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Adrenocortical carcinoma

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https://www.readbyqxmd.com/read/28523067/extension-of-adrenocortical-carcinoma-into-the-right-atrium
#1
Yulanka Castro-Dominguez, Fatima Samad, Hayder Hashim, Alfonso Waller
Adrenocortical carcinoma (ACC) is a rare and highly aggressive malignant neoplasm which can produce intravascular extension into the inferior vena cava (IVC) rarely extend into the right atrium (RA). We report a case of a male patient with large ACC with extension into the IVC and RA. Computed tomography showed a large right adrenal mass with contiguous tumor thrombus extending into IVC and RA with extension to the level of tricuspid valve. Patient underwent combined cardiac and abdominal surgical intervention on cardiopulmonary bypass with removal of the mass...
March 2017: Pakistan Journal of Medical Sciences Quarterly
https://www.readbyqxmd.com/read/28501574/androgen-production-in-pediatric-adrenocortical-tumors-may-occur-via-both-the-classic-and-or-the-alternative-backdoor-pathway
#2
Nesa Marti, Jana Malikova, José A Galván, Maude Aebischer, Marco Janner, Zdenek Sumnik, Barbora Obermannova, Genevieve Escher, Aurel Perren, Christa E Flück
Children with adrenocortical tumors (ACTs) often present with virilization due to high tumoral androgen production, with dihydrotestosterone (DHT) as most potent androgen. Recent work revealed two pathways for DHT biosynthesis, the classic and the backdoor pathway. Usage of alternate routes for DHT production has been reported in castration-resistant prostate cancer, CAH and PCOS. To assess whether the backdoor pathway may contribute to the virilization of pediatric ACTs, we investigated seven children suffering from androgen producing tumors using steroid profiling and immunohistochemical expression studies...
May 10, 2017: Molecular and Cellular Endocrinology
https://www.readbyqxmd.com/read/28499267/a-novel-germline-tp53-mutation-p-pro190arg-detected-in-a-patient-with-lung-and-bilateral-breast-cancers
#3
Małgorzata Krześniak, Dorota Butkiewicz, Jadwiga Rachtan, Iwona Matuszczyk, Ewa Grzybowska, Marek Rusin
PURPOSE: Li-Fraumeni syndrome (LFS) is a rare genetic disease with strong predispositions to multiple early-onset neoplasms, mostly sarcomas, breast cancers, brain tumors and adrenocortical carcinomas (LFS core cancers). In most LFS families the germline mutations of TP53 tumor suppressor gene were found. Lung cancer does not belong to the core cancers of LFS, however its higher incidence is observed in families with TP53 mutations. Our aim was to search for TP53 mutations in female lung cancer patients whose clinico-demographic characteristics suggested a probable genetic predisposition to the disease...
May 9, 2017: Advances in Medical Sciences
https://www.readbyqxmd.com/read/28497219/robot-assisted-adrenalectomy-indications-and-drawbacks
#4
C Nomine-Criqui, A Germain, A Ayav, L Bresler, L Brunaud
Adrenal tumors can vary from a benign adrenocortical adenoma with no hormonal secretion to a secretory adrenocortical malignancy (adrenocortical carcinoma) or a hormone-secreting tumor of the adrenal medulla (pheochromocytoma). Currently, laparoscopic adrenalectomy is regarded as the preferred surgical approach for the management of most adrenal surgical disorders, although there are no prospective randomized trials comparing this technique with open adrenalectomy. However, widespread adoption of robotic technology has positioned robotic adrenalectomy as an option in some medical centers...
May 12, 2017: Updates in Surgery
https://www.readbyqxmd.com/read/28458841/adrenocortical-carcinoma-co-occurrence-with-a-hepatocellular-carcinoma-within-an-adrenohepatic-fusion-a-case-report
#5
Faisal A Alfaqeeh, Ghadeer Algehani, Mazin Hassanain, Abdulsalam Alsharaabi, Mohammad Alsohaibani
Adrenohepatic fusion is a relatively common condition that has been reported in the literature and explained as a result of normal aging. However, the presence of a neoplastic process in the adrenohepatic fusion is relatively uncommon. A 48-year-old male presented with RUQ abdominal pain with findings of a huge liver mass with adrenohepatic fusion in the computed tomography scan. Histopathological studies revealed a mixed, although distinctly separated, adrenocortical carcinoma and a hepatocellular carcinoma within an adrenoheaptic fusion...
