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adrenocortical adenoma

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https://www.readbyqxmd.com/read/28641336/suppression-of-forkhead-box-protein-o1-foxo1-transcription-factor-may-promote-adrenocortical-tumorigenesis
#1
Adam Stenman, Timothy Murtha, Reju Korah, Tobias Carling
Despite recent comprehensive genetic analyses, molecular evidence for a pathophysiological continuum linking benign adrenocortical adenoma (ACA) and highly aggressive adrenocortical carcinoma (ACC) is still elusive. Using human tumor samples and the established ACC cell line SW-13, this study investigated potential regulatory roles for FOXO transcription factors, in modulating adrenocortical tumorigenesis. Adrenocortical tumor specimens (20 ACAs, 10 ACCs, and 9 normal adrenal tissue samples) obtained from 30 patients were analyzed for ubiquitously expressed FOXO transcription factors, FOXO1 and FOXO3 using qRT-PCR and immunohistochemistry...
June 22, 2017: Hormone and Metabolic Research, Hormon- und Stoffwechselforschung, Hormones et Métabolisme
https://www.readbyqxmd.com/read/28604387/macrolides-selectively-inhibit-mutant-kcnj5-potassium-channels-that-cause-aldosterone-producing-adenoma
#2
Ute I Scholl, Laura Abriola, Chengbiao Zhang, Esther N Reimer, Mark Plummer, Barbara I Kazmierczak, Junhui Zhang, Denton Hoyer, Jane S Merkel, Wenhui Wang, Richard P Lifton
Aldosterone-producing adenomas (APAs) are benign tumors of the adrenal gland that constitutively produce the salt-retaining steroid hormone aldosterone and cause millions of cases of severe hypertension worldwide. Either of 2 somatic mutations in the potassium channel KCNJ5 (G151R and L168R, hereafter referred to as KCNJ5MUT) in adrenocortical cells account for half of APAs worldwide. These mutations alter channel selectivity to allow abnormal Na+ conductance, resulting in membrane depolarization, calcium influx, aldosterone production, and cell proliferation...
June 12, 2017: Journal of Clinical Investigation
https://www.readbyqxmd.com/read/28584012/nefm-neurofilament-medium-polypeptide-a-marker-for-zona-glomerulosa-cells-in-human-adrenal-inhibits-d1r-dopamine-d1-receptor-mediated-secretion-of-aldosterone
#3
Carmela Maniero, Sumedha Garg, Wanfeng Zhao, Timothy Isaac Johnson, Junhua Zhou, Mark Gurnell, Morris J Brown
Heterogeneity among aldosterone-producing adenomas (APAs) has been highlighted by the discovery of somatic mutations. KCNJ5 mutations predominate in large zona fasciculata (ZF)-like APAs; mutations in CACNA1D, ATP1A1, ATP2B3, and CTNNB1 are more likely to be found in small zona glomerulosa (ZG)-like APAs. Microarray comparison of KCNJ5 mutant versus wild-type APAs revealed significant differences in transcriptomes. NEFM, encoding a neurofilament subunit which is a D1R (dopamine D1 receptor)-interacting protein, was 4-fold upregulated in ZG-like versus ZF-like APAs and 14-fold more highly expressed in normal ZG versus ZF...
June 5, 2017: Hypertension
https://www.readbyqxmd.com/read/28578183/central-precocious-puberty-secondary-to-adrenocortical-adenoma-in-a-female-child-case-report-and-review-of-the-literature
#4
Betül Ersoy, Deniz Kizilay, Hasan Çayirli, Peyker Temiz, Cüneyt Günşar
BACKGROUND: Pediatric adrenocortical tumors are rare but significant causes of virilization and peripheral precocious puberty (PPP). CASE: A 4-year-old girl presented with development of breast, pubic hair, and facial acne. Her bone age was advanced, and gonadotropins did not elevate in gonadotropin-releasing hormone (GnRH) test. High levels of dehydroepiandrosterone-sulfate, estradiol, and testosterone and detection of a tumor in the left adrenal gland of the abdomen by computed tomography led to a diagnosis of PPP due to adrenal tumor...
May 31, 2017: Journal of Pediatric and Adolescent Gynecology
https://www.readbyqxmd.com/read/28566446/cortisol-related-metabolic-alterations-assessed-by-mass-spectrometry-assay-in-patients-with-cushing-s-syndrome
#5
Guido Di Dalmazi, Marcus Quinkler, Timo Deutschbein, Cornelia Prehn, Nada Rayes, Matthias Kroiss, Christina Maria Berr, Günter K Stalla, Martin Fassnacht, Jerzy Adamski, Martin Reincke, Felix Beuschlein
OBJECTIVE: Endogenous hypercortisolism is a chronic condition associated with severe metabolic disturbances and cardiovascular sequela. The aim of this study was to characterize metabolic alterations in patients with different degrees of hypercortisolism by mass-spectrometry-based targeted plasma metabolomic profiling and correlate the metabolomic profile with clinical and hormonal data. DESIGN: Cross-sectional study. METHODS: Subjects (n=149) were classified according to clinical and hormonal characteristics: Cushing's syndrome (n=46), adrenocortical adenomas with autonomous cortisol secretion (n=31) or without hypercortisolism (n=27)...
