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Adrenocortical carcinoma

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https://www.readbyqxmd.com/read/28087475/immunohistochemical-approach-for-the-diagnosis-of-a-liver-mass-on-small-biopsy-specimens
#1
Won-Tak Choi, Rageshree Ramachandran, Sanjay Kakar
Well-differentiated hepatocellular carcinoma (HCC) shares overlapping histological features with benign hepatocellular lesions, including hepatocellular adenoma (HCA) and focal nodular hyperplasia (FNH) in non-cirrhotic liver, and with high-grade dysplastic nodule (HGDN) in cirrhotic liver. Several metastatic tumors, such as neuroendocrine tumor, renal cell carcinoma, adrenocortical carcinoma, melanoma, and epithelioid angiomyolipoma, can be indistinguishable from HCC on histologic grounds. Since this distinction has important therapeutic implications, judicious use of immunohistochemical markers plays an important role in establishing an accurate diagnosis, especially when limited material of tumor is available on cell block or a small core biopsy...
January 10, 2017: Human Pathology
https://www.readbyqxmd.com/read/28079787/oral-and-vulvo-vaginal-lichenoid-reactions-due-to-mitotane-lysodren-a-case-report
#2
Arthur Schmouchkovitch, Héloïse Herry, Philippe Thuillier, Véronique Kerlan, Camille Fleuret, Guy Le Toux, Sylvie Boisramé
BACKGROUND: The purpose of pharmacovigilance (drug safety) is collection, detection, assessment, monitoring, and prevention of adverse effects with pharmaceutical products. It is meant to identify, characterize, prevent, or minimize actual or potential risks relating to medicinal products. To prevent these adverse effects and improve our practice, health professionals have a duty to report side effects to assess this risk and evaluate the benefit/risk requirements. Mitotane (Lysodren) is used for treating adrenocortical carcinoma...
January 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28073588/overexpression-of-cytochrome-p450-2a6-in-adrenocortical-carcinoma
#3
Timothy D Murtha, Taylor C Brown, Jill C Rubinstein, Felix Haglund, C Christofer Juhlin, Catharina Larsson, Reju Korah, Tobias Carling
BACKGROUND: Cytochrome P450-mediated metabolism of chemotherapeutic agents contributes to chemotherapy resistance in multiple malignancies. Adrenocortical carcinoma is known to have a poor response to adjuvant therapies; however, the mechanism remains unknown. Recent comprehensive genetic analyses of adrenocortical carcinomas demonstrated recurrent copy number gains in multiple cytochrome P450 genes prompting investigation into whether cytochrome P450 overexpression potentiates adrenocortical carcinoma chemoresistance...
January 7, 2017: Surgery
https://www.readbyqxmd.com/read/28071637/adrenocortical-carcinoma-in-children-and-adults-two-decades-experience-in-a-single-institution
#4
M Sabaretnam, A Mishra, G Agarwal, A Agarwal, A K Verma, S K Mishra
CONTEXT: Adrenocortical carcinoma (ACC) occurring in children and adults show distinct characteristics. However, due to rarity of the disease no large series addressing this issue has been published. AIMS: The aim of this study was to study clinico-pathologic profile and outcome of ACC in children and adults. SETTINGS AND DESIGN: Tertiary referral center. Retrospective study (January 1990-June 2011). SUBJECTS AND METHODS: Forty-five patients with ACC were included; 16children (aged < 18 years) and 29 adults...
April 2016: Indian Journal of Cancer
https://www.readbyqxmd.com/read/28070481/lung-adenocarcinoma-and-adrenocortical-carcinoma-in-a-patient-with-multiple-endocrine-neoplasia-type-1
#5
Nobumasa Ohara, Masanori Kaneko, Masahiro Ikeda, Fumio Ishizaki, Kazuya Suzuki, Ryo Maruyama, Takeshi Komeyama, Kazuhiro Sato, Kenichi Togashi, Hiroyuki Usuda, Yuto Yamazaki, Hironobu Sasano, Kenzo Kaneko, Kyuzi Kamoi
Multiple endocrine neoplasia type 1 (MEN1) is an autosomal dominant disorder caused by heterozygous germline mutations in the tumor suppressor gene MEN1, which encodes a nuclear protein, menin. MEN1 is characterized by the combined occurrence of tumors involving the pituitary gland, pancreatic islets, and parathyroid glands. Additionally, patients with MEN1 often exhibit adrenal tumors. Although most MEN1-associated tumors are benign, malignant lesions arising in these endocrine organs have been reported. Additionally, malignant diseases of non-endocrine organs concomitant with MEN1 have also been reported...