March 2017: Journal of Surgical Case Reports
https://www.readbyqxmd.com/read/28456766/understanding-mitotane-mode-of-action
#6
U Waszut, P Szyszka, D Dworakowska
Adrenocortical carcinoma is a rare disease with poor prognosis. Mitotane is the most effective agent in post-operative treatment (or when inoperable). It selectively limits growth and bioactivity of adrenal tissue. Despite 60 years of use, the basis for its action has yet to be convincingly established. This review summarizes current knowledge of mitotane effects, based on studies on adrenal tissue and primary cell cultures, with emphasis on more recent studies of cell lines. We consider features of the adrenal cortex that might explain mitotane selectivity, and review effects on non-adrenal cells...
February 2017: Journal of Physiology and Pharmacology: An Official Journal of the Polish Physiological Society
https://www.readbyqxmd.com/read/28450646/effect-of-mitotane-on-mouse-ovarian-follicle-development-and-fertility
#7
Federica Innocenti, Lidia Cerquetti, Serena Pezzilli, Barbara Bucci, Vincenzo Toscano, Rita Canipari, Antonio Stigliano
Mitotane (MTT) is an adrenolytic drug used in advanced and adjuvant treatment of adrenocortical carcinoma, in Cushing's disease and in ectopic syndrome. However, knowledge about its effects on the ovary is still scarce. The purpose of this study is to investigate the effect of MTT on the ovary using in vivo and in vitro models. The study was performed in CD1 mice and in the COV-434 human ovarian granulosa cell line. We examined ovarian morphology, follicle development, steroidogenesis and procreative function in mice and the effect of MTT on cell growth in vitro...
April 27, 2017: Journal of Endocrinology
https://www.readbyqxmd.com/read/28446715/establishment-of-a-mouse-xenograft-model-of-metastatic-adrenocortical-carcinoma
#8
Aurélie Morin, Carmen Ruggiero, Estelle Robidel, Mabrouka Doghman-Bouguerra, Atze T Das, Rémy Castellano, Emmanuelle Josselin, Judith Favier, Enzo Lalli
Adrenocortical carcinoma is a rare neoplasm with a poor prognosis. Very important advances have been made in the identification of the genetic determinants of adrenocortical carcinoma pathogenesis but our understanding is still limited about the mechanisms that determine cancer spread and metastasis. One major problem hindering preclinical experimentation for new therapies for adrenocortical carcinoma is represented by the lack of suitable animal models for metastatic disease. With the aim to overcome these limitations, in this study we tested several protocols in order to establish a mouse xenograft model of metastatic adrenocortical carcinoma...
April 7, 2017: Oncotarget
https://www.readbyqxmd.com/read/28445297/the-efficacy-of-radiation-therapy-in-adrenocortical-carcinoma-a-propensity-score-analysis-of-a-population-based-study
#9
You Luo, San-San Chen, Xiang-Guang Zheng, Li Luo, Sen Wang
Adrenocortical carcinoma (ACC) is a rare and malignant tumor. The main treatment is primary surgical resection with or without mitotane therapy. The role of radiation therapy is still controversial. We aim to investigate the survival efficacy of radiotherapy in a large population-based cohort.We queried the Surveillance, Epidemiology, and End Results (SEER) database (1973-2013) to identify cases with ACC. Traditional multivariate Cox regression and propensity score analysis were used to evaluate the effect of radiotherapy on cancer survival...
April 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28432798/therapeutic-drug-monitoring-of-mitotane-analytical-assay-and-patient-follow-up
#10
Yoann Cazaubon, Helene Guillemin, Damien Vautier, Olivier Oget, Hervé Millart, Claire Gozalo, Zoubir Djerada, Catherine Feliu
BACKGROUND: Adrenocortical carcinoma (ACC) is an aggressive malignancy of the adrenal gland. Mitotane (o,p'-DDD) is the most effective chemotherapy for ACC. According to literature, mitotane plasma trough concentrations within 14-20 mg.L(1) are correlated with a higher response rate with acceptable toxicity. Therapeutic drug monitoring (TDM) of mitotane is therefore recommended. The aim of this study was to propose a robust, and simple method for mitotane quantification in plasma. The validation procedures were based on international guidelines...
April 22, 2017: Biomedical Chromatography: BMC
https://www.readbyqxmd.com/read/28432084/topoisomerase2%C3%AE-and-thymidylate-synthase-expression-in-adrenocortical-cancer
#11
Elisa Roca, Alfredo Berruti, Silviu Sbiera, Ida Rapa, Ester Oneda, Paola Sperone, Cristina L Ronchi, Laura Ferrari, Salvatore Grisanti, Antonina Germano, Barbara Zaggia, Giorgio Scagliotti, Martin Fassnacht, Marco Volante, Massimo Terzolo, Mauro Papotti
Topoisomerase II alpha (TOP2A) and Thymidylate Synthase (TS) are known prognostic parameters in several tumors and also predictors of efficacy of anthracyclines, topoisomerase inhibitors and fluoropirimidines, respectively. Expression of TOP2A and TS mRNA was assessed in 98 patients with adrenocortical carcinoma (ACC) and protein expression was assessed by immunohistochemistry in a subset of 39 tumors. Ninety-two patients were radically resected for stage II-III disease and 38 of them received adjuvant mitotane...
