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Adrenocortical carcinoma

Karine Renaudin, Sarra Smati, Matthieu Wargny, Abir Al Ghuzlan, Sébastien Aubert, Emmanuelle Leteurtre, Martine Patey, Mathilde Sibony, Nathalie Sturm, Frédérique Tissier, Laurence Amar, Jérôme Bertherat, Claudine Berthozat, Olivier Chabre, Christine Do Cao, Magalie Haissaguerre, Peggy Pierre, Claire Briet, Delphine Vezzosi, Jean Christophe Lifante, François Pattou, Eric Mirallie, Eric Baudin, Bertrand Cariou, Rossella Libe, Delphine Drui
Oncocytic adrenocortical tumors are a rare subtype of adrenal tumors with challenging diagnosis and histoprognostic assessment. It is usually believed that oncocytic adrenocortical tumors have a more indolent clinical behavior than conventional adrenocortical tumors. As the Weiss score overestimates the malignancy of oncocytic adrenocortical tumors owing to intrinsic parameters, alternative scores have been proposed. The Lin-Weiss-Bisceglia score is currently recommended. We performed a large nationwide multicenter retrospective clinicopathologic study of oncocytic adrenocortical tumors...
June 19, 2018: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
Abdallah Al-Salameh, Camille Baudry, Régis Cohen
Multiple endocrine neoplasia type 1 is a rare genetic syndrome, characterized by the co-occurrence, in the same individual or in related individuals of the same family, of hyperparathyroidism, duodenopancraetic neuroendocrine tumors, pituitary adenomas, adrenocortical tumors, and neuroendocrine tumors (carcinoids) in the thymus, the bronchi, or the stomach. Multiple endocrine neoplastic type 2 is a rare genetic syndrome, characterized by the familial occurrence of medullary thyroid carcinoma either isolated or associated with pheochromocytoma, primary hyperparathyroidism, or typical features (Marfanoid habitus, mucosal neuromas)...
June 13, 2018: La Presse Médicale
Vasileios Chortis
The diagnostic work up of adrenal tumours, often incidentally discovered, has emerged as an ever-increasing diagnostic problem for clinical endocrinologists. No imaging modality has sufficiently high sensitivity and specificity at differentiating benign from malignant adrenal lesions. It has long been observed that adrenocortical carcinomas (ACCs) present an immature pattern of steroidogenesis, dominated by steroid hormone precursors. Modern mass spectrometry-based assays can generate multi-steroid metabolite profiles in urine collections, which can detect differences between ACCs and benign adrenocortical adenomas (ACAs)...
June 14, 2018: Minerva Endocrinologica
Md Shuayb, Arunangshu Das, Mirza Nazim Uddin
Adrenocortical carcinoma (ACC) comprises approximately 0.02% of all malignant tumours, which are a very small fraction of a group of cancers that affect in 0.7 to 2 in 1 000 000 people per year. Recurrence is very common even after complete resection and prognosis is poor. We report a case of a sporadic form of ACC found in a 41-year-old Asian Bangladeshi man. His tumour was surgically excised completely with negative margins and he did not receive any adjuvant therapy. Four years later, adrenal adenoma was developed at his opposite side which was also excised...
June 13, 2018: BMJ Supportive & Palliative Care
Taylor C Brown, Timothy D Murtha, Jill C Rubinstein, Reju Korah, Tobias Carling
BACKGROUND: Altered expression of Solute Carrier Family 12 Member 7 (SLC12A7) is implicated to promote malignant behavior in multiple cancer types through an incompletely understood mechanism. Recent studies have shown recurrent gene amplifications and overexpression of SLC12A7 in adrenocortical carcinoma (ACC). The potential mechanistic effect(s) of SLC12A7 amplifications in portending an aggressive behavior in ACC has not been previously studied and is investigated here using two established ACC cell lines, SW-13 and NCI-H295R...
June 8, 2018: Cell Communication and Signaling: CCS
Annika M A Berends, Michiel N Kerstens, Janne W Bolt, Thera P Links, Esther Korpershoek, Ronald R de Krijger, Annemiek M E Walenkamp, Walter Noordzij, Boudewijn van Etten, Gursah Kats-Ugurlu, Ah Brouwers, A N A van der Horst-Schrivers
BACKGROUND/AIM: Positron Emission Tomography (PET) with 6-[18F]fluor-L-3,4-dihydroxyphenylalanine (18F-FDOPA) has been shown to be a useful imaging tool with a high sensitivity for the visualization of neuroendocrine tumors (NETs). 18F-FDOPA uptake in tumors other than NETs has been suggested previously, but data on this phenomenon are limited. We therefore studied the non-physiological, false-positive uptake of 18F-FDOPA in a large population of patients with a NET or with a high clinical suspicion of harboring a NET...
