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Christofer Juhlin C C Yale Endocrine Neoplasia Laboratory, Yale School of Medicine, 333 Cedar St, FMB130A, Box 208062, New Haven, CT 06520, USA (CCJ, JMH, ALF, JWK, TCB, RK, TC); Department of Surgery, Yale School of Medicine, New Haven, CT, 06520, USA (CCJ, JMH, ALF, JWK, TCB, RK, TC); Department of Genetics, Yale School of Medicine and Howard Hughes Medical Institute, New Haven, CT, 06520, USA (GG, CNW, MC, RPL); Department of Oncology-Pathology, Karolinska Institutet, Karolinska University Hospital, CCK, SE-171, 76 Stockholm, Sweden (CCJ, AS, AH, CL); Yale Center for Genome Analysis, Orange, CT, 06477, USA (JDO, SMM); Department of Pathology, Yale School of Medicine, New Haven, CT, 06520, USA (DLR, MLP); Department of Pharmacology, Yale School of Medicine, New Haven, CT 06520, USA (JS); Department of Molecular Medicine and Surgery, Karolinska Institutet, Karolinska University Hospital, SE-171 76 Stockholm, Sweden (MB); Division of Nephrology, University Hospital Düsseldorf, 40225 Düsseldorf, Germany (UIS); Department of Pathology, University Hospital Düsseldorf, 40225 Düsseldorf, Germany (ACS); Division of Endocrinology and Diabetology, University Hospital Düsseldorf, 40225 Düsseldorf, Germany - - 2014
Context: Adrenocortical carcinoma (ACC) is a rare and lethal malignancy with a poorly defined etiology, and the molecular genetics of ACC are incompletely understood. Objective: Utilize whole-exome sequencing for genetic characterization of the underlying somatic mutations and copy number alterations (CNA) present in ACC. Design: Screening for somatic mutation events ...
Babi Ska Anna - - 2014
Adrenal tumors are detected incidentally in 4-8% of patients in imaging studies. Adenomas, pheochromocytomas and adrenocortical carcinomas represent the most common tumors of the adrenal glands. Rarely final histopathological reports are surprising.Aim: The aim of our study is a retrospective analysis of selected clinical characteristics and hormonal studies in five ...
Agrawal Vishal V Department of Pediatrics, University of California, San - - 2014
BACKGROUNDAt birth, the large fetal adrenal involutes rapidly and the patterns of steroidogenesis change dramatically; the event(s) triggering these changes remain largely unexplored. Fetal abdominal viscera receive hypoxic blood having a partial pressure of oxygen of only ~2 kPa (20-23 mmHg); perinatal circulatory changes change this to adult values (~20 ...
Lu Hao - - 2014
BackgroundIn prognosis and therapeutics of adrenal cortical carcinoma (ACC), the selection of the most active areas in proliferative rate (hotspots) within a slide and objective quantification of immunohistochemical Ki67 Labelling Index (LI) are of critical importance. In addition to intratumoral heterogeneity in proliferative rate i.e. levels of Ki67 expression within ...
Duan Kai K Department of Pathology, University Health Network, Toronto, Ontario, Canada Department of Laboratory Medicine and Pathobiology, University of Toronto, Ontario, - - 2014
Endogenous Cushing's syndrome is a rare endocrine disorder that incurs significant cardiovascular morbidity and mortality, due to glucocorticoid excess. It comprises adrenal (20%) and non-adrenal (80%) aetiologies. While the majority of cases are attributed to pituitary or ectopic corticotropin (ACTH) overproduction, primary cortisol-producing adrenal cortical lesions are increasingly recognised in ...
Burotto Mauricio M Center for Cancer Research, National Cancer Institute, National Institutes of Health - - 2014
Introduction: Adrenocortical carcinoma (ACC) is a heterogeneous and rare disease. At presentation or at the time of a recurrence the disease commonly spreads to the liver, lungs, lymph nodes, and bones. The brain has only rarely been reported as a site of metastases. Objective: The aims of the study were ...
