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Adrenal cortical carcinoma

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https://www.readbyqxmd.com/read/27857836/refractory-hypoglycemia-in-a-patient-with-functional-adrenal-cortical-carcinoma
#1
Katia Regina Marchetti, Maria Adelaide Albergaria Pereira, Arnaldo Lichtenstein, Edison Ferreira Paiva
: Adrenacarcinomas are rare, and hypoglycemic syndrome resulting from the secretion of insulin-like growth factor II (IGF-II) by these tumors have been described infrequently. This study describes the case of a young woman with severe persistent hypoglycemia and a large adrenal tumor and discusses the physiopathological mechanisms involved in hypoglycemia. The case is described as a 21-year-old woman who presented with 8 months of general symptoms and, in the preceding 3 months, with episodes of mental confusion and visual blurring secondary to hypoglycemia...
2016: Endocrinology, Diabetes & Metabolism Case Reports
https://www.readbyqxmd.com/read/27716880/peritoneal-metastases-from-adrenal-cortical-carcinoma-treated-by-cytoreductive-surgery-and-hyperthermic-intraperitoneal-chemotherapy
#2
Paul H Sugarbaker
PURPOSE: Adrenal cortical carcinoma is a rare cancer that often presents in an advanced stage. Not only systemic metastases but also local recurrence and peritoneal metastases prevent long-term survival in these patients. METHODS: A profoundly symptomatic patient with extensive peritoneal metastases and local recurrence was treated using cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) with melphalan as the chemotherapy agent. RESULTS: Relative sparing of the small bowel within the abdomen and pelvis allowed a visible complete resection of all cancer...
September 27, 2016: Tumori
https://www.readbyqxmd.com/read/27708447/mammary-development-hyperestrogenemia-and-hypocortisolemia-in-a-male-cat-with-an-adrenal-cortical-carcinoma
#3
Amy C Nadolski, Jessica E Markovich, Samuel H Jennings, Orla M Mahony
A 14-year-old neutered male domestic shorthaired cat was diagnosed with an adrenal cortical carcinoma causing hyperestrogenemia that resulted in mammary hyperplasia and sexual behavior. A right adrenalectomy and mammary gland biopsy were performed. Adrenal cortical neoplasia should be ruled out in any neutered male cat with mammary development and/or exhibiting sexual behavior.
October 2016: Canadian Veterinary Journal. la Revue Vétérinaire Canadienne
https://www.readbyqxmd.com/read/27688079/sarcomatoid-adrenal-carcinoma-case-report-with-contribution-to-pathogenesis
#4
Wolfgang Saeger, Werner Mohren, Matthias Behrend, Peter Iglauer, Waldemar Wilczak
A tumor in the adrenal region with two metastases in the liver was classified as poorly differentiated sarcoma on the base of extensive immunostainings (expression of vimentin, desmin, myogenin, and CD31, no expression of inhibin, melan A). Four years later in a second examination with molecular methods for a study of adrenal sarcomas, this diagnosis must be revised due to the lack of MDM-2 gene amplification and FKHR translocation which exclude sarcoma. Further immunostainings of many other parts of the tumor showed in one area more mature tumor tissue expressing synaptophysin, SF-1, and melan A...
September 29, 2016: Endocrine Pathology
https://www.readbyqxmd.com/read/27622479/li-fraumeni-syndrome
#5
Carlos Andrés Ossa, Gustavo Molina, Alicia María Cock-Rada
The Li-Fraumeni syndrome is characterized clinically by the appearance of tumors in multiple organs generally at an early age. This hereditary condition is caused by germinal mutations in the TP53 gene, which codifies for the tumoural suppressor gene p53. We present the case of a patient aged 31 with clinical and molecular diagnosis of Li-Fraumeni syndrome who presented two synchronous tumors: a leiomyosarcoma on the forearm and a phyllodes breast tumour. She had a family history of cancer, including a son diagnosed with a cortical adrenal carcinoma when he was three years old, who died at five from the disease...
June 3, 2016: Biomédica: Revista del Instituto Nacional de Salud
https://www.readbyqxmd.com/read/27617148/li-fraumeni-syndrome
#6
REVIEW
Hernán Correa
Li-Fraumeni syndrome (LFS) is a cancer predisposition syndrome caused by a germline mutation of the TP53 gene on chromosome 17p13.1. It has an autosomal dominant pattern of inheritance with high penetrance. These patients have a very high lifetime cumulative risk of developing multiple malignancies and have a strong family history of early-onset malignancies. The protein p53, encoded by TP53, has a complex set of genome-preserving functions initiated during episodes of cellular stress and DNA damage. In LFS, TP53 gene mutations cause the loss of function of p53, leading to downstream events permissive for development of various malignancies throughout life...
