de Melo-Leite, Paula Condé Lamparelli Elias, Sara Reis Teixeira, Silvio Tucci, Gyl Eanes Barros, Sonir R Antonini, Valdair Francisco Muglia, Jorge Elias
Adrenocortical tumors (ACT) in adulthood and childhood vary in clinical, histopathological, molecular, prognostic, and imaging aspects. ACT are relatively common in adults, as adenomas are often found incidentally on imaging. ACT are rare in children, though they have a significantly higher prevalence in the south and southeast regions of Brazil. In clinical manifestation, adults with ACT present more frequently with glucocorticoid overproduction (Cushing syndrome), mineralocorticoid syndromes (Conn syndrome), or the excess of androgens in women...
February 7, 2017: Journal of Pediatric Endocrinology & Metabolism: JPEM
https://www.readbyqxmd.com/read/28102088/hyperandrogenism-in-a-postmenopausal-woman-a-rare-case-of-ectopic-adrenal-cortical-gland
#5
Antonella Guarino, Luisa Di Benedetto, Valentina Giovanale, Gian Luca Rampioni Vinciguerra, Antonella Stoppacciaro, Filippo Bellati, Donatella Caserta
Most frequent causes of androgenic manifestation are Cushing's syndrome, PCO, benign and malignant androgen-secreting non adrenal tumors and iatrogenic hirsutism. Hyperplasia or neoplasms of ectopic adrenocortical gland are rare. We report a case of a 63-year old female with hirsutism and alopecia. Laboratory data highlighted increased levels of androgens. Diagnostic imaging revealed normal morphology of adrenocortical gland and ovaries. In view of the clinical picture and suspected diagnosis of extra-adrenal cause, she underwent bilateral salpingo-oophorectomy...
January 19, 2017: Gynecological Endocrinology
https://www.readbyqxmd.com/read/28101185/hypokalemic-myopathy-in-primary-aldosteronism-a-case-report
#6
Chuifen Wu, Jun Xin, Minghua Xin, Hai Zou, Lie Jing, Caoyong Zhu, Wenhui Lei
Primary aldosteronism (PA) is a rare disorder. The majority of patients with PA present with typical features and are easily diagnosed. This disorder is usually diagnosed with hypokalemia, hypertension or an adrenal mass. However, patients with atypical symptoms may present a challenge for diagnosis and treatment. In the present study, a case of PA is described that presented with hypokalemic myopathy simulating polymyositis. The patient was a 44-year-old woman who presented with weakness and difficulty walking...
December 2016: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/28095038/pure-androgen-producing-adrenal-tumor-clinical-features-and-pathogenesis
#7
Anli Tong, Jun Jiang, Fen Wang, Chunyan Li, Yushi Zhang, Xueyan Wu
OBJECTIVE: Pure androgen-secreting adrenal tumors (PASATs) are extremely rare, most reports involving only a single case. This study examined 9 cases of PASAT, with an attempt to characterize its clinical features and to explore their possible pathogenesis. METHODS: Clinical data of 9 patients with PASAT were retrospectively reviewed. Immunostaining were conducted, and Aryl hydrocarbon receptor-interacting protein (AIP) were amplified and directly sequenced. RESULTS: The onset age of the patients ranged from 3...
January 17, 2017: Endocrine Practice
https://www.readbyqxmd.com/read/28087475/immunohistochemical-approach-for-the-diagnosis-of-a-liver-mass-on-small-biopsy-specimens
#8
Won-Tak Choi, Rageshree Ramachandran, Sanjay Kakar
Well-differentiated hepatocellular carcinoma (HCC) shares overlapping histological features with benign hepatocellular lesions, including hepatocellular adenoma (HCA) and focal nodular hyperplasia (FNH) in non-cirrhotic liver, and with high-grade dysplastic nodule (HGDN) in cirrhotic liver. Several metastatic tumors, such as neuroendocrine tumor, renal cell carcinoma, adrenocortical carcinoma, melanoma, and epithelioid angiomyolipoma, can be indistinguishable from HCC on histologic grounds. Since this distinction has important therapeutic implications, judicious use of immunohistochemical markers plays an important role in establishing an accurate diagnosis, especially when limited material of tumor is available on cell block or a small core biopsy...
January 10, 2017: Human Pathology
https://www.readbyqxmd.com/read/28030838/livin-birc7-expression-as-malignancy-marker-in-adrenocortical-tumors
#9
Barbara Altieri, Silviu Sbiera, Silvia Della Casa, Isabel Weigand, Vanessa Wild, Sonja Steinhauer, Guido Fadda, Arkadius Kocot, Michaela Bekteshi, Egle M Mambretti, Andreas Rosenwald, Alfredo Pontecorvi, Martin Fassnacht, Cristina L Ronchi
Livin/BIRC7 is a member of the inhibitors of apoptosis proteins family, which are involved in tumor development through the inhibition of caspases. Aim was to investigate the expression of livin and other members of its pathway in adrenocortical tumors and in the adrenocortical carcinoma (ACC) cell line NCI-H295R.The mRNA expression of livin, its isoforms α and β, XIAP, CASP3 and DIABLO was evaluated by qRT-PCR in 82 fresh-frozen adrenal tissues (34 ACC, 25 adenomas = ACA, 23 normal adrenal glands = NAG)...