Salenave Sylvie S S Salenave, Department of Endocrinology and Reproductive Medicine, CHU de Bicêtre, le Kremlin-Bicêtre, - - 2014
Context: Mitotane (M) is an adrenolytic and anticortisolic drug used in adrenocortical carcinoma (ACC), Cushing disease (CD) and ectopic ACTH syndrome. Its effects on the ovaries are unknown. Objective: To evaluate the ovarian and gonadotrope effects of Mitotane therapy in premenopausal woman. Patients: We studied 21 premenopausal women (ACC: n=13; ...
Tada Hayato H Division of Cardiovascular Medicine Kanazawa University Graduate School of - - 2014
We herein report a case of marked transient hypercholesterolemia in a man receiving low-dose mitotane as adjuvant chemotherapy for adrenocortical carcinoma. A 58-year-old man without any clinical symptoms or history of hypercholesterolemia was admitted to our hospital to treat an adrenocortical carcinoma detected on general screening using computed tomography. He ...
Bellantone Rocco R Division of Endocrine and Metabolic Surgery, Università Cattolica del Sacro Cuore, Rome, - - 2014
The role of endoscopic adrenalectomy for adrenocortical carcinoma is the most controversial and debated points in adrenal surgery. We reviewed the most recent literature on this topic. From the amount of available data (even if not conclusive), the following could be extrapolated: first, for patients with apparently localized disease the ...
Livhits Masha M Section of Endocrine Surgery, UCLA David Geffen School of Medicine, Los Angeles, - - 2014
Adrenocortical carcinoma (ACC) is a rare but lethal tumor. Predictors of survival include earlier stage at presentation and complete operative resection. We assessed effect of treatment and demographic variables on survival. ACC cases were abstracted from the California Cancer Registry and Office of Statewide Health Planning and Development (1999-2008). Predictors ...
Asare Elliot A EA American Joint Committee on Cancer (AJCC), Chicago, IL; Cancer Programs, American College of Surgeons, Chicago, IL; Department of Surgery, Medical College of Wisconsin, Milwaukee, WI. Electronic address: - - 2014
Current American Joint Committee on Cancer/International Union against Cancer (AJCC/UICC) and European Network for the Study of Adrenal Tumors staging for adrenocortical carcinoma (ACC) have not shown a survival difference between patients with stage I/II disease. This study evaluates current staging systems for survival prediction using a larger cohort and ...
Courtney Rachael R Divisions of *Pediatric Hematology/Oncology †Pediatric Pathology, Children's Hospital of Pittsburgh of University of Pittsburgh Medical Center, Pittsburgh, - - 2014
We present an infant with 2 simultaneous, but histologically distinct tumors with a novel germline p53 mutation. The child was found to have a paraspinal neuroblastoma, a concurrent adrenal cortical carcinoma, and an I162F p53 gene mutation. We review the associations of germline p53 mutations (or Li-Fraumeni syndrome) with both ...
Ui Akiko A Department of Thoracic Surgery, Tokyo Metropolitan Cancer and Infectious Disease Center Komagome Hospital, Tokyo, - - 2014
A 43-year-old man presented with a pulmonary nodule in the left lower lobe and he consulted our hospital one year after resection of left adrenocortical carcinoma. We performed a wedge resection of the left lower pulmonary lobe for diagnosis. The tumor was diagnosed as a metastatic sarcoma, but the primary ...
Huang Dihua D Department of Endocrinology and Metabolism, Shaoxing People's Hospital, Shaoxing Hospital of Zhejiang University, Shaoxing, Zhejiang 312000, P.R. - - 2014
Head and neck paraganglioma is a rare and predominantly asymptomatic tumor. In the present study, an extremely rare case of asymptomatic paraganglioma located between the left common carotid artery and the left thyroid is described. The clinical presentation, cytomorphology and the immunohistochemical characteristics for the diagnosis of head and neck ...
