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Adrenocortical carcinoma

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https://www.readbyqxmd.com/read/28432798/therapeutic-drug-monitoring-of-mitotane-analytical-assay-and-patient-follow-up
#1
Yoann Cazaubon, Helene Guillemin, Damien Vautier, Olivier Oget, Hervé Millart, Claire Gozalo, Zoubir Djerada, Catherine Feliu
BACKGROUND: Adrenocortical carcinoma (ACC) is an aggressive malignancy of the adrenal gland. Mitotane (o,p'-DDD) is the most effective chemotherapy for ACC. According to literature, mitotane plasma trough concentrations within 14-20 mg.L(1) are correlated with a higher response rate with acceptable toxicity. Therapeutic drug monitoring (TDM) of mitotane is therefore recommended. The aim of this study was to propose a robust, and simple method for mitotane quantification in plasma. The validation procedures were based on international guidelines...
April 22, 2017: Biomedical Chromatography: BMC
https://www.readbyqxmd.com/read/28432084/topoisomerase2%C3%AE-and-thymidylate-synthase-expression-in-adrenocortical-cancer
#2
Elisa Roca, Alfredo Berruti, Silviu Sbiera, Ida Rapa, Ester Oneda, Paola Sperone, Cristina L Ronchi, Laura Ferrari, Salvatore Grisanti, Antonina Germano, Barbara Zaggia, Giorgio Scagliotti, Martin Fassnacht, Marco Volante, Massimo Terzolo, Mauro Papotti
Topoisomerase II alpha (TOP2A) and Thymidylate Synthase (TS) are known prognostic parameters in several tumors and also predictors of efficacy of anthracyclines, topoisomerase inhibitors and fluoropirimidines, respectively. Expression of TOP2A and TS mRNA was assessed in 98 patients with adrenocortical carcinoma (ACC) and protein expression was assessed by immunohistochemistry in a subset of 39 tumors. Ninety-two patients were radically resected for stage II-III disease and 38 of them received adjuvant mitotane...
April 21, 2017: Endocrine-related Cancer
https://www.readbyqxmd.com/read/28431167/performance-of-18f-fdg-pet-ct-in-the-characterization-of-adrenal-masses-in-non-cancer-patients-a-prospective-study
#3
Carole Guerin, François Pattou, Laurent Brunaud, Jean-Christophe Lifante, Eric Mirallié, Magalie Haissaguerre, Damien Huglo, Pierre Olivier, Claire Houzard, Catherine Ansquer, Elif Hindié, Anderson Loundou, Cendrine Archange, Antoine Tabarin, Fréderic Sebag, Karine Baumstarck, David Taïeb
Context: Few prospective studies have evaluated the role of 18F-Fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG PET/CT) in the characterization of adrenal masses. Objective: To assess the performance of 18F-FDG PET/CT in the malignancy diagnosis of adrenal masses in non-cancer patients. Design: Prospective multicenter study. Material and methods: The study population consisted of 87 patients (87 adrenal masses) referred to endocrine surgeons: 56 with mass diameter ≥40 mm and 31 with a diameter <40 mm and of indeterminate nature based on unenhanced and washout CT attenuation densities...
April 20, 2017: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/28423559/rottlerin-as-a-novel-chemotherapy-agent-for-adrenocortical-carcinoma
#4
Yi Zhu, Minjie Wang, Xu Zhao, Lei Zhang, Yigao Wu, Bangqi Wang, Weilie Hu
Adrenocortical carcinoma (ACC) is a rare, but aggressive endocrine malignancy with a generally poor clinical outcome. There is no effective therapy for advanced and metastatic ACC. In our study, we found that an existing drug (rottlerin) exerted its tumour-suppressive function in ACC. Specifically, rottlerin inhibited cellular proliferation of ACC cell lines (NCI-H295R and SW-13) in a dose- and time-dependent manner. We also found that rottlerin induced cell apoptosis and promoted G0/G1 cell cycle arrest in ACC cell lines...
April 4, 2017: Oncotarget
https://www.readbyqxmd.com/read/28423361/next-generation-sequencing-reveals-microrna-markers-of-adrenocortical-tumors-malignancy
#5
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/28421464/a-novel-tp53-mutation-associated-with-twist1-and-sip1-expression-in-an-aggressive-adrenocortical-carcinoma
#6
Daniel Bulzico, Davi Coe Torres, Gerson Moura Ferreira, Bruno Ricardo Barreto Pires, Paulo Antônio Silvestre de Faria, Rocio Hassan, Eliana Abdelhay, Mario Vaisman, Leonardo Vieira Neto
Adrenocortical carcinomas (ACC) are very rare tumors related to TP53 mutations mostly in childhood onset cases. Epithelial-mesenchymal transition (EMT) transcription factors TWIST1 and Smad interacting protein 1 (SIP1) are related to poorer outcomes in other malignancies, but their role in ACC is unknown. We describe a case of an advanced metastatic ACC (Weiss-score of 9) in a patient at age 76. After primary tumor resection, mitotane therapy was started as palliation to low-volume liver metastasis. After a 2-year period of stable disease, the patient died due to brain metastasis...