May 31, 2017: European Journal of Endocrinology
https://www.readbyqxmd.com/read/28559117/myxoid-adrenocortical-adenoma-a-report-of-two-cases-and-literature-review
#6
Hongmei Wu, Fen Zhang, Yu Chen, Chao Liu, Su Yao, Xiaolan Zhu, Xinlan Luo, Hengguo Zhuang, Yanhui Liu
Myxoid adrenocortical adenomas are uncommon. There were only 61 cases reports documented, and the tumors are tended to be misdiagnosed in virtue of being rare and distinctive histological features. Recently we encountered two myxoid adrenocortical adenoma cases of a 31-year-old Chinese woman and a 45-year-old Chinese man. The patients did not receive further treatment after surgery and were still alive after following up for 20 months. Myxoid adrenocortical adenomas is extremely rare. Recognition of this entity would be beneficial for pathologists to avoid msidiagnosis, and unnecessary treatment...
April 20, 2017: Pathology, Research and Practice
https://www.readbyqxmd.com/read/28554744/analysis-of-histological-and-immunohistochemical-patterns-of-benign-and-malignant-adrenocortical-tumors-by-computerized-morphometry
#7
Paolo Dalino Ciaramella, Maurizio Vertemati, Duccio Petrella, Edgardo Bonacina, Erika Grossrubatscher, Eleonora Duregon, Marco Volante, Mauro Papotti, Paola Loli
Diagnosis of benign and purely localized malignant adrenocortical lesions is still a complex issue. Moreover, histology-based diagnosis may suffer of a moment of subjectivity due to inter- and intra-individual variations. The aim of the present study was to assess, by computerized morphometry, the morphological features in benign and malignant adrenocortical neoplasms. Eleven adrenocortical adenomas (ACA) were compared with 18 adrenocortical cancers (ACC). All specimens were stained with H&E, cellular proliferation marker Ki-67 and reticulin...
March 9, 2017: Pathology, Research and Practice
https://www.readbyqxmd.com/read/28551383/a-exceptional-collision-tumor-of-primary-adrenal-angiosarcoma-and-non-functioning-adrenocortical-adenoma
#8
Katsumi Takizawa, Kenichi Kohashi, Takahito Negishi, Kenichi Taguchi, Yuichi Yamada, Motonobu Nakamura, Yoshinao Oda
Primary adrenal angiosarcoma is an extremely rare vascular tumor. We report a case of a 63-year-old man with a collision tumor of epithelioid angiosarcoma and adrenocortical adenoma of the right adrenal gland. The adrenal tumor was incidentally observed by a preoperative computed tomography (CT) scan of penis squamous cell carcinoma. The patient underwent a right laparoscopic adrenalectomy, and the tumor size measured 34×34×15mm. Histological examination revealed two different tumor cell proliferations, namely epithelioid angiosarcoma and adrenocortical adenoma...
June 2017: Pathology, Research and Practice
https://www.readbyqxmd.com/read/28550366/minimally-invasive-resection-of-adrenal-masses-in-infants-and-children-results-of-a-european-multi-center-survey
#9
Francesco Fascetti-Leon, Giovanni Scotton, Luca Pio, Raimundo Beltrà, Paolo Caione, Ciro Esposito, Girolamo Mattioli, Amulya K Saxena, Sabine Sarnaki, Piergiorgio Gamba
BACKGROUND: Minimal access adrenal surgery (MAAS) for adrenal pathologies is the standard for many pediatric surgical centers. However, the literature offers few reports and minimal evidence from small case series. The aim of this study was to evaluate the outcomes of pediatric MAAS through a multi-center data analysis. METHOD: Pediatric patients who underwent MAAS between January 2002 and December 2013 were retrospectively included. Data analysis was conducted using Spss software (Welch's t-test, X-square, Fisher tests, multiple regression model)...
