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Adrenocortical carcinoma

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https://www.readbyqxmd.com/read/29452402/mitotane-monotherapy-in-patients-with-advanced-adrenocortical-carcinoma
#1
Felix Megerle, Wiebke Herrmann, Wiebke Schloetelburg, Cristina L Ronchi, Alina Pulzer, Marcus Quinkler, Felix Beuschlein, Stefanie Hahner, Matthias Kroiss, Martin Fassnacht
Context: While mitotane is the only approved drug for the treatment of adrenocortical carcinoma (ACC), data on monotherapy in advanced disease is still scarce. Objective: To assess the efficacy of mitotane in advanced ACC in a contemporary setting and to identify predictive factors. Design/Setting: Multicenter cohort study of three German referral centers. Patients: 127 patients with advanced ACC treated with mitotane monotherapy...
February 14, 2018: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/29444511/analysis-of-10-adrenocortical-carcinoma-patients-in-the-cohort-of-the-precision-medicine-platform-mondti
#2
Markus Kieler, Leonhard Müllauer, Oskar Koperek, Daniela Bianconi, Matthias Unseld, Markus Raderer, Gerald W Prager
OBJECTIVE: Adrenocortical carcinoma (ACC) is a rare disease with a dismal prognosis. We aimed to evaluate if a personalized medicine approach may be useful for matching patients with ACC to targeted therapies. METHODS: This is an analysis of 10 molecularly profiled ACCs that were progressing under standard of care treatment. The profile consisted of a 50-gene next-generation sequencing panel, immunohistochemistry (IHC), and fluorescence in situ hybridization for several proteins or chromosomal aberrations...
February 14, 2018: Oncology
https://www.readbyqxmd.com/read/29442479/adrenocortical-carcinoma-where-we-stand
#3
Raffaele Pezzani
No abstract text is available yet for this article.
February 13, 2018: Minerva Endocrinologica
https://www.readbyqxmd.com/read/29435068/common-module-analysis-reveals-prospective-targets-and-mechanisms-of-pediatric-adrenocortical-adenoma-and-carcinoma
#4
Anurag Kulshrestha, Shikha Suman
Pediatric adrenocortical carcinoma and adrenocortical adenoma are two rare diseases affecting children. Molecular analyses were performed to identify commonalities in gene expression between the diseases. Differentially expressed genes were identified for the pediatric adrenocortical adenoma and carcinoma tissues, as compared with normal tissues, using the expression dataset. Protein-protein interaction (PPI) networks were constructed for adenoma and carcinoma disease models, and common modules among the diseases were identified...
March 2018: Oncology Letters
https://www.readbyqxmd.com/read/29428231/bcl9-upregulation-in-adrenocortical-carcinoma-a-novel-wnt-%C3%AE-catenin-activating-event-driving-adrenocortical-malignancy
#5
Taylor C Brown, Norman G Nicolson, Reju Korah, Tobias Carling
BACKGROUND: B-Cell CLL/Lymphoma 9 (BCL9) is a recently described oncogene that promotes tumorigenesis via activation of the Wnt/β-Catenin signaling cascade. Though constitutively active Wnt/β-Catenin signaling is a molecular hallmark of adrenocortical carcinoma (ACC), a potential role for BCL9 to promote Wnt/β-Catenin pathway dysregulation in adrenocortical tumorigenesis remains to be elucidated. STUDY DESIGN: This study involved a retrospective analysis at a tertiary academic referral center of 27 patients with adrenocortical tumors, including in vitro investigation of BCL9...
February 8, 2018: Journal of the American College of Surgeons
https://www.readbyqxmd.com/read/29426273/testosterone-and-cortisol-secreting-oncocytic-adrenocortical-adenoma-in-the-pediatric-age-group
#6
Maryam K Al Badi, Ibrahim Al-Alwan, Mohammed Al-Dubayee, Ayed Al-Anzi, Maram S Al Turki, Noura Aloudah, Khaled O Alsaad
Oncocytic tumors are epithelial neoplasms that occur in various organs, including adrenal glands. Oncocytic adrenocortical adenomas and carcinomas are uncommon but well-known pathological entities in adults. However, generally oncocytic tumors, particularly in the adrenal glands, are very rare in the pediatric age-group. Most oncocytic adrenal tumors are not functional. We present a rare case of right-sided, functional oncocytic adrenocortical adenoma in a 5-year-old boy, who presented with clinical manifestations of precocious puberty and Cushing syndrome...
