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Adrenocortical carcinoma

Yasunori Minami, Tomohiro Minami, Hirokazu Chishina, Masashi Kono, Tadaaki Arizumi, Masahiro Takita, Norihisa Yada, Satoru Hagiwara, Hiroshi Ida, Kazuomi Ueshima, Naoshi Nishida, Masatoshi Kudo
OBJECTIVE: Radiofrequency ablation (RFA) induces gas bubbles in ablation zones, and the ablative margin cannot be evaluated accurately on ultrasound (US) during and immediately after RFA. This study assessed the usefulness of US-US fusion imaging to visualize the ablative margin of RFA for liver metastasis. METHODS: RFA guided by US-US fusion imaging was performed on 12 targeted tumors in 10 patients. Secondary hepatic malignancies included patients with colorectal cancer (n = 4), breast cancer (n = 2), lung cancer (n = 1), gastrointestinal stromal tumor (n = 1), pancreatic neuroendocrine tumor (n = 1), and adrenocortical carcinoma (n = 1)...
2016: Digestive Diseases
Bhuvana Cn, H V Nataraju, Nayana Hk
No abstract text is available yet for this article.
January 2016: Journal of the Association of Physicians of India
Raissa Coelho Andrade, Anna Claudia Evangelista Dos Santos, Joaquim Caetano de Aguirre Neto, Julián Nevado, Pablo Lapunzina, Fernando Regla Vargas
Li-Fraumeni and Li-Fraumeni like syndromes (LFS/LFL) represent rare cancer-prone conditions associated mostly with sarcomas, breast cancer, brain tumors, and adrenocortical carcinomas. TP53 germline mutations are present in up to 80 % of families with classic Li-Fraumeni syndrome, and in 20-60 % of families with Li-Fraumeni like phenotypes. The frequency of LFS/LFL families with no TP53 mutations detected suggests the involvement of other genes in the syndrome. In this study, we searched for mutations in TP53 in 39 probands from families with criteria for LFS/LFL...
October 6, 2016: Familial Cancer
Renate Louw-du Toit, Meghan S Perkins, Jacky L Snoep, Karl-Heinz Storbeck, Donita Africander
Progestins used in contraception and hormone replacement therapy are synthetic compounds designed to mimic the actions of the natural hormone progesterone and are classed into four consecutive generations. The biological actions of progestins are primarily determined by their interactions with steroid receptors, and factors such as metabolism, pharmacokinetics, bioavailability and the regulation of endogenous steroid hormone biosynthesis are often overlooked. Although some studies have investigated the effects of select progestins on a few steroidogenic enzymes, studies comparing the effects of progestins from different generations are lacking...
2016: PloS One
Rajiv D Reddy, Gayle R Spill
No abstract text is available yet for this article.
September 2016: PM & R: the Journal of Injury, Function, and Rehabilitation
Maria Isabel Achatz, Gerard P Zambetti
A common criticism of studying rare diseases is the often-limited relevance of the findings to human health. Here, we review ∼15 years of research into an unusual germline TP53 mutation (p.R337H) that began with its detection in children with adrenocortical carcinoma (ACC), a remarkably rare childhood cancer that is associated with poor prognosis. We have come to learn that the p.R337H mutation exists at a very high frequency in Southern and Southeastern Brazil, occurring in one of 375 individuals within a total population of ∼100 million...
September 23, 2016: Cold Spring Harbor Perspectives in Medicine
Kaoru Yamashita, Atsuhiro Ichihara, Kentaro Ito, Naohiro Yoshida, Fumiko Mitani, Jin Endo, Motoaki Sano, Keiichi Fukuda, Satoshi Morimoto
OBJECTIVE: Numerous clinical trials demonstrated that renin-angiotensin-aldosterone system (RAAS) was involved in the pathogenesis of hypertension-induced heart failure. While RAS is a major machinery required for aldosterone production, multiple minor systems, including catecholamine, could give rise to the aberrant aldosterone production beyond the RAS activation. We investigated the mechanism underlying the RAS-independent aldosterone production in heart failure. DESIGN AND METHOD: Dahl-salt sensitive rats fed high salt diet developed malignant hypertension, resulting in heart failure with inappropriately increased plasma aldosterone level...