May 26, 2017: Surgical Endoscopy
https://www.readbyqxmd.com/read/28522647/prkar1a-mutation-causing-pituitary-dependent-cushing-disease-in-a-patient-with-carney-complex
#10
Florian W Kiefer, Yvonne Winhofer, Donato Iacovazzo, Marta Korbonits, Stefan Wolfsberger, Engelbert Knosp, Franz Trautinger, Romana Höftberger, Michael Krebs, Anton Luger, Alois Gessl
CONTEXT: Carney complex (CNC) is an autosomal dominant condition caused, in most cases, by an inactivating mutation of the PRKAR1A gene, which encodes for the type 1 alpha regulatory subunit of protein kinase A. CNC is characterized by the occurrence of endocrine overactivity, myxomas and typical skin manifestations. Cushing syndrome due to primary pigmented nodular adrenocortical disease (PPNAD) is the most frequent endocrine disease observed in CNC. CASE DESCRIPTION: Here we describe the first case of a patient with CNC and adrenocorticotropic hormone (ACTH)-dependent Cushing disease due to a pituitary corticotroph adenoma...
May 18, 2017: European Journal of Endocrinology
https://www.readbyqxmd.com/read/28501574/androgen-production-in-pediatric-adrenocortical-tumors-may-occur-via-both-the-classic-and-or-the-alternative-backdoor-pathway
#11
Nesa Marti, Jana Malikova, José A Galván, Maude Aebischer, Marco Janner, Zdenek Sumnik, Barbora Obermannova, Genevieve Escher, Aurel Perren, Christa E Flück
Children with adrenocortical tumors (ACTs) often present with virilization due to high tumoral androgen production, with dihydrotestosterone (DHT) as most potent androgen. Recent work revealed two pathways for DHT biosynthesis, the classic and the backdoor pathway. Usage of alternate routes for DHT production has been reported in castration-resistant prostate cancer, CAH and PCOS. To assess whether the backdoor pathway may contribute to the virilization of pediatric ACTs, we investigated seven children suffering from androgen producing tumors using steroid profiling and immunohistochemical expression studies...
May 10, 2017: Molecular and Cellular Endocrinology
https://www.readbyqxmd.com/read/28497219/robot-assisted-adrenalectomy-indications-and-drawbacks
#12
C Nomine-Criqui, A Germain, A Ayav, L Bresler, L Brunaud
Adrenal tumors can vary from a benign adrenocortical adenoma with no hormonal secretion to a secretory adrenocortical malignancy (adrenocortical carcinoma) or a hormone-secreting tumor of the adrenal medulla (pheochromocytoma). Currently, laparoscopic adrenalectomy is regarded as the preferred surgical approach for the management of most adrenal surgical disorders, although there are no prospective randomized trials comparing this technique with open adrenalectomy. However, widespread adoption of robotic technology has positioned robotic adrenalectomy as an option in some medical centers...
May 12, 2017: Updates in Surgery
https://www.readbyqxmd.com/read/28433999/circadian-plasma-cortisol-measurements-reflect-severity-of-hypercortisolemia-in-children-with-different-etiologies-of-endogenous-cushing-syndrome
#13
Amit Tirosh, Maya B Lodish, Charalampos Lyssikatos, Elena Belyavskaya, Georgios Z Papadakis, Constantine A Stratakis
BACKGROUND: The utility of circadian cortisol variation in estimating the degree of hypercortisolemia in different forms of endogenous Cushing syndrome (CS) has not been evaluated in children yet. METHODS: A retrospective cohort study, including children who underwent surgery due to CS (n = 115), was divided into children with a pituitary adenoma (Cushing disease) (n = 88), primary adrenal CS (n = 21), or ectopic adrenocorticotropin- or corticotropin-releasing hormone (ACTH-/CRH)-secreting tumors (n = 6)...
April 21, 2017: Hormone Research in Pædiatrics
https://www.readbyqxmd.com/read/28423361/next-generation-sequencing-reveals-microrna-markers-of-adrenocortical-tumors-malignancy
#14
Łukasz Koperski, Marta Kotlarek, Michał Świerniak, Monika Kolanowska, Anna Kubiak, Barbara Górnicka, Krystian Jażdżewski, Anna Wójcicka
BACKGROUND: Adrenocortical carcinoma is a rare finding among common adrenocortical tumors, but it is highly aggressive and requires early detection and treatment. Still, the differential diagnosis between benign and malignant lesions is difficult even for experienced pathologists and there is a significant need for novel diagnostic methods. In this study we aimed to reveal a complete set of microRNAs expressed in the adrenal gland and to identify easily detectable, stable and objective biomarkers of adrenocortical malignancy...
April 3, 2017: Oncotarget
https://www.readbyqxmd.com/read/28416583/physiological-and-pathological-roles-in-human-adrenal-of-the-glomeruli-defining-matrix-protein-npnt-nephronectin
#15
Ada Ee Der Teo, Sumedha Garg, Timothy Isaac Johnson, Wanfeng Zhao, Junhua Zhou, Celso Enrique Gomez-Sanchez, Mark Gurnell, Morris Jonathan Brown
Primary aldosteronism is a common cause of hypertension, which becomes refractory if undiagnosed, but potentially curable when caused by an aldosterone-producing adenoma (APA). The discovery of somatic mutations and differences in clinical presentations led to recognition of small but common zona glomerulosa (ZG)-like adenomas, distinct from classical large zona fasciculata-like adenomas. The inverse correlation between APA size and aldosterone synthase expression prompted us to undertake a systematic study of genotype-phenotype relationships...