January 1, 2018: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/29411361/targeting-the-multidrug-transporter-patched-potentiates-chemotherapy-efficiency-on-adrenocortical-carcinoma-in-vitro-and-in-vivo
#7
Anida Hasanovic, Carmen Ruggiero, Sara Jung, Ida Rapa, Laurie Signetti, Monia Ben Hadj, Massimo Terzolo, Felix Beuschlein, Marco Volante, Constanze Hantel, Enzo Lalli, Isabelle Mus-Veteau
One of the crucial challenges in the clinical management of cancer is the resistance to chemotherapeutics. We recently demonstrated that the Hedgehog receptor Patched, which is overexpressed in many recurrent and metastatic cancers, is a multidrug transporter for chemotherapeutic agents such as doxorubicin. The present work provides evidences that Patched is expressed in adrenocortical carcinoma (ACC) patients, and is a major player of the doxorubicin efflux and the doxorubicin resistance in the human ACC cell line H295R...
February 7, 2018: International Journal of Cancer. Journal International du Cancer
https://www.readbyqxmd.com/read/29403275/development-of-biocompatible-and-vegf-targeted-paclitaxel-nanodrugs-on-albumin-and-graphene-oxide-dual-carrier-for-photothermal-triggered-drug-delivery-in-vitro-and-in-vivo
#8
Wentao Deng, Juhui Qiu, Shaoting Wang, Zhi Yuan, Yuefeng Jia, Hailin Tan, Jiru Lu, Ruqiang Zheng
In this study, we performed the characterization and synthesis of biocompatible and targeted albumin and graphene oxide (GO) dual-carrier paclitaxel (PTX) nanoparticles for photothermal-triggered tumor therapy. PTX absorbed on GO nanosheets as cores were coated with human serum albumin (HSA), following surface conjugation with monoclonal antibodies (mAb) against vascular endothelial growth factor (VEGF; denoted as mAbVEGF) via polyethylene glycol linker to form targeted nanoparticles (PTX-GHP-VEGF). The spherical nanoparticles were 191±5 nm in size with good stability and biocompatibility...
2018: International Journal of Nanomedicine
https://www.readbyqxmd.com/read/29403152/adrenocortical-carcinoma-characterized-by-gynecomastia-a-case-report
#9
Takako Takeuchi, Yuko Yoto, Akira Ishii, Takeshi Tsugawa, Masaki Yamamoto, Tsukasa Hori, Hotaka Kamasaki, Kazutaka Nogami, Takanori Oda, Akihiro Nui, Sachiko Kimura, Takuya Yamagishi, Keiko Homma, Tomonobu Hasegawa, Maki Fukami, Yoko Watanabe, Hidehiko Sasamoto, Hiroyuki Tsutsumi
We present a 4-yr-old boy with adrenocortical carcinoma (ACC), diagnosed due to the appearance of gynecomastia as the presenting symptom. Six months prior to admission, an acute growth spurt along with the development of bilateral breast swelling was observed. He did not present any features of virilization, including enlargement of the testes, increase in testis volume, and penis size. Laboratory investigations showed gonadotropin-independent hypergonadism, with low LH/ FSH levels and elevated estradiol/testosterone levels...
2018: Clinical Pediatric Endocrinology: Case Reports and Clinical Investigations: Official Journal of the Japanese Society for Pediatric Endocrinology
https://www.readbyqxmd.com/read/29390437/chronic-primary-adrenal-insufficiency-after-unilateral-adrenonephrectomy-a-case-report
#10
Satoshi Yoshiji, Kimitaka Shibue, Toshihito Fujii, Takeshi Usui, Keisho Hirota, Daisuke Taura, Mayumi Inoue, Masakatsu Sone, Akihiro Yasoda, Nobuya Inagaki
RATIONALE: Unilateral adrenalectomy as part of surgical resection of renal cell carcinoma (RCC) is not thought to increase the risk of chronic adrenal insufficiency, as the contralateral adrenal gland is assumed to be capable of compensating for the lost function of the resected gland. However, recent studies have indicated that adrenalectomy might cause irreversible impairment of the adrenocortical reserve. We describe a case of chronic primary adrenal insufficiency in a 68-year-old man who previously underwent unilateral adrenonephrectomy, which was complicated by severe postoperative adrenal stress that involved cardiopulmonary disturbance and systemic infection...
