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Adrenocortical carcinoma

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https://www.readbyqxmd.com/read/28324035/long-term-outcomes-of-adjuvant-mitotane-therapy-in-patients-with-radically-resected-adrenocortical-carcinoma
#1
A Berruti, S Grisanti, A Pulzer, M Claps, F Daffara, P Loli, M Mannelli, M Boscaro, E Arvat, G Tiberio, S Hahner, B Zaggia, F Porpiglia, M Volante, M Fassnacht, M Terzolo
Context: in 2007, a large retrospective case-control study provided evidence that adjuvant mitotane prolongs recurrence-free survival (RFS) in patients with radically resected adrenocortical carcinoma (ACC). Objective and design: We aimed to confirm the prognostic role of adjuvant mitotane in the same series after 9 additional years of follow-up. Setting, Patients and Interventions: 162 ACC patients included in the previous study, who did not recur or die after a landmark period of 3 months, were considered...
January 11, 2017: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/28277340/update-on-adrenocortical-carcinoma-management-and-future-directions
#2
Jeena Varghese, Mouhammed Amir Habra
PURPOSE OF REVIEW: To present an update on the management of and future directions in adrenocortical carcinoma (ACC). RECENT FINDINGS: ACC is a rare malignancy with high morbidity and mortality. Surgery remains the mainstay treatment for localized disease, but it is often not feasible in more advanced cases. There is an ongoing controversy about the routine use of adjuvant treatments after surgery. Hormonal overproduction can complicate the management and worsen the prognosis of the disease...
March 8, 2017: Current Opinion in Endocrinology, Diabetes, and Obesity
https://www.readbyqxmd.com/read/28271381/tissue-expression-and-pharmacological-in-vitro-analyses-of-mtor-and-sstr-pathways-in-adrenocortical-carcinoma
#3
Antonina Germano, Ida Rapa, Eleonora Duregon, Arianna Votta, Jessica Giorcelli, Consuelo Buttigliero, Giorgio V Scagliotti, Marco Volante, Massimo Terzolo, Mauro Papotti
New therapies for advanced adrenocortical carcinoma (ACC) are urgently needed, as the majority of the patients experience a rapid and inexorable progression despite surgery and adjuvant mitotane. In vitro data suggest that somatostatin receptors (SSTRs) and mTOR pathway might represent reasonable targets for novel therapies, being involved in functionality and growth of ACC cells. However, in vitro analysis of combination treatments targeting both mTOR and SSTR as compared to mitotane are poorly explored in ACC...
March 7, 2017: Endocrine Pathology
https://www.readbyqxmd.com/read/28270555/dosage-dependent-regulation-of-vav2-expression-by-steroidogenic-factor-1-drives-adrenocortical-carcinoma-cell-invasion
#4
Carmen Ruggiero, Mabrouka Doghman-Bouguerra, Silviu Sbiera, Iuliu Sbiera, Maddy Parsons, Bruno Ragazzon, Aurélie Morin, Estelle Robidel, Judith Favier, Jérôme Bertherat, Martin Fassnacht, Enzo Lalli
Adrenocortical carcinoma (ACC) is a rare endocrine malignancy with a dismal prognosis. Genomic studies have enabled progress in our understanding of the molecular bases of ACC, but factors that influence its prognosis are lacking. Amplification of the gene encoding the transcription factor steroidogenic factor-1 (SF-1; also known as NR5A1) is one of the genetic alterations common in ACC. We identified a transcriptional regulatory mechanism involving increased abundance of VAV2, a guanine nucleotide exchange factor for small GTPases that control the cytoskeleton, driven by increased expression of the gene encoding SF-1 in ACC...
March 7, 2017: Science Signaling
https://www.readbyqxmd.com/read/28259701/-long-term-blood-pressure-outcome-after-unilateral-adrenalectomy-for-primary-hyperaldosteronism
#5
F Saint, R Prader, C Cordonnier, G Choukroun, N Elesper, R Desailloud
OBJECTIVES: To evaluate long-term results of adrenalectomy for primary aldosteronism (PA) and to identify prognostic factors associated. METHODS: Exhaustive retrospective review of all consecutive patients undergoing adrenalectomy for PA between 2002 and 2013 in our department. All patients underwent preoperative: clinical evaluation (age, sex, height, weight, systolic and diastolic BP under treatment, identification of anti-hypertension treatment), biological evaluation (potassium, renin, aldosterone) and radiological evaluation (CT and/or MRI)...
