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Adrenocortical carcinoma

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https://www.readbyqxmd.com/read/28646944/oncocytic-adrenocortical-carcinoma-a-rare-adrenal-tumor-subtype
#1
Emma Sumner, Behram Cenk Acar, Matthew R Acker
Oncocytic tumors arising from the adrenal gland are rare. Oncocytic adrenal neoplasms (OAN) may mimic adrenocortical carcinoma (ACC) at presentation, and can only be definitively diagnosed histologically. Most OANs are benign, and carry a favorable prognosis. We report on an 83-year-old female who, while being investigated for anemia and weight loss, was found to have a 23 cm adrenal mass concerning for ACC. Adrenalectomy and histopathology confirmed a malignant OAN, based on the Lin-Weiss-Bisceglia criteria...
June 2017: Canadian Journal of Urology
https://www.readbyqxmd.com/read/28643981/-adrenal-surgery-multidisciplinarity-is-mandatory
#2
Marie-Laure Matthey Gié, Marie Nicod Lalonde, Elena Gonzalez Rodriguez, Nicolas Demartines, Maurice Matter
Adrenal tumours are rare and their management is challenging. Every patient presenting with adrenal mass or symptoms of hormones hypersecretion should be investigated. The two important questions to be answered are : 1. Is the tumour secreting ? Is the tumour malignant or not ? A complete endocrine work-up and a nativ CT-scan may usually answer these two questions but have to be interpreted by specialists in a multidisciplinary team setting. The decisions about managements of adrenal pathologies do follow international guidelines which are regularly updated...
June 14, 2017: Revue Médicale Suisse
https://www.readbyqxmd.com/read/28641336/suppression-of-forkhead-box-protein-o1-foxo1-transcription-factor-may-promote-adrenocortical-tumorigenesis
#3
Adam Stenman, Timothy Murtha, Reju Korah, Tobias Carling
Despite recent comprehensive genetic analyses, molecular evidence for a pathophysiological continuum linking benign adrenocortical adenoma (ACA) and highly aggressive adrenocortical carcinoma (ACC) is still elusive. Using human tumor samples and the established ACC cell line SW-13, this study investigated potential regulatory roles for FOXO transcription factors, in modulating adrenocortical tumorigenesis. Adrenocortical tumor specimens (20 ACAs, 10 ACCs, and 9 normal adrenal tissue samples) obtained from 30 patients were analyzed for ubiquitously expressed FOXO transcription factors, FOXO1 and FOXO3 using qRT-PCR and immunohistochemistry...
June 22, 2017: Hormone and Metabolic Research, Hormon- und Stoffwechselforschung, Hormones et Métabolisme
https://www.readbyqxmd.com/read/28614035/personalized-care-of-patients-with-adrenocortical-carcinoma-a-comprehensive-approach
#4
Barbra S Miller, Tobias Else
A concerted effort has been made in the past decade to better differentiate benign from malignant adrenocortical tumors. Of those tumors found to be adrenocortical carcinomas (ACCs) and through the use of multiple modalities including biochemical, radiologic, and genomic analysis, significant strides have been made in understanding what drives ACC development, how various treatments may result in different outcomes, which ACCs are more likely to respond to various treatments, and overall prognosis. While most patients will have recurrence of their ACC and succumb to their disease, the disease course is highly variable; it is therefore imperative that each patient is treated with individualized attention paid to their particular ACC...
June 2017: Endocrine Practice
https://www.readbyqxmd.com/read/28601306/disparities-in-access-to-care-and-outcomes-in-patients-with-adrenocortical-carcinoma
#5
Abdulrahman Y Hammad, Tina W F Yen, Azadeh A Carr, Douglas B Evans, Tracy S Wang
BACKGROUND: Surgical resection remains the mainstay of treatment for patients with adrenocortical carcinoma (ACC). The aim of the present study is to examine disparities in access to surgical resection and identify factors associated with overall survival following surgical resection. METHODS: The National Cancer Database was queried for patients with ACC (2004-2013). Patient characteristics and disease details were abstracted. Logistic regression analysis was performed to examine the factors associated with surgical resection, and a multivariate Cox proportional hazards model was used to identify predictors of survival in the surgical cohort...
June 1, 2017: Journal of Surgical Research
https://www.readbyqxmd.com/read/28583923/triple-synchronous-primary-malignancies-a-rare-occurrence
#6
Heather Katz, Hassaan Jafri, Linda Brown, Toni Pacioles
Triple synchronous primary malignant neoplasms are rare. The exact aetiology is unknown; however, risk factors include older age, family history, genetic aberrations, prolonged exposure to carcinogens and smoking. We describe a previously healthy 48-year-old woman who presented with abdominal pain and a palpable abdominal mass. Imaging revealed a complex cystic, solid pelvic mass and another mass in the right upper quadrant. She received an extensive abdominal surgery including exploratory laparotomy, pelvic mass resection, total abdominal hysterectomy, bilateral salpingo-oophorectomy, bilateral pelvic lymphadenectomy, omentectomy and right adrenalectomy...