2017: Respiratory Medicine Case Reports
https://www.readbyqxmd.com/read/28060131/nonfunctioning-adrenocortical-carcinoma-in-pediatric-acute-lymphoblastic-leukemia-a-case-report-of-a-rare-multiple-primaries-combination
#6
Wafaa M Rashed, Wael Zekri, Madiha Awad, Hala Taha, Badr Abdalla, Ahmad S Alfaar
Childhood adrenocortical carcinoma (ACC) is a rare tumor and its association with acute lymphoblastic leukemia (ALL) is even rarer. One such case is discussed in this case report. A 3-year-old patient was concomitantly diagnosed with ALL and an initially nonmetastatic ACC. Management started by following the Total XV protocol without a window phase. Left adrenalectomy was conducted after the consolidation phase. Recurrence of a mass at the tumor bed was discovered at week 33 of the continuation phase. Reexcision was conducted, followed by the administration of an ACC protocol including cisplatin, etoposide, and doxirubicin...
January 5, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28031665/splenorenal-arterial-bypass-description-of-technique-and-case-example-in-an-instance-of-renal-revascularization-during-adrenalectomy-for-adrenocortical-carcinoma
#7
J Yozawitz, M Kissin, M Szuchmacher, J Sullivan, J Nicastro, G Coppa, E Molmenti
We present a patient with a 16 cm adrenocortical carcinoma that underwent a left adrenalectomy en bloc with resection of the involved segment of the left renal artery. A splenectomy and splenorenal bypass was performed to revascularize the left kidney. To our knowledge, this is the first instance in the literature of a splenorenal arterial bypass being reported for renal revascularization during an extirpative oncologic procedure. A 64-year-old male patient, with history significant for adrenocortical carcinoma, status post prior right adrenalectomy with partial right nephrectomy, presented for an elective left adrenalectomy...
December 2016: International Journal of Angiology: Official Publication of the International College of Angiology, Inc
https://www.readbyqxmd.com/read/28030838/livin-birc7-expression-as-malignancy-marker-in-adrenocortical-tumors
#8
Barbara Altieri, Silviu Sbiera, Silvia Della Casa, Isabel Weigand, Vanessa Wild, Sonja Steinhauer, Guido Fadda, Arkadius Kocot, Michaela Bekteshi, Egle M Mambretti, Andreas Rosenwald, Alfredo Pontecorvi, Martin Fassnacht, Cristina L Ronchi
Livin/BIRC7 is a member of the inhibitors of apoptosis proteins family, which are involved in tumor development through the inhibition of caspases. Aim was to investigate the expression of livin and other members of its pathway in adrenocortical tumors and in the adrenocortical carcinoma (ACC) cell line NCI-H295R.The mRNA expression of livin, its isoforms α and β, XIAP, CASP3 and DIABLO was evaluated by qRT-PCR in 82 fresh-frozen adrenal tissues (34 ACC, 25 adenomas = ACA, 23 normal adrenal glands = NAG)...
December 21, 2016: Oncotarget
https://www.readbyqxmd.com/read/28009746/curative-surgical-resection-of-adrenocortical-carcinoma-determining-long-term-outcome-based-on-conditional-disease-free-probability
#9
Yuhree Kim, Georgios A Margonis, Jason D Prescott, Thuy B Tran, Lauren M Postlewait, Shishir K Maithel, Tracy S Wang, Jason A Glenn, Ioannis Hatzaras, Rivfka Shenoy, John E Phay, Kara Keplinger, Ryan C Fields, Linda X Jin, Sharon M Weber, Ahmed Salem, 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, George A Poultsides, Timothy M Pawlik
OBJECTIVE: To evaluate conditional disease-free survival (CDFS) for patients who underwent curative intent surgery for adrenocortical carcinoma (ACC). BACKGROUND: ACC is a rare but aggressive tumor. Survival estimates are usually reported as survival from the time of surgery. CDFS estimates may be more clinically relevant by accounting for the changing likelihood of disease-free survival (DFS) according to time elapsed after surgery. METHODS: CDFS was assessed using a multi-institutional cohort of patients...