September 2016: Journal of Hypertension
Thuy B Tran, Lauren M Postlewait, Shishir K Maithel, Jason D Prescott, Tracy S Wang, Jason Glenn, John E Phay, Kara Keplinger, Ryan C Fields, Linda X Jin, Sharon M Weber, Ahmed Salem, Jason K Sicklick, Shady Gad, Adam C Yopp, John C Mansour, Quan-Yang Duh, Natalie Seiser, Carmen C Solorzano, Colleen M Kiernan, Konstantinos I Votanopoulos, Edward A Levine, Ioannis Hatzaras, Rivfka Shenoy, Timothy M Pawlik, Jeffrey A Norton, George A Poultsides
BACKGROUND: Adrenocortical carcinoma (ACC) is a rare and aggressive malignancy with limited therapeutic options beyond surgical resection. The characteristics of actual long-term survivors following surgical resection for ACC have not been previously reported. METHOD: Patients who underwent resection for ACC at one of 13 academic institutions participating in the US Adrenocortical Carcinoma Group from 1993 to 2014 were analyzed. Patients were stratified into four groups: early mortality (died within 2 years), late mortality (died within 2-5 years), actual 5-year survivor (survived at least 5 years), and actual 10-year survivor (survived at least 10 years)...
September 16, 2016: Journal of Surgical Oncology
Matthias Kroiss, Silviu Sbiera, Sabine Kendl, Max Kurlbaum, Martin Fassnacht
Mitotane is the only drug approved for treatment of the orphan disease adrenocortical carcinoma (ACC) and was recently shown to be the first clinically used drug acting through endoplasmic reticulum (ER)-stress induced by toxic lipids. Since mitotane has limited clinical activity as monotherapy, we here study the potential of activating ER-stress through alternative pathways. The single reliable NCI-H295 cell culture model for ACC was used to study the impact MG132, bortezomib (BTZ) and carfilzomib (CFZ) on mRNA and protein expression of ER-stress markers, cell viability and steroid hormone secretion...
September 8, 2016: Hormones & Cancer
Chiara Fiorentini, Martina Fragni, Paola Perego, Sara Vezzoli, Sara A Bonini, Monica Tortoreto, Diego Galli, Melanie Claps, Guido A Tiberio, Massimo Terzolo, Cristina Missale, Maurizio Memo, Giuseppe Procopio, Nadia Zaffaroni, Alfredo Berruti, Sandra Sigala
CONTEXT: Patients with adrenocortical carcinoma (ACC) frequently suffer from cortisol excess, which portends a negative prognosis. Rapid control of cortisol hypersecretion and tumor growth are the main goals of ACC therapy. Abiraterone acetate (AA) is a potent inhibitor of 17alpha-hydroxylase/17,20-lyase, a key enzyme of adrenal steroidogenesis. OBJECTIVE: To investigate the therapeutic use of AA in preclinical models of ACC. DESIGN: AA antisecretive and antiproliferative effects were investigated in vitro using NCI-H295R and SW13 ACC cell lines and human primary ACC cell cultures, as well as in vivo using immunodeficient mice...