December 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29383116/overexpression-of-hsd17b4-exerts-tumor-suppressive-function-in-adrenocortical-carcinoma-and-is-not-associated-with-hormone-excess
#11
Guanxiong Ding, Shenghua Liu, Qiang Ding, Chenchen Feng
Aim: Adrenocortical carcinoma (ACC) is characterized with excessive hormone production. We therefore investigated expression of hormone-related genes in ACC. Results: We queried status of 14 key genes directly involved in adrenal hormone production and found HSD17B4 expression was upregulated in 39% of ACC cases on top of all queried genes. Overexpression of HSD17B4 was significantly associate with a normo-hormonal phenotype. Constitutive HSD17B4 expression was higher in ACC cell line NCI-H295R than in adrenocortical small cell carcinoma cell line SW13...
December 29, 2017: Oncotarget
https://www.readbyqxmd.com/read/29377868/characteristics-of-adrenal-masses-in-familial-adenomatous-polyposis
#12
Jonah S Shiroky, Jordan P Lerner-Ellis, Anand Govindarajan, David R Urbach, Karen M Devon
BACKGROUND: Adrenal masses are a known extraintestinal manifestation of familial adenomatous polyposis. However, the literature on this association is largely confined to case reports. OBJECTIVE: This study aimed to determine the characteristics of adrenal masses in familial adenomatous polyposis and their clinical significance, as well as to estimate their prevalence. Mutational analysis was conducted to determine if any potential genotype-phenotype correlations exist...
January 24, 2018: Diseases of the Colon and Rectum
https://www.readbyqxmd.com/read/29371329/development-of-new-preclinical-models-to-advance-adrenocortical-carcinoma-research
#13
Katja Kiseljak-Vassiliades, Yu Zhang, Stacey Bagby, Adwitiya Kar, Nikita Pozdeyev, Mei Xu, Katherine Gowan, Vibha Sharma, Christopher D Raeburn, Maria Albuja-Cruz, Kenneth L Jones, Lauren Fishbein, Rebecca Schweppe, Hilary L Somerset, Todd M Pitts, Stephen Leong, Margaret Wierman
Adrenocortical cancer (ACC) is an orphan malignancy that results in heterogeneous clinical phenotypes and molecular genotypes. There are no curative treatments for this deadly cancer with 35% survival at five years. Our understanding of the underlying pathobiology and our ability to test novel therapeutic targets has been limited due to the lack of preclinical models. Here, we report the establishment of two new ACC cell lines and corresponding patient derived xenograft (PDX) models. CU-ACC1 cell line and PDX were derived from a perinephric metastasis in a patient whose primary tumor secreted aldosterone...
January 25, 2018: Endocrine-related Cancer
https://www.readbyqxmd.com/read/29367375/delayed-presentation-of-a-virilising-pure-testosterone-secreting-adrenocortical-carcinoma-with-coexistent-composite-myelolipoma-and-a-venous-thrombus-extending-to-the-heart
#14
Rakesh M Sharma, Sanjay Sinha, Hari B Kishan, Michelle De Padua
A 40-year-old normotensive woman presented with abnormal facial hair for 4 years and amenorrhoea for 13 years. Hormonal, biochemical and haematological evaluation showed isolated elevation of serum testosterone and free testosterone. Her follicle-stimulating hormone and luteinising hormone were in the premenopausal range. Until recently she had reconciled to early 'menopause' and visited beauty clinics but never sought medical evaluation. Imaging revealed an enhancing left adrenal mass with fat densities and venous thrombus extending through the inferior vena cava to a 7 cm mass in the right atrium...
January 23, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29362311/-three-cases-of-advanced-or-recurrent-adrenocortical-carcinoma-patients-treated-with-etoposide-doxorubicin-and-cisplatin-plus-mitotane
#15
Sayuri Sasaki
Adrenocortical carcinoma is a rare cancer with poor clinical outcomes due to its propensity to transform or relapse. Reported here are 3 cases of adrenocortical carcinoma that were treated between January 2007a nd December 2013. Etoposide, doxorubicin, and cisplatin plus mitotane(EDP plus mitotane)were administered as adjuvant therapy to 1 patient, while the other patients received the therapy upon relapse of their cancers following surgery. One patient experienced PR and another 1 experienced SD during the course of their treatment...