January 2017: Annals of Surgery
https://www.readbyqxmd.com/read/27965001/calcifying-nested-stromal-epithelial-tumor-cnset-of-the-liver-in-beckwith-wiedemann-syndrome
#10
REVIEW
Nasim Khoshnam, Haynes Robinson, Michael R Clay, Lauren R Schaffer, Scott E Gillespie, Bahig M Shehata
Calcifying nested stromal-epithelial tumor (CNSET) is a rare neoplasm. In the 31 reported cases, CNSET is predominantly found in young girls and women. Beckwith-Wiedemann syndrome (BWS) (OMIM #130650) is an overgrowth syndrome with an increased risk to develop cancer. Associations have been seen between BWS and embryonal tumors, especially Wilms tumor, hepatoblastoma, and adrenocortical carcinoma. Here we report on a female patient with BWS who presented with CNSET. Two other cases with the same association have been reported, with our case representing the third such reported in the literature...
December 10, 2016: European Journal of Medical Genetics
https://www.readbyqxmd.com/read/27957799/outcome-and-prognostic-factors-in-high-risk-childhood-adrenocortical-carcinomas-a-report-from-the-european-cooperative-study-group-on-pediatric-rare-tumors-expert
#11
Giovanni Cecchetto, Alba Ganarin, Ewa Bien, Peter Vorwerk, Gianni Bisogno, Jan Godzinski, Patrizia Dall'Igna, Yves Reguerre, Dominik Schneider, Laurence Brugières, Pierre Leblond, Andrea Ferrari, Ines Brecht, Angela De Paoli, Daniel Orbach
OBJECTIVES: The aim of this retrospective international analysis was to evaluate the role of risk factors in pediatric patients with adrenocortical carcinoma (ACC) observed in European countries (2000-2013) in an attempt to identify factors associated with poor prognosis. PROCEDURES: Data were retrieved from databases of Germany, France, Poland, and Italy, which form the European Cooperative Study Group on Pediatric Rare Tumors (EXPeRT). Patients were less than 18 years old, with at least one of the following tumor-related risk factors: metastases, volume more than 200 cm(3) , Cushing syndrome, vascular or regional lymph node invasion, initial biopsy, or incomplete excision...
December 13, 2016: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/27957352/nonislet-cell-tumor-hypoglycemia-in-a-patient-with-adrenal-cortical-carcinoma
#12
Se Won Kim, Seung-Eun Lee, Young Lyun Oh, Seokhwi Kim, Sun Hee Park, Jae Hyeon Kim
Nonislet cell tumor hypoglycemia (NICTH) is a rare but serious paraneoplastic syndrome in which a tumor secretes incompletely processed precursors of insulin-like growth factor-II (IGF-II), causing hypoglycemia. Here, we report an exceptional case of NICTH caused by nonfunctioning adrenocortical carcinoma in a 39-year-old male with recurrent hypoglycemia. The patient's serum IGF-II/IGF-I ratio had increased to 27.8. The serum level of the IGF-II/IGF-I ratio was normalized after removal of the tumor, and the hypoglycemic attacks no longer occurred after the operation...
2016: Case Reports in Endocrinology
https://www.readbyqxmd.com/read/27940298/cell-signaling-pathways-in-the-adrenal-cortex-links-to-stem-progenitor-biology-and-neoplasia
#13
Morgan K Penny, Isabella Finco, Gary D Hammer
The adrenal cortex is a dynamic tissue responsible for the synthesis of steroid hormones, including mineralocorticoids, glucocorticoids, and androgens in humans. Advances have been made in understanding the role of adrenocortical stem/progenitor cell populations in cortex homeostasis and self-renewal. Recently, large molecular profiling studies of adrenocortical carcinoma (ACC) have given insights into proteins and signaling pathways involved in normal tissue homeostasis that become dysregulated in cancer. These data provide an impetus to examine the cellular pathways implicated in adrenocortical disease and study connections, or lack thereof, between adrenal homeostasis and tumorigenesis, with a particular focus on stem and progenitor cell pathways...