April 18, 2017: Endocrine Pathology
https://www.readbyqxmd.com/read/28410270/the-aurora-kinase-inhibitor-amg-900-increases-apoptosis-and-induces-chemosensitivity-to-anticancer-drugs-in-the-nci-h295-adrenocortical-carcinoma-cell-line
#7
Kleiton S Borges, Augusto F Andrade, Vanessa S Silveira, David S Marco Antonio, Elton J R Vasconcelos, Sonir R R Antonini, Luiz G Tone, Carlos A Scrideli
Adrenocortical tumor (ACT) is a malignancy with a low incidence rate and the current therapy for advanced disease has a limited impact on overall patient survival. A previous study from our group suggested that elevated expression of aurora-A and aurora-B is associated with poor outcome in childhood ACT. Similar results were also reported for adult ACTs. The present in-vitro study shows that AMG 900 inhibits aurora kinases in adrenocortical carcinoma cells. AMG 900 inhibited cell proliferation in NCI-H295 cells as well as in the ACT primary cultures and caused apoptosis in the cell line NCI-H295...
April 13, 2017: Anti-cancer Drugs
https://www.readbyqxmd.com/read/28378549/myxoid-and-sarcomatoid-variants-of-adrenocortical-carcinoma-analysis-of-rare-variants-in-single-tertiary-care-center
#8
Tae Yon Sung, Yun Mi Choi, Won Gu Kim, Yu Mi Lee, Tae Yong Kim, Young Kee Shong, Won Bae Kim, Dong Eun Song
The aim of this study is to describe rare variants of adrenocortical carcinoma (ACC) and to compare the prognosis with that of conventional ACC. We retrospectively reviewed 8 cases of myxoid variant, 1 sarcomatoid variant, and 14 cases of conventional ACC, who underwent surgical resection at the Asan Medical Center between 1996 and 2014. An analysis of the clinicopathological characteristics, including the Weiss score, Ki-67 labeling index, and reticulin framework assessment is presented. The mean age of patients with myxoid/sarcomatoid ACC was 45 years; 4 out of 9 patients were women...
May 2017: Journal of Korean Medical Science
https://www.readbyqxmd.com/read/28368424/p53-rb-inhibition-induces-metastatic-adrenocortical-carcinomas-in-a-preclinical-transgenic-model
#9
M Batisse-Lignier, I Sahut-Barnola, F Tissier, T Dumontet, M Mathieu, C Drelon, J-C Pointud, C Damon-Soubeyrand, G Marceau, J-L Kemeny, J Bertherat, I Tauveron, P Val, A Martinez, A-M Lefrançois-Martinez
Adrenocortical carcinoma (ACC) is a rare cancer with poor prognosis. Pan-genomic analyses identified p53/Rb and WNT/β-catenin signaling pathways as main contributors to the disease. However, isolated β-catenin constitutive activation failed to induce malignant progression in mouse adrenocortical tumors. Therefore, there still was a need for a relevant animal model to study ACC pathogenesis and to test new therapeutic approaches. Here, we have developed a transgenic mice model with adrenocortical specific expression of SV40 large T-antigen (AdTAg mice), to test the oncogenic potential of p53/Rb inhibition in the adrenal gland...
April 3, 2017: Oncogene
https://www.readbyqxmd.com/read/28356253/adrenocortical-carcinoma-masquerading-as-cushing-s-disease
#10
Kush Dev Jarial, Rama Walia, Santosh Kumar, Anil Bhansali
Cushing's syndrome (CS) can be classified as adrenocorticotropic hormone (ACTH)-dependent or ACTH-independent depending on the ACTH levels. However, 30% of the patients with CS have ACTH levels in the 'grey zone' (5-20 pg/mL), thereby posing a challenge in establishing the aetiological diagnosis. In a patient with full-blown features of Cushing's syndrome with equivocal ACTH levels, and a pituitary microadenoma on contrast-enhanced MRI sella, can falsely lead to a diagnosis of Cushing's disease. Pituitary microadenoma, if <6 mm in size, may be an incidental finding (incidentaloma) in this scenario and can be present in ∼3-27% of the healthy population...
March 29, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28348073/from-benign-adrenal-incidentaloma-to-adrenocortical-carcinoma-an-exceptional-random-event
#11
Ines Belmihoub, Stephane Silvera, Mathilde Sibony, Bertrand Dousset, Paul Legmann, Xavier Bertagna, Jerome Bertherat, Guillaume Assie
New European guidelines for the management of adrenal incidentalomas were recently released. One of the most novel recommendations is to stop following patients when they present a typical, small and non-secreting adenoma. We report here the case of a 71-year-old man with such an adenoma, who developed an adrenocortical carcinoma (ACC) fourteen years later, with subsequent metastases and death. Clinically he had a normal blood pressure and no sign of hormonal hypersecretion. The hormonal work-up showed no hormone excess: urinary free cortisol level was normal, the diurnal cortisol rhythm was respected and urinary catecholamine metabolites levels were normal...
March 27, 2017: European Journal of Endocrinology
https://www.readbyqxmd.com/read/28324035/long-term-outcomes-of-adjuvant-mitotane-therapy-in-patients-with-radically-resected-adrenocortical-carcinoma
#12
Alfredo Berruti, Salvatore Grisanti, Alina Pulzer, Mélanie Claps, Fulvia Daffara, Paola Loli, Massimo Mannelli, Marco Boscaro, Emanuela Arvat, Guido Tiberio, Stefanie Hahner, Barbara Zaggia, Francesco Porpiglia, Marco Volante, Martin Fassnacht, Massimo Terzolo
Context: In 2007, a 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: One hundred sixty-two ACC patients who did not recur or die after a landmark period of 3 months were considered...
April 1, 2017: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/28277340/update-on-adrenocortical-carcinoma-management-and-future-directions
#13
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
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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