Almarzouq Ahmad - - 2014
Adrenocortical carcinoma is a rare and aggressive malignancy. Patients usually present early with manifestation of abnormal hormone secretion. However, adrenocortical carcinoma can also be nonfunctioning, and such patients present late with a mass effect or distant metastases. We herein report a case of a 30-year-old Sri-Lankan woman who presented with ...
Wu Deyao D Department of Urology, The Fourth Affiliated Hospital of Nantong Medical College, Yancheng City First People's Hospital, Yancheng, Jiangsu 224001, P.R. - - 2014
microRNA‑99a (miR‑99a) was reported to be among the most frequently downregulated miRNAs in numerous types of human cancers, including prostate, bladder, hepatocellular and ovarian carcinoma, squamous cell carcinoma of the tongue, squamous cell lung carcinoma as well as childhood adrenocortical tumors. The aim of the present study was to determine the ...
Wei Yong-Bao YB Department of Urology, The Second Xiangya Hospital, Central South University, Changsha, Hunan 410011, P.R. - - 2015
Adrenocortical carcinoma (ACC) is a rare, but highly aggressive type of tumor with an incidence of one to two per million annually. Adrenocortical carcinosarcoma is an exceptional variant of ACC, which is characterized by the presence of histological regions of carcinoma and sarcoma. To date, to the best of our ...
Gordon Benjamin L BL Research Fellow, Department of Surgery, Weill Cornell Medical College/New York Presbyterian Hospital, NY, New - - 2014
Genomic diagnostics in cancer has evolved since the completion of the Human Genome Project and the advancements made in diagnosis and therapy in chronic myelogenous leukemia. Among the diseases to achieve limited success or potentially benefit from diagnostic genetic testing are thyroid cancer, Burkitt's lymphoma, gastrointestinal stromal tumors, adrenocortical carcinoma, ...
Ip Julian C Y JC Cancer Genetics, Kolling Institute of Medical Research, Royal North Shore Hospital and The University of Sydney, Sydney, NSW, - - 2014
Adrenocortical carcinoma (ACC) is a rare malignancy that carries a poor prognosis. There has yet to be a large Australian series that documents the characteristics of ACC and there are a paucity of data on management and the long-term outcomes. We sought to provide a unique insight into the management ...
Robitaille Christina N CN INRS-Institut Armand-Frappier, Université du Québec, 531 boulevard des Prairies, Laval, QC, H7V 1B7, - - 2014
Several pesticides suspected or known to have endocrine disrupting effects were screened for pro- or antiandrogenic proprieties by determining their effects on proliferation, prostatic specific antigen (PSA) secretion and androgen receptor (AR) expression and AR phosphorylation in androgen-dependent LNCaP human prostate cancer cells, as well as on the expression and ...
Chen Jin J Department of Biochemistry and Molecular Biology, Sichuan University, Chengdu, Sichuan 610072, P.R. - - 2014
In the last decade, the overexpression of hepatoma upregulated protein (HURP) has been reported in hepatocellular carcinoma, adrenocortical tumors and urogenital carcinoma. However, the role of HURP in breast cancer remains unknown. In the present study, a comprehensive analysis was performed to examine the HURP expression level in 43 breast ...
Gaujoux S S Department of Digestive and Endocrine Surgery, Cochin Hospital, AP-HP, 27, rue du Faubourg-Saint-Jacques, 75014 Paris, France; Université Paris Descartes, 12, rue de l'École-de-Médecine, 75006 Paris, - - 2014
Tong Shi-Jun SJ Department of Urologic Surgery, Huashan Hospital Affiliated to Fudan University, Shanghai 200040, P.R. - - 2014
microRNAs (miRNAs) are a class of short noncoding RNA molecules that have a critical role in the initiation and progression of types of human cancer, including prostate cancer. In the present study, the expression of miR-181 in prostate cancer tissues was evaluated and was demonstrated to be significantly upregulated in ...
