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Adrenocortical cancer

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https://www.readbyqxmd.com/read/27899191/genetic-predisposition-to-endocrine-tumors-diagnosis-surveillance-and-challenges-in-care
#1
REVIEW
Elisabeth Joye Petr, Tobias Else
Endocrine tumor syndromes, eg, multiple endocrine neoplasia types 1 and 2, were among the first recognized hereditary predisposition syndromes to tumor development. Over time, the number of endocrine tumor syndromes has significantly expanded, eg, with the recent inclusion of hereditary paraganglioma syndromes. Associations of non-endocrine tumors with hereditary endocrine tumor syndromes and endocrine tumors with non-classical endocrine tumor syndromes have emerged. These findings have certainly expanded the scope of care, necessitating a multidisciplinary approach by a team of medical professionals and researchers, integrating shared patient decision-making at every step of surveillance, diagnosis, and treatment...
October 2016: Seminars in Oncology
https://www.readbyqxmd.com/read/27886397/telomerase-and-n-cadherin-differential-importance-in-adrenocortical-cancers-and-adenomas
#2
Sofia S Pereira, Valdemar Máximo, Ricardo Coelho, Rui Batista, Paula Soares, Susana G Guerreiro, Manuel Sobrinho-Simões, Mariana P Monteiro, Duarte Pignatelli
Adrenocortical carcinomas (ACC) are most frequently highly aggressive tumors. We assessed the telomerase reverse transcriptase (TERT) and N-cadherin role in the biology of ACC and their potential utility as molecular biomarkers, in different types of tumoral adrenocortical tissue. A total of 48 adrenal cortex samples (39 tumoral and 9 normal adrenal glands) were studied. TERT promoter mutations were searched by PCR and Sanger sequencing in two hotspots positions (-124 and -146). Also, telomerase and N-cadherin expression were evaluated by immunohistochemistry...
November 25, 2016: Journal of Cellular Biochemistry
https://www.readbyqxmd.com/read/27819856/investigating-the-chemokine-receptor-4-as-potential-theranostic-target-in-adrenocortical-cancer-patients
#3
Christina Bluemel, Stefanie Hahner, Britta Heinze, Martin Fassnacht, Matthias Kroiss, Thorsten A Bley, Hans-Juergen Wester, Saskia Kropf, Constantin Lapa, Andreas Schirbel, Andreas K Buck, Ken Herrmann
PURPOSE: Adrenocortical carcinoma (ACC) is a rare but aggressive endocrine tumor with limited treatment options. Preclinical studies confirmed overexpression of the chemokine receptor 4 (CXCR4) in this cancer type. This study aimed to analyze the role of CXCR4 imaging using Ga-pentixafor for ACC staging and selection of patients for CXCR4-directed endoradiotherapy. METHODS: Thirty patients with histologically proven advanced, metastasized ACC underwent F-FDG PET/CT and Ga-pentixafor PET/CT within a time interval of 3 ± 4 days to evaluate suitability for CXCR4-directed endoradiotherapy...
November 4, 2016: Clinical Nuclear Medicine
https://www.readbyqxmd.com/read/27819669/a-cdk2-activity-signature-predicts-outcome-in-cdk2-low-cancers
#4
S R McCurdy, M Pacal, M Ahmad, R Bremner
The role of cyclin-dependent kinase 2 (CDK2) in cancer is controversial. A major hurdle is the availability of tools to easily assess its activity across many samples. Here, we introduce a transcriptional signature to specifically track CDK2 activity. It responds to genetic and chemical perturbations in the CDK-RB-E2F axis, correlates with mitotic rate in vitro and in vivo and reacts rapidly to changes in CDK2 activity during cell cycle progression. We find that CDK2 activity is specifically elevated in human testes, mirroring its critical function in mice, and report very distinct profiles across human cancers...
November 7, 2016: Oncogene
https://www.readbyqxmd.com/read/27771624/non-androgen-secreting-adrenocortical-carcinoma-in-preadolescence-a-case-report-and-literature-review
#5
Hiroko Narumi, Shunji Hasegawa, Kazuyuki Waki, Ken Fukuda, Yuji Ohnishi, Takuya Ichimura, Yousuke Fujimoto, Shunsaku Katsura, Hiroo Kawano, Eiji Ikeda, Satoshi Okada, Shouichi Ohga
Adrenocortical carcinoma (ACC) is a rare malignancy in childhood. Affected children with ACC mostly present with virilization, but not the pure form of Cushing's syndrome. A 9-year-old Japanese girl was hospitalized, because of the unstable emotions and excessive weight gain. She was diagnosed as having Cushing's syndrome and a left adrenal tumor. The adrenalectomy led to the pathological diagnosis of ACC without metastasis. There was no mutation of PRKACA in the tumor-derived DNA, or p53 in peripheral blood-derived DNA...