June 6, 2018: European Journal of Endocrinology
Rajani Maharjan, Samuel Backman, Tobias Åkerström, Per Hellman, Peyman Björklund
The Wnt/β-Catenin signaling pathway is one of the most frequently altered pathways in adrenocortical carcinomas (ACCs). The aim of this study was to investigate the status of Wnt/β-Catenin signaling pathway by analyzing the expression level of β-Catenin and the mutational status of APC, AXIN2, CTNNB1, and ZNRF3 in ACCs. Mutations in APC, CTNNB1, ZNRF3 and homozygous deletions in ZNRF3 were observed in 3.8% (2/52), 11.5% (6/52), 1.9% (1/52) and 17.3% (9/52) of the cohort respectively. Novel interstitial deletions in CTNNB1 spanning intron 1 to exon 3/intron 3 were also found in 7...
June 5, 2018: Scientific Reports
Paula Marincola Smith, Colleen M Kiernan, Thuy B Tran, Lauren M Postlewait, Shishir K Maithel, Jason Prescott, Timothy Pawlik, Tracy S Wang, Jason Glenn, Ioannis Hatzaras, Rivka Shenoy, John Phay, Lawrence A Shirley, Ryan C Fields, Linda Jin, Sharon Weber, Ahmed Salem, Jason Sicklick, Shady Gad, Adam Yopp, John Mansour, Quan-Yang Duh, Natalie Seiser, Konstantinos Votanopoulos, Edward A Levine, George Poultsides, Carmen C Solórzano
BACKGROUND: Adrenocortical carcinoma (ACC) is a rare and aggressive cancer. This report describes factors and outcomes associated with resection of extra-adrenal organs en bloc during index adrenalectomy. METHODS: Patients who underwent ACC resection for non-metastatic disease from 1993 to 2014 at 13 participating institutions of the US-ACC Group were included in the study. Factors associated with en bloc resection were assessed by uni- and multivariate analysis...
June 4, 2018: Annals of Surgical Oncology
Vasileios Chortis, Angela E Taylor, Craig L Doig, Mark D Walsh, Eirini Meimaridou, Carl Jenkinson, Giovanny Rodriguez-Blanco, Cristina L Ronchi, Alisha Jafri, Louise A Metherell, Daniel Hebenstreit, Warwick B Dunn, Wiebke Arlt, Paul A Foster
Adrenocortical Carcinoma (ACC) is an aggressive malignancy with poor response to chemotherapy. Here we evaluated a potential new treatment target for ACC, focusing on the mitochondrial NADPH generator Nicotinamide Nucleotide Transhydrogenase (NNT). NNT has a central role within mitochondrial antioxidant pathways, protecting cells from oxidative stress. Inactivating human NNT mutations result in congenital adrenal insufficiency. We hypothesized NNT silencing in ACC cells will induce toxic levels of oxidative stress...
April 20, 2018: Endocrinology
Jacopo Manso, Raffaele Pezzani
Adrenocortical carcinoma (ACC) is a rare malignancy with poor prognosis. It has been undergone to in-depth clinical and laboratory investigations, with the help of the most important research groups of all over the world. Nonetheless the cure for this kind of neoplasia is not right around the corner, given its complexity and multi-faceted feature, that lead researchers to think at "one person one ACC". Currently total resection is the most concrete option for ACC patients, whenever possible. Although years are still necessary for an effective treatment, indubitably the use of mitotane, among the available pharmacological treatments, in the early 60's revolutionized the approach to this rare disease...
May 28, 2018: Minerva Endocrinologica
Nidhi Gupta, Michael Rivera, Paul Novotny, Vilmarie Rodriguez, Irina Bancos, Aida Lteif
BACKGROUND/AIMS: Adrenocortical carcinoma (ACC) is an aggressive childhood cancer. Limited evidence exists on a definite histopathological criterion to differentiate ACC from adrenocortical adenoma. The aim of this study was to investigate the clinicopathological data of children with ACC, identify prognostic factors, and validate a histopathological criterion to differentiate ACC from adrenocortical adenoma. METHODS: This retrospective cohort included 41 children, followed at the Mayo Clinic from 1950 to 2017 (onset of symptoms ≤21 years)...
May 25, 2018: Hormone Research in Pædiatrics
Katja Kiseljak-Vassiliades, Yu Zhang, Adwitiya Kar, Raud Razzaghi, Mei Xu, Katherine Gowan, Christopher D Raeburn, Maria Albuja-Cruz, Kenneth L Jones, Hilary Somerset, Lauren Fishbein, Stephen Leong, Margaret E Wierman
Adrenocortical carcinoma (ACC) is an aggressive cancer with a five-year survival less than 35%. Mortality remains high due to lack of targeted therapies. Using bioinformatic analyses, we identified Maternal Embryonic Leucine Zipper Kinase (MELK) as 4.1-fold overexpressed in ACC compared to normal adrenal samples. High MELK expression in human tumors correlated with shorter survival and with increased expression of genes involved in cell division and growth. We investigated the functional effects of MELK inhibition using newly developed ACC cell lines with variable MELK expression, CU-ACC1 and CU-ACC2, compared with H295R cells...