March 1, 2017: Progrès en Urologie
https://www.readbyqxmd.com/read/28258518/adrenocortical-carcinoma-arising-in-an-adrenal-rest-a-case-report-and-review-of-the-literature
#6
Kristine M Cornejo, Henrietta A Afari, Peter M Sadow
Carcinomas arising from embryonic adrenal rests are rare with only a handful of reported cases. We report a case of an adrenocortical carcinoma arising from an adrenal rest located between the bladder and prostate in a 51 year-old man. The patient presented following a year of rectal pain and constipation. Computed tomography (CT) scan revealed a 9 cm pelvic mass that appeared to arise from the soft tissue between the bladder and prostate, with displacement of the organs and narrowing of the rectal lumen, suspected to be a sarcoma...
March 3, 2017: Endocrine Pathology
https://www.readbyqxmd.com/read/28249601/a-novel-foxo1-mediated-dedifferentiation-blocking-role-for-dkk3-in-adrenocortical-carcinogenesis
#7
Joyce Y Cheng, Taylor C Brown, Timothy D Murtha, Adam Stenman, C Christofer Juhlin, Catharina Larsson, James M Healy, Manju L Prasad, Wolfram T Knoefel, Andreas Krieg, Ute I Scholl, Reju Korah, Tobias Carling
BACKGROUND: Dysregulated WNT signaling dominates adrenocortical malignancies. This study investigates whether silencing of the WNT negative regulator DKK3 (Dickkopf-related protein 3), an implicated adrenocortical differentiation marker and an established tumor suppressor in multiple cancers, allows dedifferentiation of the adrenal cortex. METHODS: We analyzed the expression and regulation of DKK3 in human adrenocortical carcinoma (ACC) by qRT-PCR, immunofluorescence, promoter methylation assay, and copy number analysis...
March 1, 2017: BMC Cancer
https://www.readbyqxmd.com/read/28199934/mixed-corticomedullary-adrenal-carcinoma-case-report-comparison-in-features-treatment-and-prognosis-with-the-other-two-reported-cases
#8
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...
2017: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/28199015/european-society-of-endocrine-surgeons-eses-and-european-network-for-the-study-of-adrenal-tumours-ensat-recommendations-for-the-surgical-management-of-adrenocortical-carcinoma
#9
S Gaujoux, R Mihai
BACKGROUND: Radical surgery provides the best chance of cure for adrenocortical carcinoma (ACC), but perioperative surgical care for these patients is yet to be standardized. METHODS: A working group appointed jointly by ENSAT and ESES used Delphi methodology to produce evidence-based recommendations for the perioperative surgical care of patients with ACC. Papers were retrieved from electronic databases. Evidence and recommendations were classified according to the Grading of Recommendations, Assessment, Development and Evaluation (GRADE) system, and were discussed until consensus was reached within the group...
March 2017: British Journal of Surgery
https://www.readbyqxmd.com/read/28184938/new-insights-and-future-perspectives-in-the-therapeutic-strategy-of-adrenocortical-carcinoma-review
#10
Antonio Stigliano, Lidia Cerquetti, Pina Lardo, Elisa Petrangeli, Vincenzo Toscano
Adrenocortical carcinoma (ACC) is a rare endocrine malignancy with an incidence ranging from 0.7 to 2.0 cases/million people per year. Hypercortisolism represents the most common clinical presentation in many patients although, less frequently, some ACC secreting androgens and estrogens are even more pathognomonic compared to cortisol secretion. Currently, radical surgery, when feasible, is still the only curative therapy. Mitotane, an adrenolytic drug, is used in the adjuvant setting and in combination with chemotherapy drugs in metastatic disease...
March 2017: Oncology Reports
https://www.readbyqxmd.com/read/28181818/recommendations-for-the-management-of-adrenal-incidentalomas-what-is-pertinent-for-radiologists
#11
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 summarizes and highlights radiological recommendations within the recently issued guidelines for the management of adrenal incidentalomas from the European Society of Endocrinology Clinical Practice in collaboration with the European Network for Study of Adrenal Tumours...
April 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
#12
Johanne Marie Holst, Erzsébet Horváth-Puhó, Rikke Beck Jensen, Mariane Rix, Kurt Kristensen, Niels Thomas Hertel, Olaf M Dekkers, Henrik Toft Sørensen, Anders Juul, Jens Otto L Jørgensen
OBJECTIVE: Cushing's syndrome (CS) affects all age groups, but epidemiologic data in young patients 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 Classification of Diseases (ICD) codes and the Danish Civil Registration System...
May 2017: European Journal of Endocrinology
https://www.readbyqxmd.com/read/28149812/chylous-ascites-after-resection-of-giant-adrenocortical-carcinoma
#13
Mani Habibi, Rojbin Karakoyun, Erkan Demirci, Arsenal Sezgin Alikanoglu
Postoperative chylous ascites (PCA) is a rare clinical state that occurs during abdominal surgery. Despite its rarity, the need to diagnose and treat PCA is increasing in importance with the increased number of wide resections and lymph node dissections being performed and the serious consequences of treatment. Here we describe the PCA complications we observed after resection for treating a case of giant adrenocortical carcinoma and we have the brief review of the PCA complication.