April 21, 2017: Endocrine-related Cancer
https://www.readbyqxmd.com/read/28431167/performance-of-18f-fdg-pet-ct-in-the-characterization-of-adrenal-masses-in-non-cancer-patients-a-prospective-study
#12
Carole Guerin, François Pattou, Laurent Brunaud, Jean-Christophe Lifante, Eric Mirallié, Magalie Haissaguerre, Damien Huglo, Pierre Olivier, Claire Houzard, Catherine Ansquer, Elif Hindié, Anderson Loundou, Cendrine Archange, Antoine Tabarin, Fréderic Sebag, Karine Baumstarck, David Taïeb
Context: Few prospective studies have evaluated the role of 18F-Fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG PET/CT) in the characterization of adrenal masses. Objective: To assess the performance of 18F-FDG PET/CT in the malignancy diagnosis of adrenal masses in non-cancer patients. Design: Prospective multicenter study. Material and methods: The study population consisted of 87 patients (87 adrenal masses) referred to endocrine surgeons: 56 with mass diameter ≥40 mm and 31 with a diameter <40 mm and of indeterminate nature based on unenhanced and washout CT attenuation densities...
April 20, 2017: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/28423559/rottlerin-as-a-novel-chemotherapy-agent-for-adrenocortical-carcinoma
#13
Yi Zhu, Minjie Wang, Xu Zhao, Lei Zhang, Yigao Wu, Bangqi Wang, Weilie Hu
Adrenocortical carcinoma (ACC) is a rare, but aggressive endocrine malignancy with a generally poor clinical outcome. There is no effective therapy for advanced and metastatic ACC. In our study, we found that an existing drug (rottlerin) exerted its tumour-suppressive function in ACC. Specifically, rottlerin inhibited cellular proliferation of ACC cell lines (NCI-H295R and SW-13) in a dose- and time-dependent manner. We also found that rottlerin induced cell apoptosis and promoted G0/G1 cell cycle arrest in ACC cell lines...
April 4, 2017: Oncotarget
https://www.readbyqxmd.com/read/28423361/next-generation-sequencing-reveals-microrna-markers-of-adrenocortical-tumors-malignancy
#14
Łukasz Koperski, Marta Kotlarek, Michał Świerniak, Monika Kolanowska, Anna Kubiak, Barbara Górnicka, Krystian Jażdżewski, Anna Wójcicka
BACKGROUND: Adrenocortical carcinoma is a rare finding among common adrenocortical tumors, but it is highly aggressive and requires early detection and treatment. Still, the differential diagnosis between benign and malignant lesions is difficult even for experienced pathologists and there is a significant need for novel diagnostic methods. In this study we aimed to reveal a complete set of microRNAs expressed in the adrenal gland and to identify easily detectable, stable and objective biomarkers of adrenocortical malignancy...
April 3, 2017: Oncotarget
https://www.readbyqxmd.com/read/28421464/a-novel-tp53-mutation-associated-with-twist1-and-sip1-expression-in-an-aggressive-adrenocortical-carcinoma
#15
Daniel Bulzico, Davi Coe Torres, Gerson Moura Ferreira, Bruno Ricardo Barreto Pires, Paulo Antônio Silvestre de Faria, Rocio Hassan, Eliana Abdelhay, Mario Vaisman, Leonardo Vieira Neto
Adrenocortical carcinomas (ACC) are very rare tumors related to TP53 mutations mostly in childhood onset cases. Epithelial-mesenchymal transition (EMT) transcription factors TWIST1 and Smad interacting protein 1 (SIP1) are related to poorer outcomes in other malignancies, but their role in ACC is unknown. We describe a case of an advanced metastatic ACC (Weiss-score of 9) in a patient at age 76. After primary tumor resection, mitotane therapy was started as palliation to low-volume liver metastasis. After a 2-year period of stable disease, the patient died due to brain metastasis...