September 14, 2016: Journal of Clinical Endocrinology and Metabolism
Coralie Drelon, Annabel Berthon, Isabelle Sahut-Barnola, Mickaël Mathieu, Typhanie Dumontet, Stéphanie Rodriguez, Marie Batisse-Lignier, Houda Tabbal, Igor Tauveron, Anne-Marie Lefrançois-Martinez, Jean-Christophe Pointud, Celso E Gomez-Sanchez, Seppo Vainio, Jingdong Shan, Sonia Sacco, Andreas Schedl, Constantine A Stratakis, Antoine Martinez, Pierre Val
Adrenal cortex physiology relies on functional zonation, essential for production of aldosterone by outer zona glomerulosa (ZG) and glucocorticoids by inner zona fasciculata (ZF). The cortex undergoes constant cell renewal, involving recruitment of subcapsular progenitors to ZG fate and subsequent lineage conversion to ZF identity. Here we show that WNT4 is an important driver of WNT pathway activation and subsequent ZG differentiation and demonstrate that PKA activation prevents ZG differentiation through WNT4 repression and WNT pathway inhibition...
2016: Nature Communications
Thuy B Tran, Shishir K Maithel, Timothy M Pawlik, Tracy S Wang, Ioannis Hatzaras, John E Phay, Ryan C Fields, Sharon M Weber, Jason K Sicklick, Adam C Yopp, Quan-Yang Duh, Carmen C Solorzano, Konstantinos I Votanopoulos, George A Poultsides
BACKGROUND: Adrenocortical carcinoma (ACC) is an aggressive malignancy typically resistant to chemotherapy and radiation. Surgery, even in the setting of locally recurrent or metastatic disease, remains the only potentially curative option. However, the subset of patients who will benefit from repeat resection in this setting remains ill defined. The objective of this study is to propose a prognostic clinical score that facilitates selection of patients for repeat resection of recurrent ACC...
September 9, 2016: Journal of the American College of Surgeons
Benjamin Miron, Benjamin T Ristau, Jeffrey J Tomaszewski, Josh Jones, Bart Milestone, Yu-Ning Wong, Robert G Uzzo, Donna Edmondson, Walter Scott, Alexander Kutikov
Adrenocortical carcinoma (ACC) is a rare malignancy that is generally associated with a poor prognosis whose existence dictates the management of incidental renal masses. We report a case of ACC diagnosed and treated at its apparent inception in a patient undergoing close surveillance imaging of a prior malignancy. Despite timely detection and resection of a localized ACC this patient rapidly progressed to systemic disease. This case highlights the rapid growth kinetics of ACC and puts into perspective the challenges associated with the established treatment paradigm for patients diagnosed with an adrenal mass...
November 2016: Urology Case Reports
H Dralle
No abstract text is available yet for this article.
September 7, 2016: Der Chirurg; Zeitschrift Für Alle Gebiete der Operativen Medizen
S G Creemers, P M van Koetsveld, E S R van den Dungen, E Korpershoek, F J van Kemenade, G J H Franssen, W W de Herder, R A Feelders, L J Hofland
CONTEXT: Treatment of patients with adrenocortical carcinomas (ACC) with mitotane and/or chemotherapy is often associated with toxicity and poor tumor response. New therapeutic options are urgently needed. OBJECTIVE: To evaluate the therapeutic possibilities of temozolomide (TMZ) in ACC cells and to assess the potential predictive role of the DNA repair gene O6-Methylguanine-DNA methyltransferase (MGMT) in adrenocortical tumors. METHODS: 3 human ACC cell lines and 8 primary ACC cultures were used to assess effects of TMZ in vitro...
September 7, 2016: Journal of Clinical Endocrinology and Metabolism
Nada El Ghorayeb, Solange Grunenwald, Serge Nolet, Vanessa Primeau, Stéphanie Côté, Christine M Maugard, André Lacroix, Louis Gaboury, Isabelle Bourdeau
BACKGROUND: Adrenocortical carcinoma (ACC) may rarely be a component of inherited cancer syndromes such as Li-Fraumeni syndrome and Beckwith-Wiedemann syndrome. ACC caused by a BRCA2 mutation has never been reported. METHODS: Nucleotide sequencing of BRCA2 in lymphocyte and tumoral DNA of a 50-year-old male who presented with an androgen-secreting ACC and a strong family history of breast, ovarian, and pancreatic cancers. RESULTS: A germline BRCA2 2 bp heterozygous deletion at nucleotide 8765 (8765delAG) leading to a frameshift mutation (p...