October 24, 2016: Journal of Pediatric Endocrinology & Metabolism: JPEM
https://www.readbyqxmd.com/read/27770290/minimally-invasive-resection-of-adrenocortical-carcinoma-a-multi-institutional-study-of-201-patients
#6
Christina W Lee, Ahmed I Salem Md, David F Schneider, Glen E Leverson, Thuy B Tran, George A Poultsides, Lauren M Postlewait, Shishir K Maithel, Tracy S Wang, Ioannis Hatzaras, Rivfka Shenoy, John E Phay, Lawrence Shirley, Ryan C Fields, Linda X Jin, Timothy M Pawlik, Jason D Prescott, 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, Sharon M Weber
BACKGROUND AND OBJECTIVES: Minimally invasive surgery for adrenocortical carcinoma (ACC) is controversial. We sought to evaluate the perioperative and long-term outcomes following minimally invasive (MIS) and open resection (OA) of ACC in patients treated with curative intent surgery. METHODS: Retrospective data from patients who underwent adrenalectomy for primary ACC at 13 tertiary care cancer centers were analyzed, including demographics, clinicopathological, and operative outcomes...
October 21, 2016: Journal of Gastrointestinal Surgery: Official Journal of the Society for Surgery of the Alimentary Tract
https://www.readbyqxmd.com/read/27750238/us-us-fusion-imaging-in-radiofrequency-ablation-for-liver-metastases
#7
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
https://www.readbyqxmd.com/read/27742785/adrenocortical-cancer-a-molecularly-complex-disease-where-surgery-matters
#8
Eden C Payabyab, Sanjeeve Balasubramaniam, Maureen Edgerly, Margarita Velarde, Maria J Merino, Aradhana M Venkatesan, Harshraj Leuva, Thomas Litman, Susan E Bates, Tito Fojo
The development of new therapies has lagged behind for rare cancers without defined therapeutic targets. Adrenocortical cancer is no exception. Mitotane, an older agent considered "adrenolytic," is used both to control symptoms in advanced disease and as adjuvant therapy after surgical resection. Molecular characterization of adrenocortical cancer has deepened our understanding of this genetically complex disease while identifying subgroups whose importance remains to be determined. Unfortunately, such studies have yet to demonstrate a therapeutic target for drug development, and to date, no targeted therapy has achieved meaningful outcomes...
October 15, 2016: Clinical Cancer Research: An Official Journal of the American Association for Cancer Research
https://www.readbyqxmd.com/read/27725909/cell-cycle-dependent-rrm2-may-serve-as-proliferation-marker-and-pharmaceutical-target-in-adrenocortical-cancer
#9
Vince Kornél Grolmusz, Katalin Karászi, Tamás Micsik, Eszter Angéla Tóth, Katalin Mészáros, Gellért Karvaly, Gábor Barna, Péter Márton Szabó, Kornélia Baghy, János Matkó, Ilona Kovalszky, Miklós Tóth, Károly Rácz, Péter Igaz, Attila Patócs
Adrenocortical cancer (ACC) is a rare, but agressive malignancy with poor prognosis. Histopathological diagnosis is challenging and pharmacological options for treatment are limited. By the comparative reanalysis of the transcriptional malignancy signature with the cell cycle dependent transcriptional program of ACC, we aimed to identify novel biomarkers which may be used in the histopathological diagnosis and for the prediction of therapeutical response of ACC. Comparative reanalysis of publicly available microarray datasets included three earlier studies comparing transcriptional differences between ACC and benign adrenocortical adenoma (ACA) and one study presenting the cell cycle dependent gene expressional program of human ACC cell line NCI-H295R...