June 2016: Journal of Pediatric Genetics
https://www.readbyqxmd.com/read/27562041/-etiology-analysis-of-522-hospitalized-cases-with-cushing-syndrome
#7
L L Li, J T Dou, G Q Yang, W J Gu, Z H Lü, Y M Mu
OBJECTIVE: To summarize the characteristics of the etiology distribution of Cushing syndrome (CS) patients in Chinese PLA general hospital during the past 20 years. METHODS: All data of CS patients admitted to Chinese PLA General Hospital between 1995 and 2014 were collected. The sex ratio of patients, visiting age, final diagnosis were retrospectively analyzed. RESULTS: From January 1995 to December 2014, 522 patients with CS were admitted, of which, 134 (25...
August 16, 2016: Zhonghua Yi Xue za Zhi [Chinese medical journal]
https://www.readbyqxmd.com/read/27493811/paediatric-nonfunctioning-adrenocortical-carcinoma-with-extension-up-to-right-side-heart-cardiac-surgery-approach
#8
Federica Iezzi, Andrea Quarti, Chiara Surace, Marco Pozzi
Adrenocortical carcinoma is a rare malignancy. Due to late diagnosis and no adequate effective adjuvant treatment, prognosis remains poor. Only approximately 30% of these malignancies are confined to the adrenal gland when they are diagnosed, as these tumors tend to be found years after their genesis. Cardiac involvement of adrenal carcinoma is very rare. We report a rare case of a 7-year-old female with right adrenal cortical carcinoma, involving the right-side heart.
2016: Case Reports in Cardiology
https://www.readbyqxmd.com/read/27438375/adrenal-pathology-in-the-adult-a-urological-pathologist-s-perspective
#9
Donna E Hansel, Victor E Reuter
Adrenal gland diagnostics can pose significant challenges. In most academic and community practice settings, adrenal gland resections are encountered less frequently than other endocrine or genitourinary specimens, leading to less familiarity with evolving classifications and criteria. The unique dichotomy between cortical and medullary lesions reflects the developmental evolution of these functionally independent components. Adrenal cortical lesions at resection include hyperplasia, adenoma, and carcinoma, with some cases straddling the boundary between these distinct clinical classifications...
September 2016: Advances in Anatomic Pathology
https://www.readbyqxmd.com/read/27424218/pediatric-adrenal-cortical-carcinomas-histopathological-criteria-and-clinical-trials-a-systematic-review
#10
REVIEW
Xia Xu, Consolato Sergi
Adrenal tumors are quite rare in infancy and childhood with the exception of neuroblastoma. In fact, adrenocortical tumors (ACT) account for only 0.2% of all malignant cancers in children and adolescents. According to a multicenter registry investigation, the median interval between first endocrine symptoms and the diagnosis of ACT is 5months, and death is seen in 38% of patients, who suffer from tumor progression following the diagnosis in about 2½years. The prognosis of pediatric ACC is poor with a 5-year event-free survival of 54%...
September 2016: Contemporary Clinical Trials
https://www.readbyqxmd.com/read/27422613/synchronous-vs-metachronous-metastases-in-adrenocortical-carcinoma-an-analysis-of-the-dutch-adrenal-network
#11
M H T Ettaieb, J C Duker, R A Feelders, E P M Corssmit, C W Menke-van der Houven van Oordt, H J L M Timmers, M N Kerstens, J W Wilmink, P M Zelissen, B Havekes, H R Haak
Adrenal Cortical Carcinoma (ACC) is a rare malignancy with an incidence of 1.0 per million per year in the Netherlands. Median survival varies according to the European Network for the Study of Adrenal Tumours (ENS@T) tumour stage. It is unknown whether time until development of metastases is of influence on prognosis. To asses this, data were retrospectively obtained from centres of the Dutch Adrenal Network. Patients who presented with ACC between January 1, 2004 and October 31, 2013 were included. Date of detection of metastases, number of metastases and affected organs were registered...
July 15, 2016: Hormones & Cancer
https://www.readbyqxmd.com/read/27385106/proteome-analysis-of-adrenal-cortical-tumors
#12
Hye Min Kim, Yu Kyung Lee, Ja Seung Koo
INTRODUCTION: Adrenal tumor is a relatively common tumor. The discrimination between adrenal cortical adenoma (ACA) and adrenal cortical carcinoma (ACC) is crucial as these two diseases have distinct prognosis. ACA is a benign tumor curable by surgical excision, while the prognosis of ACC is extremely poor, with a 5-year mortality of 75-90%. Therefore, previous proteomic studies focused on markers allowing the differentiation between ACA and ACC. AREAS COVERED: Several proteomic approaches based on the analysis of various samples such as human tissues, urine, and cell lines...
August 2016: Expert Review of Proteomics
https://www.readbyqxmd.com/read/27358692/endocrine-radionuclide-scintigraphy-with-fusion-single-photon-emission-computed-tomography-computed-tomography
#13
Ka-Kit Wong, Arpit Gandhi, Benjamin L Viglianti, Lorraine M Fig, Domenico Rubello, Milton D Gross
AIM: To review the benefits of single photon emission computed tomography (SPECT)/computed tomography (CT) hybrid imaging for diagnosis of various endocrine disorders. METHODS: We performed MEDLINE and PubMed searches using the terms: "SPECT/CT"; "functional anatomic mapping"; "transmission emission tomography"; "parathyroid adenoma"; "thyroid cancer"; "neuroendocrine tumor"; "adrenal"; "pheochromocytoma"; "paraganglioma"; in order to identify relevant articles published in English during the years 2003 to 2015...