December 21, 2016: Oncotarget
https://www.readbyqxmd.com/read/27988912/clinical-characteristics-of-adrenal-tumors-in-children-a-retrospective-review-of-a-15-year-single-center-experience
#10
Xiaokun Lin, Dazhou Wu, Congde Chen, Na Zheng
OBJECTIVE: Adrenal tumors are rare in children. The aim of this study is to review and analyze clinical data on the diagnosis and management of adrenal tumors in children. METHODS: Between 2001 and 2015, 48 pediatric patients (<14 years old) admitted to our institute with adrenal tumors were reviewed. Clinical features, imaging studies, surgical approaches, as well as pathological diagnoses were recorded. RESULTS: The series comprised 28 males and 20 females...
December 17, 2016: International Urology and Nephrology
https://www.readbyqxmd.com/read/27943004/carney-complex-a-familial-lentiginosis-predisposing-to-a-variety-of-tumors
#11
REVIEW
Constantine A Stratakis
Carney complex is a familial lentiginosis syndrome; these disorders cover a wide phenotypic spectrum ranging from a benign inherited predisposition to develop cutaneous spots not associated with systemic disease to associations with several syndromes. Carney complex is caused by PRKAR1A mutations and perturbations of the cyclic AMP-dependent protein kinase (PKA) signaling pathway. In addition to the cutaneous findings, the main tumors associated with Carney complex are endocrine: 1) primary pigmented nodular adrenocortical disease, a bilateral adrenal hyperplasia leading to Cushing syndrome; 2) growth-hormone secreting pituitary adenoma or pituitary somatotropic hyperplasia leading to acromegaly; 3) thyroid and gonadal tumors, including a predisposition to thyroid cancer...
September 2016: Reviews in Endocrine & Metabolic Disorders
https://www.readbyqxmd.com/read/27928434/adrenalectomy-for-cushing-s-syndrome-do-s-and-don-ts
#12
REVIEW
Paduraru Dn, Nica A, Carsote M, Valea A
Aim. To present specific aspects of adrenalectomy for Cushing's syndrome (CS) by introducing well established aspects ("do's") and less known aspects ("don'ts"). Material and Method. This is a narrative review. Results. The "do's" for laparoscopic adrenalectomy (LA) are the following: it represents the "gold standard" for secretor and non-secretor adrenal tumors and the first line therapy for CS with an improvement of cardio-metabolic co-morbidities; the success rate depending on the adequate patients' selection and the surgeon's skills...
October 2016: Journal of Medicine and Life
https://www.readbyqxmd.com/read/27886397/telomerase-and-n-cadherin-differential-importance-in-adrenocortical-cancers-and-adenomas
#13
Sofia S Pereira, Valdemar Máximo, Ricardo Coelho, Rui Batista, Paula Soares, Susana G Guerreiro, Manuel Sobrinho-Simões, Mariana P Monteiro, Duarte Pignatelli
Adrenocortical carcinomas (ACC) are most frequently highly aggressive tumors. We assessed the telomerase reverse transcriptase (TERT) and N-cadherin role in the biology of ACC and their potential utility as molecular biomarkers, in different types of tumoral adrenocortical tissue. A total of 48 adrenal cortex samples (39 tumoral and 9 normal adrenal glands) were studied. TERT promoter mutations were searched by PCR and Sanger sequencing in two hotspots positions (-124 and -146). Also, telomerase and N-cadherin expression were evaluated by immunohistochemistry...
November 25, 2016: Journal of Cellular Biochemistry
https://www.readbyqxmd.com/read/27875378/cushing-syndrome-in-carney-complex-clinical-pathologic-and-molecular-genetic-findings-in-the-17-affected-mayo-clinic-patients
#14
Kathleen M Lowe, William F Young, Charalampos Lyssikatos, Constantine A Stratakis, J Aidan Carney
Carney complex (CNC) is a rare dominantly inherited multiorgan tumoral disorder that includes Cushing syndrome (CS). To establish the Mayo Clinic experience with the CS component, including its clinical, laboratory, and pathologic findings, we performed a retrospective search of the patient and pathologic databases of Mayo Clinic in Rochester, MN, for patients with CNC and clinical or laboratory findings of CS. Thirty-seven patients with CNC were identified. Twenty-nine had clinical, pathologic, or laboratory evidence of an adrenocortical disorder...