June 2017: Hypertension
https://www.readbyqxmd.com/read/28391254/primary-pigmented-nodular-adrenocortical-disease-literature-review-and-case-report-of-a-6-year-old-boy
#16
Dragan Katanić, Dejan Kafka, Mirjana Živojinov, Jovan Vlaški, Zorana Budakov, Marija Knežević Pogančev, Ivana Vorgučin, Tomislav Ćuk
Cushing's syndrome is rare in childhood and is usually caused by a pituitary adenoma. Primary hyperfunction of adrenal glands is less frequent, particularly primary pigmented nodular adrenocortical disease (PPNAD). It occurs usually in children and adolescents, with female preponderance, while Cushing's disease has increased frequency in prepubertal males. A case of a 6-year-old boy is presented with isolated non-familiar PPNAD. The clinical pattern involved Cushingoid appearance, hypertension, virilization and depressive mood...
April 10, 2017: Journal of Pediatric Endocrinology & Metabolism: JPEM
https://www.readbyqxmd.com/read/28369983/microinsertions-in-prkaca-cause-activation-of-the-protein-kinase-a-pathway-in-cardiac-myxoma
#17
I-Ching Tseng, Wei-Ju Huang, Yu-Ling Jhuang, Ya-Yun Chang, Hung-Pin Hsu, Yung-Ming Jeng
Cardiac myxoma is the most common cardiac tumour. Most lesions occur sporadically, but occasional lesions develop in patients with Carney complex, a syndrome characterized by cardiac myxoma, spotty pigmentation, and endocrine overactivity. Two-thirds of patients with Carney complex harbour germline mutations in PRKAR1A, which encodes the type I regulatory subunit of protein kinase A (PKA). Most studies have not found a mutation in PRKAR1A in sporadic cardiac myxoma cases. Recent studies identified frequent mutations in PRKACA, which encodes the catalytic subunit of PKA, in cortisol-secreting adrenocortical adenoma cases...
June 2017: Journal of Pathology
https://www.readbyqxmd.com/read/28368480/genetic-and-histopathologic-intertumor-heterogeneity-in-primary-aldosteronism
#18
Kei Omata, Yuto Yamazaki, Yasuhiro Nakamura, Sharath K Anand, Justine A Barletta, Hironobu Sasano, William E Rainey, Scott A Tomlins, Anand Vaidya
Context: Whether primary aldosteronism (PA) is the consequence of a monoclonal or multiclonal process is unclear. Case Description: A 48-year-old man with severe bilateral PA refractory to medical therapy underwent unilateral adrenalectomy of the dominant adrenal. Although computed tomography showed three left-sided cortical nodules, postsurgical histopathology and genetic analysis revealed five different adrenocortical adenomas. Two zona fasciculata (ZF)-like aldosterone-producing adenomas (APAs) each harbored distinct known somatic KCNJ5 mutations (L168R and T158A)...
June 1, 2017: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/28365908/spinal-adrenal-cortical-adenoma-associated-with-beckwith-wiedemann-syndrome-case-report-and-review-of-the-literature
#19
Javier Giner, Isabel Esteban, Fernando Carceller, Javier Saceda, R M Regojo
PURPOSE: Ectopic adrenal cortical adenoma in the spinal region is extremely rare. The majority of cases of ectopic adrenocortical tissue are found along the path of embryonic migration within the urogenital tract. Beckwith-Wiedemann syndrome (BWS) is a pediatric overgrowth disorder involving a predisposition to tumor development, including adrenal lesions. To date, only eight spinal cases have been reported. This is the third reported case in pediatric population, the first one associated with genetic syndrome and the first benign to recur...
April 1, 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/28357529/differentiating-pheochromocytoma-from-lipid-poor-adrenocortical-adenoma-by-ct-texture-analysis-feasibility-study
#20
Gu-Mu-Yang Zhang, Bing Shi, Hao Sun, Zheng-Yu Jin, Hua-Dan Xue
OBJECTIVES: To investigate the feasibility of using CT texture analysis (CTTA) to differentiate pheochromocytoma from lipid-poor adrenocortical adenoma (lp-ACA). METHODS: Ninety-eight pheochromocytomas and 66 lp-ACAs were included in this retrospective study. CTTA was performed on unenhanced and enhanced images. Receiver operating characteristic (ROC) analysis was performed, and the area under the ROC curve (AUC) was calculated for texture parameters that were significantly different for the objective...
March 29, 2017: Abdominal Radiology
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