Scatena Cristian C 1Department of Pathology 2Department of Experimental and Biomedical Sciences, University of Florence, - - 2014
Adrenocortical carcinoma (ACC) is a rare and heterogeneous malignancy, which gains access to the circulation very early during disease progression. Within this scenario, detection of circulating tumor cells (CTCs), released into the peripheral blood before any detectable metastasis is established, may represent a promising diagnostic tool in ACC patients. Our ...
Basso F F INSERM U1016, CNRS (UMR 8104), Institut Cochin, Université Paris Descartes, Sorbonne Paris Cité, Faculté de Médecine, Paris, France, Paris, - - 2014
The cyclic AMP/protein kinase A signaling cascade is one of the main pathways involved in the pathogenesis of adrenocortical tumors. The PKA R1A and R2B proteins are the most abundant regulatory subunits in endocrine tissues. Inactivating mutations of PRKAR1A are associated with Carney complex and a subset of sporadic tumors ...
Feinmesser Meora M Departments of *Pathology †Endocrinology, Beilinson Hospital, Rabin Medical Center (affiliated with Sackler School of Medicine, Tel Aviv University, Tel Aviv) §Department of Pediatric Endocrinology, Schneider Children's Medical Center of Israel (affiliated with Sackler School of Medicine, Tel Aviv University, Tel Aviv), Petach Tikva ‡Rosetta Genomics, Rehovot, - - 2014
MicroRNAs (miRs) play a central role in regulating gene expression and are strongly associated with cancer development. This study sought to determine if adrenocortical carcinomas can be differentiated from adenomas by their miR profiles and to correlate the findings with the histologic Weiss system for identifying malignancy in adrenocortical tumors ...
Sasano Hironobu H Department of Pathology, Tohoku University School of Medicine, 2-1 Seiryou-machi, Aoba-ku, Sendai, 980-8575, Japan, - - 2014
Adrenocortical carcinoma is a rare malignancy. Medical treatment including op'DDD or mitotane with or without platinum-based cytotoxic chemotherapy is frequently administered to the patients in an adjuvant setting following surgery or in advanced disease, because of aggressive clinical behavior in some cases. Potential roles of pathologists in determining the clinical ...
Sato Naoko N Tohoku University Graduate School of Medicine, Sendai, - - 2014
Corticotropin-releasing hormone (CRH), a major regulator of the stress response, regulates various biological functions through its interaction with CRH receptors 1 (CRHR1) and 2 (CRHR2). CRH, CRHR1, and CRHR2 have recently been reported in several types of carcinoma, but the significance of these proteins has remained largely unknown in human ...
Kerkhofs T M A TM Department of Internal Medicine, Máxima Medical Centre, Eindhoven/Veldhoven, The - - 2014
Adrenocortical carcinoma (ACC) is rare in both adult and pediatric populations. Literature suggests significant differences between children and adults in presentation, histological properties and outcome. The aim of this first nationwide study on pediatric ACC was to describe the incidence, presentation, pathological characteristics, treatment and survival in The Netherlands. All ...
Etzold Anna A Institute of Human Genetics, University Medical Centre of the Johannes Gutenberg University Mainz, Langenbeckstraße 1, 55131, Mainz, Germany, - - 2014
Li-Fraumeni syndrome (LFS) is a rare genetic disease with a highly significant predisposition to multiple early-onset neoplasms. These neoplasms include adrenocortical carcinoma, sarcoma, leukemia and CNS tumors in children and sarcoma, breast cancer and lung cancer in adults. LFS is inherited in an autosomal dominant manner. In most patients germline ...
Sadahira Ken K Division of Hematology, Department of Internal Medicine, Keio University School of Medicine, Tokyo 160-8582, - - 2014
Multiple myeloma (MM) is a clonal plasma cell disorder affecting the immune system with various systemic symptoms. MM remains incurable even with high dose chemotherapy using conventional drugs, thus necessitating development of novel therapeutic strategies. Gossypol (Gos) is a natural polyphenolic compound extracted from cotton plants, and has been shown to ...