February 2017: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/27865598/suppression-of-cytochrome-p450-4b1-an-early-event-in-adrenocortical-tumorigenesis
#15
Timothy D Murtha, Reju Korah, Tobias Carling
BACKGROUND: Adrenocortical carcinoma is a rare neoplasm with a poor prognosis. Conversely, adrenocortical adenomas are common and benign. Despite their shared histologic origin, little evidence exists to suggest that adrenocortical adenoma arises from adrenocortical carcinoma. Recent genetic analyses of adrenocortical carcinoma have shown recurrent gene copy deletion of CYP4B1, a cytochrome P450 isozyme. This study investigates a potential role for CYP4B1 in modulating adrenocortical tumorigenesis and/or conferring chemoresistance to adrenocortical carcinomas...
January 2017: Surgery
https://www.readbyqxmd.com/read/27864864/genetics-of-adrenocortical-tumours
#16
T Åkerström, T Carling, F Beuschlein, P Hellman
The recently available genomic sequencing techniques have led to breakthroughs in understanding of the underlying genetic mechanisms in adrenocortical tumours. Disease-causing mutations have been described for aldosterone-producing adenomas, cortisol-producing adenomas and adrenocortical carcinomas. Further, knowledge gained from transcriptome analyses and methylation arrays has provided new insights into the development of these tumours. Elucidation of the genomic landscape of adrenocortical tumours and improved techniques may in the future be useful for early diagnosis through the detection of mutated DNA in the circulation...
December 2016: Journal of Internal Medicine
https://www.readbyqxmd.com/read/27862984/of-channels-and-pumps-different-ways-to-boost-the-aldosterone
#17
REVIEW
S Bandulik
The mineralocorticoid aldosterone is a major factor controlling the salt and water balance and thereby also the arterial blood pressure. Accordingly, primary aldosteronism (PA) characterized by an inappropriately high aldosterone secretion is the most common form of secondary hypertension. The physiological stimulation of aldosterone synthesis in adrenocortical glomerulosa cells by angiotensin II and an increased plasma K(+) concentration depends on a membrane depolarization and an increase in the cytosolic Ca(2+) activity...
November 15, 2016: Acta Physiologica
https://www.readbyqxmd.com/read/27853054/expression-of-cyp11b2-in-aldosterone-producing-adrenocortical-adenoma-regulatory-mechanisms-and-clinical-significance
#18
Yasuhiro Nakamura, Yuto Yamazaki, Yuta Tezuka, Fumitoshi Satoh, Hironobu Sasano
Aldosterone-producing adrenocortical adenoma (APA) is responsible for the majority of cases clinically diagnosed as primary aldosteronism. Aldosterone synthase (CYP11B2) is one of the enzymes that play essential roles in aldosterone synthesis and is involved in the pathogenesis of APA. Recent studies have demonstrated that various factors and regulators influence the expression and function of CYP11B2 in APA. In particular, somatic mutations, such as gain-of-function and loss-of-function mutations, have been identified in several genes, each of which encodes a pivotal protein that affects the calcium signaling pathway, the expression of CYP11B2, and aldosterone production...
2016: Tohoku Journal of Experimental Medicine
https://www.readbyqxmd.com/read/27813054/mechanisms-of-aberrant-pka-activation-by-c%C3%AE-subunit-mutations
#19
D Calebiro, K Bathon, I Weigand
Somatic mutations in PRKACA, coding for the catalytic α subunit of protein kinase A (PKA), have been recently identified as the most frequent genetic alteration in cortisol-secreting adrenocortical adenomas, which are responsible for adrenal Cushing's syndrome. The mutations identified so far lie at the interface between the catalytic (C) and regulatory (R) subunit of PKA. Detailed functional studies of the most frequent of these mutations (L206R) as well as of another one in the same region of the C subunit (199_200insW) have revealed that these mutations cause constitutive activation of PKA and lack of regulation by cAMP...
November 3, 2016: Hormone and Metabolic Research, Hormon- und Stoffwechselforschung, Hormones et Métabolisme
https://www.readbyqxmd.com/read/27754862/hypomethylation-of-cyp11b2-in-aldosterone-producing-adenoma
#20
Yoko Yoshii, Kenji Oki, Celso E Gomez-Sanchez, Haruya Ohno, Kiyotaka Itcho, Kazuhiro Kobuke, Masayasu Yoneda
The purpose of this study was to evaluate the DNA methylation levels of steroidogenic enzyme genes in aldosterone-producing adenoma (APA) and the effects of gene mutations in APA on the DNA methylation levels. DNA methylation array analysis was conducted using nonfunctioning adrenocortical adenoma (n=12) and APA (n=35) samples, including some with a KCNJ5 mutation (n=21), an ATP1A1 mutation (n=5), and without the known mutations (n=9). The quantitative polymerase chain reaction assay was performed for the detection of CYP11B2 and CYP11B1 expression levels in nonfunctioning adrenocortical adenoma and APA...
December 2016: Hypertension
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