January 2018: Gan to Kagaku Ryoho. Cancer & Chemotherapy
https://www.readbyqxmd.com/read/29345155/minimally-invasive-adrenal-surgery-virtue-or-vice
#16
Jared S Winoker, David T Ahlborn, Olamide O Omidele, Gustavo Fernandez-Ranvier, Ithaar H Derweesh, Reza Mehrazin
Adrenocortical carcinoma (ACC) is a rare malignancy associated with poor prognosis despite available treatments. In patients with localized or locally advanced disease, complete resection with negative margins offers the only potential for cure. Unfortunately, most patients develop local and distant recurrence following initial resection highlighting the importance of meticulous surgical technique in the hands of an experienced surgeon. While minimally invasive surgery (MIS) has supplanted open surgery for small to medium-sized benign adrenal tumors, controversy surrounds the use of MIS for resection of ACC...
January 18, 2018: Future Oncology
https://www.readbyqxmd.com/read/29342266/causes-patterns-and-severity-of-androgen-excess-in-1205-consecutively-recruited-women
#17
Yasir S Elhassan, Jan Idkowiak, Karen Smith, Miriam Asia, Helena Gleeson, Rachel Webster, Wiebke Arlt, Michael W O'Reilly
Context: Androgen excess in women is predominantly due to underlying polycystic ovary syndrome (PCOS). However there is a lack of clarity regarding patterns and severity of androgen excess that should be considered predictive of non-PCOS pathology. Objective: We examined the diagnostic utility of simultaneous measurement of serum dehydroepiandrosterone sulfate (DHEAS), androstenedione (A4) and testosterone (T) to delineate biochemical signatures and cut-offs predictive of non-PCOS disorders in women with androgen excess...
January 12, 2018: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/29332161/the-computed-tomography-adrenal-wash-out-analysis-properly-classifies-cortisol-secreting-adrenocortical-adenomas
#18
Anne-Laure Humbert, Guillaume Lecoanet, Sophie Moog, Fehd Bouderraoui, Laurent Bresler, Jean-Michel Vignaud, Elodie Chevalier, Laurent Brunaud, Marc Klein, Thomas Cuny
PURPOSE: Adrenocortical lesions are characterized through imaging, hormonal and histopathological analysis. Our aim was to compare the radiological features of adrenocortical lesions with their cortisol-secreting status and histopathological Weiss score. METHODS: Seventy five patients operated between 2004 and 2016 in the University Hospital of Nancy for either adrenocortical carcinomas (ACC) or adrenocortical adenomas (ACA) were enrolled in this study. We collected cortisol parameters, Computed Tomography (CT) scans (unenhanced density, wash-out (WO) analysis) and 18F-Fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG PET/CT) datas...
January 13, 2018: Endocrine
https://www.readbyqxmd.com/read/29330226/mitotane-treatment-in-patients-with-metastatic-testicular-leydig-cell-tutor-associated-with-severe-androgen-excess
#19
Vasileios Chortis, Nicholas J Johal, Irina Bancos, Matthew Evans, Kassiani Skordilis, Peter Guest, Michael H Cullen, Emilio Porfiri, Wiebke Arlt
Mitotane (o,p'DDD) is established in the adjuvant and advanced stage treatment of adrenocortical carcinoma and counteracts both tumour growth and tumour-related steroid production. Both the adrenal glands and the gonads are steroidogenically active organs and share a common embryogenic origin. Here we describe the effects of mitotane in two patients with metastatic Leydig cell tumour (LCT) of the testes and associated severe androgen excess (serum testosterone 93 and 88 nmol/l, respectively; male reference range 7-27 nmol/L)...
January 12, 2018: European Journal of Endocrinology
https://www.readbyqxmd.com/read/29319480/the-impact-of-patient-disease-and-treatment-related-factors-on-survival-in-patients-with-adrenocortical-carcinoma
#20
Nahid Punjani, Roderick Clark, Jonathan Izawa, Joseph Chin, Stephen E Pautler, Nicholas Power
INTRODUCTION: Adrenal cortical carcinoma (ACC) is a rare and aggressive endocrine tumour. Most present with advanced disease and have poor prognosis. Optimal treatment includes complete surgical resection. There is limited evidence for the efficacy of chemotherapy and radiation at different stages in this disease. There remain many inconsistencies with respect to diagnosis and workup. There is a lack of uniform guideline recommendations and consensus data. METHODS: We performed a retrospective chart review of all patients at London Health Sciences Centre between 1990 and 2015 using ICD coding...
December 22, 2017: Canadian Urological Association Journal, Journal de L'Association des Urologues du Canada
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