Jones Robin L RL Clinical Research Division, Fred Hutchinson Cancer Research Center - - 2014
Purpose: We determined the maximum tolerated dose (MTD), safety, pharmacokinetics, pharmacodynamics, and preliminary activity of OSI-906, a potent, oral, dual inhibitor of insulin-like growth factor-1 receptor (IGF-1R) and insulin receptor (IR), in patients with advanced solid tumors. Methods: This was a multicenter, open-label, dose escalation phase I study evaluating three ...
Faria André M AM Unidade de Suprarrenal & Unidade de Endocrinologia do Desenvolvimento, Laboratório de Hormônios e Genética Molecular LIM42, Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo, São Paulo, - - 2014
LIN28 control cells reprogramming and pluripotency mainly through miRNA regulation and has been overexpressed in many advanced cancers. In this study, we evaluated the prognostic role of LIN28 and its regulatory miRNAs in a large cohort of adrenocortical tumors (ACTs). LIN28 protein expression was assessed in 266 adults ACTs (78 ...
Kerkhofs Thomas T T Kerkhofs, internal medicine, Maxima Medisch Centrum, Eindhoven , 5600 PD , Netherlands - - 2014
Objective: Mitotane is the drug of choice in patients with adrenocortical carcinoma. The anti-neoplastic effect is correlated with mitotane plasma levels, which renders it crucial to reach and maintain the concentration above 14mg/L. However, mitotane pharmacokinetics are poorly understood. Aim of the present study was to investigate the variation of ...
Lerario Antonio M AM Division of Metabolism, Endocrinology & Diabetes, Medical School, University of Michigan, 109 Zina Pitcher Place, 1528 BSRB, Ann Arbor, MI, 48109, - - 2014
Dall'Igna Patrizia P Pediatric Surgery Department, University-Hospital of Padua, Padua, - - 2014
Adrenocortical tumors are very rare in children. The distinction between adenoma and carcinoma is complex because of their clinical/histological characteristics. The analysis of the cases registered in two consecutive Italian Studies is described, in order to provide additional insight into their nature and possibly identify benign and malignant lesions. The ...
Chang Kyung Yoon KY Division of Nephrology, Department of Internal Medicine, St. Vincent's Hospital, College of Medicine, The Catholic University of Korea, Suwon, - - 2014
Rhee Harin H Department of Internal Medicine, Pusan National University School of Medicine, Busan, - - 2014
Leite Fabíola A FA Department of Pediatrics, Ribeirão Preto Medicine School, University of São Paulo, Ribeirão Preto, - - 2014
Low expression of HLA class II antigens has been associated with more aggressive disease in several human malignancies including adult adrenocortical tumors (ACT), but their clinical relevance in pediatric ACT needs to be investigated. This study analyzed the expression profile of three class II histocompatibility genes (HLA-DRA, HLA-DPA1, and HLA-DPB1) ...
Dong Peixin - - 2014
Activation of the PI3K/AKT pathway, a common mechanism in all subtypes of endometrial cancers (endometrioid and non-endometrioid tumors), has important roles in contributing to epithelial-mesenchymal transition (EMT) and cancer stem cell (CSC) features. MicroRNAs (miRNAs) are small non-coding RNA molecules that concurrently affect multiple target genes, and regulate a wide ...
Jiang Hong-Lei HL The Fourth Affiliated Hospital of China Medical University, Shenyang, - - 2014
Background/Aims: Hepatocellular carcinoma (HCC) represents the most common type of liver cancer. DAX1 (dosage-sensitive sex reversal adrenal hypoplasia congenital critical region on X chromosome, gene 1), an atypical member of the nuclear receptor family due to lack of classical DNA-binding domains, has been known for its fundamental roles in the ...