September 2016: Medicine (Baltimore)
Paola Nolé, Britt Duijndam, Adam Stenman, C Christofer Juhlin, Mikael Kozyra, Catharina Larsson, Magnus Ingelman-Sundberg, Inger Johansson
Human cytochome P450 2W1 (CYP2W1) enzyme is expressed in fetal colon and in colon tumors. The level of expression is higher in colon metastases than in the parent tumors and the enzyme is a possible drug target for treatment of colorectal cancer, as demonstrated in mouse xenograft studies. A previous study published in this journal reported that CYP2W1 is highly expressed in normal and transformed adrenal tissue. However, adrenal expression of CYP2W1 protein was not seen in previous studies in our research group...
2016: PloS One
Jon M Gerry, Thuy B Tran, Lauren M Postlewait, Shishir K Maithel, Jason D Prescott, Tracy S Wang, Jason A Glenn, John E Phay, Kara Keplinger, Ryan C Fields, Linda X Jin, Sharon M Weber, Ahmed Salem, Jason K Sicklick, Shady Gad, Adam C Yopp, John C Mansour, Quan-Yang Duh, Natalie Seiser, Carmen C Solorzano, Colleen M Kiernan, Konstantinos I Votanopoulos, Edward A Levine, Ioannis Hatzaras, Rivfka Shenoy, Timothy M Pawlik, Jeffrey A Norton, George A Poultsides
BACKGROUND: Lymph node metastasis is an established predictor of poor outcome for adrenocortical carcinoma (ACC); however, routine lymphadenectomy during surgical resection of ACC is not widely performed and its therapeutic role remains unclear. METHODS: Patients undergoing margin-negative resection for localized ACC were identified from a multi-institutional database. Patients were stratified into 2 groups based on the surgeon's effort or not to perform a lymphadenectomy as documented in the operative note...
September 2, 2016: Annals of Surgical Oncology
Thomas G Papathomas, Eleonora Duregon, Esther Korpershoek, David F Restuccia, Ronald van Marion, Rocco Cappellesso, Nathalie Sturm, Giulio Rossi, Antonella Coli, Nicola Zucchini, Hans Stoop, Wolter Oosterhuis, Laura Ventura, Marco Volante, Ambrogio Fassina, Winand N M Dinjens, Mauro Papotti, Ronald R de Krijger
Adrenocortical carcinomas (ACCs) with sarcomatous areas represent an extremely rare type of highly aggressive malignancy of unknown molecular pathogenesis. The current study was planned to gain insight into its molecular genetics using a targeted next-generation sequencing approach and to explore the status of epithelial-mesenchymal transition (EMT)-associated markers (E-/P-/N-Cadherins, MMP-2/-9 and Caveolin-1), downstream transcriptional regulators of EMT-related signaling pathways (ZEB-1/-2, Slug), stem cell factors (Oct3/4, LIN28, SOX2, SO17, NANOG, CD133, nestin) and markers of adrenocortical origin/ tumorigenesis (SF-1, β-catenin, p53) in phenotypically diverse tumor components of six cases...
August 30, 2016: Human Pathology
Mabrouka Doghman-Bouguerra, Enzo Lalli
Adrenocortical carcinomas (ACC) are rare malignant tumors of the adrenal cortex whose prognosis remains ominous. Considerable progress has been made during the last few years in the identification of the molecular mechanisms implicated in their pathogenesis. Overexpression of the transcription factor Steroidogenic Factor-1 (SF-1/NR5A1), a pivotal regulator of adrenocortical development and steroidogenic function, has a stage-independent negative prognostic value in patients with ACC. (1) Studies in cell lines and animal models have shown that an increased SF-1 dosage is able to increase adrenocortical cell proliferation and triggers tumorigenesis...
August 11, 2016: Cell Cycle
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