May 21, 2018: Endocrinology
Sofia Pereira, Mariana P Monteiro, Isabelle Bourdeau, Andre Lacroix, Duarte Pignatelli
Adrenocortical carcinomas are rather rare endocrine tumors that often have a poor prognosis. The reduced survival rate associated with these tumors is due to their aggressive biological behavior, combined with the scarcity of effective treatment options that are currently available. The recent identification of the genomic alterations present in ACC have provided further molecular mechanisms to develop consistent strategies for the diagnosis, prevention of progression and treatment of advanced ACCs. Taken together, molecular and genomic advances could be leading the way to develop personalized medicine in ACCs similarly to similar developments in lung or breast cancers...
May 17, 2018: European Journal of Endocrinology
Yosuke Sekii, Mitsuhiro Yoshinaga, Masahiro Nakagawa, Hidefumi Kishikawa, Kazumasa Oka, Kenji Nishimura
A 74-year old man presented to us with difficulty urinating. The laboratory examinations revealed an elevated prostate-specific antigen (PSA) level. A prostate biopsy revealed prostate adenocarcinoma. The computed tomography scan showed metastasis to lymph nodes in the pelvic cavity and both adrenal glands. Combined androgen blockade therapy was started, and PSA levels began to decrease gradually. However, 2 months later, he was hospitalized for treatment of cellulitis and was found to have hypertension, hyperglycemia, and hypokalemia...
April 2018: Hinyokika Kiyo. Acta Urologica Japonica
Ying He, Lian Xu, Qingli Li, Min Feng, Wei Wang
RATIONALE: Adrenocortical carcinoma (ACC) is an endocrine malignancy with poor prognosis, which commonly arises in a sporadic manner, but may also become a part of a familial syndrome. ACC rarely arises simultaneously with other malignant tumors. PATIENT CONCERNS: We report a case of a 29-year-old woman with ACC synchronously followed by an ovarian malignant mixed germ cell tumor. We describe the clinical, histopathological, and immunohistochemical findings and review the English literatures...
May 2018: Medicine (Baltimore)
Soraya Puglisi, Paola Perotti, Anna Pia, Giuseppe Reimondo, Massimo Terzolo
Adrenocortical carcinoma (ACC) is a rare and aggressive tumor. ACC may be associated with different syndromes of hormone excess, most frequently Cushing's syndrome with or without hypersecretion of androgens. Recent data suggest that cortisol excess is a negative prognostic factor in advanced and localized ACC. Surgery with radical intent, when feasible, is the most effective treatment for ACC with hypercortisolism. Mitotane is the medical treatment of choice, both postoperatively and in inoperable or metastatic cases...
June 2018: Endocrinology and Metabolism Clinics of North America
Pedram Javanmard, Daisy Duan, Eliza B Geer
Cushing's syndrome is associated with increased morbidity and mortality. Cardiovascular events, sepsis, and thromboembolism are the leading causes of mortality. Patient's with Cushing's due to a pituitary adenoma and those with Cushing's due to benign adrenal adenoma have relatively good survival outcomes often mirroring that of the general population. Persistent or recurrent disease is associated with high mortality risk. Ectopic Cushing's syndrome and Cushing's due to adrenocortical carcinoma confer the highest mortality risk among Cushing's etiologies...
June 2018: Endocrinology and Metabolism Clinics of North America
Virginia Bellido, Ihintza Larrañaga, Federico Vazquez, Verónica Velasco, Sonia Gaztambide
No abstract text is available yet for this article.
May 8, 2018: Endocrinología, Diabetes y Nutrición
Seong Hee Ahn, Jae Hyeon Kim, Seung Hee Baek, Hyeonmok Kim, Yoon Young Cho, Sunghwan Suh, Beom Jun Kim, Seongbin Hong, Jung Min Koh, Seung Hun Lee, Kee Ho Song
PURPOSE: Previous studies on adrenal incidentalomas (AIs) are limited by their retrospective design, small numbers of patients, Western populations, or use of an outdated imaging technique. We investigated the characteristics of AIs in Korean patients and compared them with those reported in the largest retrospective study in Italy to discover the effects of improved imaging techniques and ethnicity differences. MATERIALS AND METHODS: This was a prospective, multicenter, cross-sectional observational study including 1005 Korean patients...
June 2018: Yonsei Medical Journal
Daniel W Nelson, Shu-Ching Chang, Brad C Bandera, Trevan D Fischer, Robert Wollman, Melanie Goldfarb
BACKGROUND: Adrenocortical carcinoma (ACC) is a rare and aggressive malignancy for which surgery is the mainstay of treatment and for which adjuvant radiation is infrequently employed; however, small, single-institution series suggest adjuvant radiation may improve outcomes. METHODS: All patients with non-metastatic ACC treated with either surgery alone or surgery followed by adjuvant radiation were identified in the 2004-2013 National Cancer Database. Factors associated with receipt of radiation and the impact of adjuvant radiation on survival were determined by multivariable analysis...
May 10, 2018: Annals of Surgical Oncology
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