December 8, 2016: Molecular and Cellular Endocrinology
https://www.readbyqxmd.com/read/27928434/adrenalectomy-for-cushing-s-syndrome-do-s-and-don-ts
#14
REVIEW
Paduraru Dn, Nica A, Carsote M, Valea A
Aim. To present specific aspects of adrenalectomy for Cushing's syndrome (CS) by introducing well established aspects ("do's") and less known aspects ("don'ts"). Material and Method. This is a narrative review. Results. The "do's" for laparoscopic adrenalectomy (LA) are the following: it represents the "gold standard" for secretor and non-secretor adrenal tumors and the first line therapy for CS with an improvement of cardio-metabolic co-morbidities; the success rate depending on the adequate patients' selection and the surgeon's skills...
October 2016: Journal of Medicine and Life
https://www.readbyqxmd.com/read/27927710/adrenocortical-carcinoma-an-unusual-cause-of-secondary-hypertension
#15
Daniel Veron Esquivel, Fernando Batiz, Alfonso Farias Vega, Perla A Carrillo Gonzalez
We present the case of a female patient aged 39 years who was admitted to our hospital due to hypertension, severe hypokalaemia and metabolic alkalosis; physical examination was remarkable for plethoric moon face, centripetal obesity and bilateral lower extremity oedema. She was admitted for intravenous potassium replacement and further assessment of hypertension and associated clinical findings. Laboratory testing showed increased levels of aldosterone, renin, cortisol, testosterone and androstenedione. An abdominal CT revealed a large mass in the right adrenal gland with hepatic involvement...
December 7, 2016: BMJ Case Reports
https://www.readbyqxmd.com/read/27916625/validation-of-the-prognostic-role-of-the-helsinki-score-in-225-cases-of-adrenocortical-carcinoma
#16
Eleonora Duregon, Rocco Cappellesso, Valeria Maffeis, Barbara Zaggia, Laura Ventura, Alfredo Berruti, Massimo Terzolo, Ambrogio Fassina, Marco Volante, Mauro Papotti
Adrenocortical carcinoma patient prognosis is extremely variable and poorly predictable. The newly introduced Helsinki Score is the first so far proposed diagnostic and prognostic system based on the combined evaluation of morphological (mitoses and necrosis) and immunohistochemical (Ki-67) parameters. The aim of the study was to validate the prognostic role of the Helsinki Score for adrenocortical carcinoma characterization. Thus, 225 adrenocortical carcinomas were reclassified using the Weiss Score and the Helsinki Score (3× mitotic count +5× necrosis + Ki-67 index)...
December 1, 2016: Human Pathology
https://www.readbyqxmd.com/read/27899191/genetic-predisposition-to-endocrine-tumors-diagnosis-surveillance-and-challenges-in-care
#17
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
#18
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/27885669/occult-adrenocortical-carcinoma-and-unexpected-early-childhood-death
#19
Mark Pilla, John Gilbert, Lynette Moore, Roger W Byard
A four-year-old previously well boy collapsed unexpectedly and was taken immediately to hospital, where he developed seizures and cardiogenic shock with lethal, rapidly progressing multi-organ failure. At autopsy, the height was >90th percentile and there were indications of early virilization. Internally, a friable tumor of the left adrenal gland was identified that had invaded the left renal vein and inferior vena cava. Histology revealed typical features of an adrenocortical carcinoma with aggregated trabeculae of cells containing abundant eosinophilic cytoplasm and large pleomorphic nuclei...
January 2017: Journal of Forensic Sciences
https://www.readbyqxmd.com/read/27869874/-pharmacological-treatment-in-adrenal-cushing-s-syndrome
#20
Antonio Stigliano, Lidia Cerquetti, Vincenzo Toscano
ACTH-independent adrenal Cushing's syndrome is the least common form of endogenous hypercortisolism. Recently, advances in genetics have allowed the description of several forms different to pathogenetic etiology, morphostructural characteristics and evolution towards the hypercortisolism. Alongside these, the adrenocortical carcinoma is also frequently responsible of a hypercortisolism clinical picture. The availability of steroidogenesis inhibitors, such as metyrapone and ketoconazole, provides to endocrinologist a therapeutic chance against different metabolic disorders sustained by hypercortisolism...
November 2016: Recenti Progressi in Medicina
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