December 2016: Gland Surgery
https://www.readbyqxmd.com/read/28127763/mutational-signature-analysis-identifies-mutyh-deficiency-in-colorectal-cancers-and-adrenocortical-carcinomas
#14
Camilla Pilati, Jayendra Shinde, Ludmil B Alexandrov, Guillaume Assié, Thierry André, Zofia Hélias-Rodzewicz, Romain Doucoudray, Delphine Le Corre, Jessica Zucman-Rossi, Jean-François Emile, Jérôme Bertherat, Eric Letouzé, Pierre Laurent-Puig
Germline alterations in DNA repair genes are implicated in cancer predisposition and can result in characteristic mutational signatures. However, specific mutational signatures associated with base excision repair (BER) defects remain to be characterized. Here, by analysing a series of colorectal cancers (CRC) using exome sequencing, we identified a particular spectrum of somatic mutations characterized by an enrichment of C > A transversions in NpCpA or NpCpT contexts, in three tumours from a MUTYH-associated polyposis (MAP) patient and in two cases harbouring pathogenic germline MUTYH mutations...
January 27, 2017: Journal of Pathology
https://www.readbyqxmd.com/read/28127190/bilateral-aldosterone-producing-adrenocortical-carcinoma-a-rare-entity
#15
Garima Daga, Sanjay Sharma, Varun Mittal
Adrenal cortical carcinomas (ACC) are rare aggressive malignant neoplasms with a reported incidence of 1-2 cases per 1 million population and account for 0.05-0.2 % of all malignancies. About 60 % are functional tumors and majority, 40 % present with Cushing's syndrome. Rarely, 2.5 to 11 % present with hypertension due to increased secretion of aldosterone and are usually unilateral. Bilateral aldosterone-producing adrenocortical carcinoma (APAC) has not been reported yet. We report a case of bilateral APAC in a 22-year-old male...
March 2017: Indian Journal of Surgical Oncology
https://www.readbyqxmd.com/read/28127186/management-of-adrenal-masses
#16
REVIEW
Hattangadi Sanjay Bhat, Balagopal Nair Tiyadath
An adrenal mass can be either symptomatic or asymptomatic in the form of adrenal incidentalomas (AIs) in up to 8 % in autopsy and 4 % in imaging series. Once a diagnosis of adrenal mass is made, we need to differentiate whether it is functioning or nonfunctioning, benign, or malignant. In this article, we provide a literature review of the diagnostic workup including biochemical evaluation and imaging characteristics of the different pathologies. We also discuss the surgical strategies with laparoscopy as the mainstay with partial adrenalectomy in select cases and adrenalectomy in large masses...
March 2017: Indian Journal of Surgical Oncology
https://www.readbyqxmd.com/read/28114280/rarres2-functions-as-a-tumor-suppressor-by-promoting-%C3%AE-catenin-phosphorylation-degradation-and-inhibiting-p38-phosphorylation-in-adrenocortical-carcinoma
#17
Y Liu-Chittenden, M Jain, K Gaskins, S Wang, M J Merino, S Kotian, S Kumar Gara, S Davis, L Zhang, E Kebebew
Tumor suppressor genes and the immune system are critical players in inhibiting cancer initiation and/or progression. However, little is known about whether a tumor suppressor gene can function through both immune-dependent and -independent mechanisms. Retinoic acid receptor responder 2 (RARRES2) is transcriptionally downregulated in multiple cancer types. Previous studies suggested that it can serve as an immune-dependent tumor suppressor by acting as a chemoattractant to recruit anticancer immune cells expressing its receptor, the chemerin chemokine receptor 1 (CMKLR1), to sites of tumor...
January 23, 2017: Oncogene
https://www.readbyqxmd.com/read/28095038/pure-androgen-producing-adrenal-tumor-clinical-features-and-pathogenesis
#18
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
#19
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/28079787/oral-and-vulvo-vaginal-lichenoid-reactions-due-to-mitotane-lysodren-a-case-report
#20
Arthur Schmouchkovitch, Héloïse Herry, Philippe Thuillier, Véronique Kerlan, Camille Fleuret, Guy Le Toux, Sylvie Boisramé
BACKGROUND: The purpose of pharmacovigilance (drug safety) is collection, detection, assessment, monitoring, and prevention of adverse effects with pharmaceutical products. It is meant to identify, characterize, prevent, or minimize actual or potential risks relating to medicinal products. To prevent these adverse effects and improve our practice, health professionals have a duty to report side effects to assess this risk and evaluate the benefit/risk requirements. Mitotane (Lysodren) is used for treating adrenocortical carcinoma...
January 2017: Medicine (Baltimore)
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