2016: American Journal of Cancer Research
https://www.readbyqxmd.com/read/27714481/tp53-and-cdkn1a-mutation-analysis-in-families-with-li-fraumeni-and-li-fraumeni-like-syndromes
#10
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
https://www.readbyqxmd.com/read/27705928/unraveling-the-expression-of-the-oncogene-yap1-a-wnt-beta-catenin-target-in-adrenocortical-tumors-and-its-association-with-poor-outcome-in-pediatric-patients
#11
Rafael H Abduch, Ana Carolina Bueno, Leticia F Leal, Marcelo M Cavalcanti, Débora C Gomes, Silvia R Brandalise, Maria J Masterallo, José A Yunes, Carlos E Martinelli, Luiz G Tone, Silvio Tucci, Carlos A F Molina, Fernando S Ramalho, Ayrton C Moreira, Izilda A Cardinalli, Carlos A Scrideli, Leandra N Z Ramalho, Margaret de Castro, Sonir R Antonini
BACKGROUND: Overexpression of the oncogene yes-associated-protein-1 (YAP1) is associated with increased cell proliferation in human cancers. YAP1 is a potential target of the Wnt/beta-catenin pathway, which plays an important role in adrenocortical tumors (ACT). The role of YAP1 in adrenocortical tumorigenesis has not been assessed. AIMS: To evaluate YAP1 expression in normal adrenals and pediatric ACT and its association with disease outcome. To investigate the interaction between YAP1 and the Wnt/beta-catenin pathway in adrenocortical cells...
October 1, 2016: Oncotarget
https://www.readbyqxmd.com/read/27663983/the-inherited-p53-mutation-in-the-brazilian-population
#12
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...
December 1, 2016: Cold Spring Harbor Perspectives in Medicine
https://www.readbyqxmd.com/read/27626976/antisecretive-and-antitumor-activity-of-abiraterone-acetate-in-human-adrenocortical-cancer-a-preclinical-study
#13
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
https://www.readbyqxmd.com/read/27625633/alterations-of-phosphodiesterases-in-adrenocortical-tumors
#14
REVIEW
Fady Hannah-Shmouni, Fabio R Faucz, Constantine A Stratakis
Alterations in the cyclic (c)AMP-dependent signaling pathway have been implicated in the majority of benign adrenocortical tumors (ACTs) causing Cushing syndrome (CS). Phosphodiesterases (PDEs) are enzymes that regulate cyclic nucleotide levels, including cyclic adenosine monophosphate (cAMP). Inactivating mutations and other functional variants in PDE11A and PDE8B, two cAMP-binding PDEs, predispose to ACTs. The involvement of these two genes in ACTs was initially revealed by a genome-wide association study in patients with micronodular bilateral adrenocortical hyperplasia...
2016: Frontiers in Endocrinology
https://www.readbyqxmd.com/read/27617213/biology-is-destiny-a-case-of-adrenocortical-carcinoma-diagnosed-and-resected-at-inception-in-a-patient-under-close-surveillance-for-lung-cancer
#15
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
https://www.readbyqxmd.com/read/27603910/inhibition-of-human-adrenocortical-cancer-cell-growth-by-temozolomide-in-vitro-and-the-role-of-the-mgmt-gene
#16
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
https://www.readbyqxmd.com/read/27603373/first-case-report-of-an-adrenocortical-carcinoma-caused-by-a-brca2-mutation
#17
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)
https://www.readbyqxmd.com/read/27598485/human-cytochrome-p450-2w1-is-not-expressed-in-adrenal-cortex-and-is-only-rarely-expressed-in-adrenocortical-carcinomas
#18
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
https://www.readbyqxmd.com/read/27594532/the-er-mitochondria-couple-in-life-and-death-from-steroidogenesis-to-tumorigenesis
#19
Mabrouka Doghman-Bouguerra, Enzo Lalli
Steroidogenesis is a multistep process where interorganelle communications between the endoplasmic reticulum and mitochondria are critical. These intimate interactions physically occur through the Mitochondria-Associated ER membranes called MAMs. MAMs play important roles in mitochondrial morphology and in many cellular functions ranging from lipid metabolism, to calcium signaling and apoptosis together with a critical effect on steroidogenesis. Moreover, our recent characterization of new MAM resident proteins in adrenocortical cells extends the function of MAM in the mechanism of resistance of cancer cells to apoptotic stimuli and offers new perspectives in targeted therapeutic approaches for adrenocortical tumorigenesis...
September 1, 2016: Molecular and Cellular Endocrinology
https://www.readbyqxmd.com/read/27566097/prognosis-of-patients-with-metastatic-spinal-cord-compression-from-adrenocortical-carcinoma
#20
Stefan Janssen, Tobias Bartscht, Dirk Rades
BACKGROUND/AIM: Adrenocortical cancer is a rare aggressive type of cancer. The prognosis is poor, particularly for metastatic disease. This study focused on metastatic spinal cord compression (MSCC) from adrenocortical carcinoma. PATIENTS AND METHODS: Data of three patients who received palliative irradiation of MSCC from adrenocortical carcinoma were retrospectively analyzed for motor function, ambulatory status and survival. RESULTS: One patient died before completion of radiotherapy...
September 2016: In Vivo
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