April 18, 2017: Endocrine Pathology
https://www.readbyqxmd.com/read/28410270/the-aurora-kinase-inhibitor-amg-900-increases-apoptosis-and-induces-chemosensitivity-to-anticancer-drugs-in-the-nci-h295-adrenocortical-carcinoma-cell-line
#16
Kleiton S Borges, Augusto F Andrade, Vanessa S Silveira, David S Marco Antonio, Elton J R Vasconcelos, Sonir R R Antonini, Luiz G Tone, Carlos A Scrideli
Adrenocortical tumor (ACT) is a malignancy with a low incidence rate and the current therapy for advanced disease has a limited impact on overall patient survival. A previous study from our group suggested that elevated expression of aurora-A and aurora-B is associated with poor outcome in childhood ACT. Similar results were also reported for adult ACTs. The present in-vitro study shows that AMG 900 inhibits aurora kinases in adrenocortical carcinoma cells. AMG 900 inhibited cell proliferation in NCI-H295 cells as well as in the ACT primary cultures and caused apoptosis in the cell line NCI-H295...
July 2017: Anti-cancer Drugs
https://www.readbyqxmd.com/read/28378549/myxoid-and-sarcomatoid-variants-of-adrenocortical-carcinoma-analysis-of-rare-variants-in-single-tertiary-care-center
#17
Tae Yon Sung, Yun Mi Choi, Won Gu Kim, Yu Mi Lee, Tae Yong Kim, Young Kee Shong, Won Bae Kim, Dong Eun Song
The aim of this study is to describe rare variants of adrenocortical carcinoma (ACC) and to compare the prognosis with that of conventional ACC. We retrospectively reviewed 8 cases of myxoid variant, 1 sarcomatoid variant, and 14 cases of conventional ACC, who underwent surgical resection at the Asan Medical Center between 1996 and 2014. An analysis of the clinicopathological characteristics, including the Weiss score, Ki-67 labeling index, and reticulin framework assessment is presented. The mean age of patients with myxoid/sarcomatoid ACC was 45 years; 4 out of 9 patients were women...
May 2017: Journal of Korean Medical Science
https://www.readbyqxmd.com/read/28368424/p53-rb-inhibition-induces-metastatic-adrenocortical-carcinomas-in-a-preclinical-transgenic-model
#18
M Batisse-Lignier, I Sahut-Barnola, F Tissier, T Dumontet, M Mathieu, C Drelon, J-C Pointud, C Damon-Soubeyrand, G Marceau, J-L Kemeny, J Bertherat, I Tauveron, P Val, A Martinez, A-M Lefrançois-Martinez
Adrenocortical carcinoma (ACC) is a rare cancer with poor prognosis. Pan-genomic analyses identified p53/Rb and WNT/β-catenin signaling pathways as main contributors to the disease. However, isolated β-catenin constitutive activation failed to induce malignant progression in mouse adrenocortical tumors. Therefore, there still was a need for a relevant animal model to study ACC pathogenesis and to test new therapeutic approaches. Here, we have developed a transgenic mice model with adrenocortical specific expression of SV40 large T-antigen (AdTAg mice), to test the oncogenic potential of p53/Rb inhibition in the adrenal gland...
April 3, 2017: Oncogene
https://www.readbyqxmd.com/read/28356253/adrenocortical-carcinoma-masquerading-as-cushing-s-disease
#19
Kush Dev Jarial, Rama Walia, Santosh Kumar, Anil Bhansali
Cushing's syndrome (CS) can be classified as adrenocorticotropic hormone (ACTH)-dependent or ACTH-independent depending on the ACTH levels. However, 30% of the patients with CS have ACTH levels in the 'grey zone' (5-20 pg/mL), thereby posing a challenge in establishing the aetiological diagnosis. In a patient with full-blown features of Cushing's syndrome with equivocal ACTH levels, and a pituitary microadenoma on contrast-enhanced MRI sella, can falsely lead to a diagnosis of Cushing's disease. Pituitary microadenoma, if <6 mm in size, may be an incidental finding (incidentaloma) in this scenario and can be present in ∼3-27% of the healthy population...
March 29, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28348073/from-benign-adrenal-incidentaloma-to-adrenocortical-carcinoma-an-exceptional-random-event
#20
Ines Belmihoub, Stephane Silvera, Mathilde Sibony, Bertrand Dousset, Paul Legmann, Xavier Bertagna, Jerome Bertherat, Guillaume Assie
New European guidelines for the management of adrenal incidentalomas were recently released. One of the most novel recommendations is to stop following patients when they present a typical, small and non-secreting adenoma. We report here the case of a 71-year-old man with such an adenoma, who developed an adrenocortical carcinoma (ACC) fourteen years later, with subsequent metastases and death. Clinically he had a normal blood pressure and no sign of hormonal hypersecretion. The hormonal work-up showed no hormone excess: urinary free cortisol level was normal, the diurnal cortisol rhythm was respected and urinary catecholamine metabolites levels were normal...
March 27, 2017: European Journal of Endocrinology
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