June 5, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28559412/complete-responses-to-mitotane-in-metastatic-adrenocortical-carcinoma-a-new-look-at-an-old-drug
#7
Diane L Reidy-Lagunes, Betty Lung, Brian R Untch, Nitya Raj, Anastasia Hrabovsky, Ciara Kelly, Scott Gerst, Seth Katz, Lewis Kampel, Joanne Chou, Anu Gopalan, Leonard B Saltz
PURPOSE: Based largely on reports that predate modern reporting standards, mitotane has been considered a systemic treatment option for both hormone control and antitumor control of metastatic adrenocortical cancer (ACC), although the therapeutic window is narrow. METHODS: We searched electronic medical records to identify patients with metastatic ACC treated and prescribed single agent mitotane at Memorial Sloan Kettering Cancer Center from March 15, 1989-September 18, 2015...
May 30, 2017: Oncologist
https://www.readbyqxmd.com/read/28551383/a-exceptional-collision-tumor-of-primary-adrenal-angiosarcoma-and-non-functioning-adrenocortical-adenoma
#8
Katsumi Takizawa, Kenichi Kohashi, Takahito Negishi, Kenichi Taguchi, Yuichi Yamada, Motonobu Nakamura, Yoshinao Oda
Primary adrenal angiosarcoma is an extremely rare vascular tumor. We report a case of a 63-year-old man with a collision tumor of epithelioid angiosarcoma and adrenocortical adenoma of the right adrenal gland. The adrenal tumor was incidentally observed by a preoperative computed tomography (CT) scan of penis squamous cell carcinoma. The patient underwent a right laparoscopic adrenalectomy, and the tumor size measured 34×34×15mm. Histological examination revealed two different tumor cell proliferations, namely epithelioid angiosarcoma and adrenocortical adenoma...
June 2017: Pathology, Research and Practice
https://www.readbyqxmd.com/read/28550366/minimally-invasive-resection-of-adrenal-masses-in-infants-and-children-results-of-a-european-multi-center-survey
#9
Francesco Fascetti-Leon, Giovanni Scotton, Luca Pio, Raimundo Beltrà, Paolo Caione, Ciro Esposito, Girolamo Mattioli, Amulya K Saxena, Sabine Sarnaki, Piergiorgio Gamba
BACKGROUND: Minimal access adrenal surgery (MAAS) for adrenal pathologies is the standard for many pediatric surgical centers. However, the literature offers few reports and minimal evidence from small case series. The aim of this study was to evaluate the outcomes of pediatric MAAS through a multi-center data analysis. METHOD: Pediatric patients who underwent MAAS between January 2002 and December 2013 were retrospectively included. Data analysis was conducted using Spss software (Welch's t-test, X-square, Fisher tests, multiple regression model)...
May 26, 2017: Surgical Endoscopy
https://www.readbyqxmd.com/read/28546721/a-rare-presentation-of-infantile-virilization-secondary-to-malignant-etiology
#10
V Dasarwar Nagesh, D Ramkrishnan Santosh, N Datla Sravya
Adrenocortical tumor is a rare malignancy (1-2/million) in children with a heterogenous presentation and generally poor prognosis. We report two cases of adrenocortical carcinoma who presented primarily with virilization along with cushingoid features and hypertension. Both children were managed with surgical resection of tumor, steroid replacement, and antihypertensives.
June 2017: Indian Journal of Surgical Oncology
https://www.readbyqxmd.com/read/28540670/open-adrenalectomy-in-the-era-of-laparoscopic-surgery-a-review
#11
REVIEW
Giovanni Taffurelli, Claudio Ricci, Riccardo Casadei, Saverio Selva, Francesco Minni
Open adrenalectomy still plays an important role in adrenal surgery. A review of the current literature has been carried out to discuss the main indication to open adrenalectomy with regards to the main adrenal pathologies. The authors underlined the role of open adrenalectomy on the basis of personal experience and a literature review. Indication to open adrenalectomy for adrenal cysts, myelolipomas, pheochromocytomas, metastases, adrenocortical carcinomas and tumour recurrences were analysed and discussed...
May 24, 2017: Updates in Surgery
https://www.readbyqxmd.com/read/28533648/increased-f-fdg-uptake-in-bilateral-gynecomastia-secondary-to-feminizing-adrenal-tumor-a-rare-case-report-and-review-of-literature
#12
Priyanka Verma, Gaurav Malhotra, Sneha Kothari, Rajlaxmi Jagtap, Ramesh V Asopa
A 21 year old male who presented with painful enlargement of both the breasts and a hyperestrogenic state, was found to harbor a heterogeneous mass arising from the right adrenal on contrast enhanced Computed Tomography abdomen. The mass was hypermetabolic with no regional, nodal or distant metastases on Fluorine-18 Fluorodeoxyglucose Positron Emission Tomography /Computed Tomography examination. Notably, substantial tracer uptake was seen in bilateral gynecomastia. The patient underwent a right adrenalectomy with the histopathology report confirming adrenocortical carcinoma...
April 2017: Indian Journal of Nuclear Medicine: IJNM: the Official Journal of the Society of Nuclear Medicine, India
https://www.readbyqxmd.com/read/28523067/extension-of-adrenocortical-carcinoma-into-the-right-atrium
#13
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
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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