Hamada Taiji T Department of Molecular and Cellular Pathology, Kagoshima University Graduate School of Medical and Dental Sciences, Kagoshima, - - 2014
The PCP4/PEP19 is a calmodulin-binding anti-apoptotic peptide in neural cells but its potential role in human cancer has largely been unknown. We investigated the expression of PCP4/PEP19 in human breast cancer cell lines MCF-7, SK-BR-3, and MDA-MB-231 cells, and found that estrogen receptor (ER)-positive MCF-7 and ER-negative SK-BR-3 cells expressed ...
Rajab Nor Fadilah - - 2014
Selective Alzheimer Disease Indicator-1 (or Seladin-1) is a multifunctional protein first discovered by downregulation of its expression in Alzheimer's disease. Interestingly, the expression of this protein is upregulated in several cancers, including primary bladder cancer. However, its role in cancer formation has yet to be discovered. Goniothalamin is a natural ...
Gagliano Teresa T Section of Endocrinology, Department of Medical Sciences, University of Ferrara, Via A. Moro, 8, 44124, Ferrara, - - 2014
Mitotane is currently employed as adjuvant therapy as well as in the medical treatment of adrenocortical carcinoma (ACC), alone or in combination with chemotherapeutic agents. It was previously demonstrated that mitotane potentiates chemotherapeutic drugs cytotoxicity in cancer cells displaying chemoresistance due to P-glycoprotein (P-gp), an efflux pump involved in cancer ...
Dy Benzon M BM Department of Surgery, Mayo Clinic, Rochester, MN, USA, - - 2014
Metastatic adrenocortical carcinoma (ACC) is rapidly fatal, with few options for treatment. Patients with metachronous recurrence may benefit from surgical resection. The survival benefit in patients with hematogenous metastasis at initial presentation is unknown. A review of all patients undergoing surgery (European Network for the Study of Adrenal Tumors) stage ...
Halperin Daniel M - - 2014
Patients with advanced endocrine cancers, such as adrenocortical carcinoma and medullary thyroid carcinoma, have few well-validated therapeutic options. Pre-clinical studies have suggested potential activity of imatinib in these tumors. We therefore sought to establish a safe, novel treatment regimen combining imatinib with cytotoxic chemotherapy for future study in endocrine cancers. ...
Li Kai K Department of Obstetrics and Gynecology, The Second Xiangya Hospital, Central South University, Changsha, Hunan 410011, P.R. - - 2014
Steroid cell tumors not otherwise specified (NOS) are a rare subgroup of sex cord-stromal tumors. The tumors can occur at any age, although the mean age of occurrence is 43 years old. The majority are benign, but have the capability of producing one or more steroids associated with virilization. The ...
Ross J S JS Department of Pathology and Laboratory Medicine, Albany Medical College, Albany, New York, USA Foundation Medicine, Inc., Cambridge, Massachusetts, - - 2014
Adrenocortical carcinoma (ACC) carries a poor prognosis and current systemic cytotoxic therapies result in only modest improvement in overall survival. In this retrospective study, we performed a comprehensive genomic profiling of 29 consecutive ACC samples to identify potential targets of therapy not currently searched for in routine clinical practice. DNA ...
Bala Margarita - - 2014
Kaposi sarcoma (KS) is a malignant disease most commonly diagnosed in the setting of a human immunodeficiency virus (HIV) infection and in patients receiving immunosuppressive treatment. Pulmonary KS has never been reported in association with endogenous Cushing's syndrome (CS). A 60-year-old woman presented with symptoms and signs of CS. Adrenal ...
Gratian Lauren L Division of Endocrinology, Department of Medicine, Duke University School of Medicine, Durham, NC, - - 2014
Adrenocortical carcinoma (ACC) is a rare, aggressive disease with no apparent change in treatment or survival in the United States over the past two decades. Our objective was to determine whether treatment patterns or clinical outcomes vary by hospital case volume. Patients with ACC were identified from the National Cancer ...
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