June 28, 2016: World Journal of Radiology
https://www.readbyqxmd.com/read/27313728/adrenal-cortical-carcinoma-with-tumor-thrombus-extension-into-the-right-atrium-a-case-report
#14
Yimin Wang, Feng Zhou, Hao Pan, Baiye Jin
Adrenal cortical carcinoma (ACC) is a rare aggressive malignant tumor associated with a poor prognosis. Patients with ACC usually present with abnormal hormone secretion, tumor growth or encroachment on the surrounding viscera. An early diagnosis of ACC is considered challenging. The present study reports a case of ACC with tumor thrombus extension into the inferior vena cava and right atrium. A 33-year-old male patient exhibited symptoms of gynecomastia and abdominal distention, which were due to the abnormal hormone secretion levels and to the presence of a large mass in the right adrenal gland...
June 2016: Oncology Letters
https://www.readbyqxmd.com/read/27296006/-ectopic-adrenal-cortical-adenoma-in-the-spinal-canal-a-case-report-and-a-review-of-the-literature
#15
A S Konstantinov, K V Shelekhova
Ectopic adrenal cortical neoplasms are extremely rare. The authors describe their own case of intradural, extramedullary conus medullaris adenoma that occurred in a 55-year-old woman, which was clinically accompanied by lumbar pains, left leg numbness, and left foot weakness during 10 years. The mass was histologically composed of rounded and polygonal cell fields with rounded, regular nuclei and abundant eosinophilic and clear cytoplasm. There were no necroses or mitoses. The cells were immunohistochemically positive for cytokeratin AE1/3, vimentin, inhibin-α, melan-A, and synaptophysin...
May 2016: Arkhiv Patologii
https://www.readbyqxmd.com/read/27227935/clinicopathological-features-associated-with-the-prognosis-of-patients-with-adrenal-cortical-carcinoma-usefulness-of-the-ki-67-index
#16
Yun Mi Choi, Hyemi Kwon, Min Ji Jeon, Tae-Yon Sung, Suck Joon Hong, Tae Yong Kim, Won Bae Kim, Young Kee Shong, Jae Lyun Lee, Dong Eun Song, Won Gu Kim
Adrenocortical carcinoma (ACC) is a rare tumor with a poor prognosis. Identification of clinicopathological features and molecular prognostic markers is important for the treatment of ACC. The aim of this study was to evaluate the clinical and histopathological features of ACC for prognostic prediction.This retrospective cohort study included 86 patients pathologically confirmed with ACC in a single center. Ki-67 index was evaluated by immunohistochemical staining of paraffin-embedded samples.The median age of the 86 (46 male and 40 female) patients with ACC was 49 years old (range 21-78), and the mean primary tumor size was 12...
May 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/27014458/adrenal-oncoctyoma-of-uncertain-malignant-potential-a-rare-etiology-of-adrenal-incidentaloma
#17
Rohit R Kedia, Lucy Muinov, Subodh M Lele, Vijay Shivaswamy
A rare cause for rapid adrenal enlargement is adrenal oncocytoma of uncertain malignant potential. A full biochemical evaluation is warranted to screen secreting adrenal adenomas as well as to evaluate adrenal cortical carcinoma. Careful pathologic evaluation is required as the diagnosis of AOC cannot be made by imaging.
March 2016: Clinical Case Reports
https://www.readbyqxmd.com/read/26989398/a-possible-new-multiple-endocrine-neoplasia-mutation-in-a-patient-with-a-prototypic-multiple-endocrine-neoplasia-presentation
#18
Rino Buzzola, Lilamani Romayne Kurukulasuriya, Mariana Touza, Norman S Litofsky, Stephen Brietzke, James R Sowers
BACKGROUND: Multiple endocrine neoplasia (MEN) type 1 syndrome is an uncommon inherited disorder characterized by the occurrence of tumors involving two or more endocrine glands. These tumors include pheochromocytoma, adrenal cortical and neuroendocrine tumors including (bronchopulmonary, thymic, gastric), lipomas, angiofibromas, collagenomas, and meningiomas. MEN-4 is very rare and has been characterized by the occurrence of parathyroid and anterior pituitary tumors in association with tumors of the adrenals, kidneys, and reproductive organs...
February 2016: Cardiorenal Medicine
https://www.readbyqxmd.com/read/26793526/testicular-adrenal-rest-tumors-tarts-with-unusual-histological-features-in-congenital-adrenal-hyperplasia-cah
#19
Valeri Marianovsky, Olga Bogdanova, Milen Tsvetkov, Denitsa Serteva, Boris Mladenov
Congenital adrenal hyperplasia (CAH) patients with testicular adrenal rest tumors (TARTs) with testicular enlargement present a serious diagnostic challenge. According to the data TARTs are usually benign. They are rare, resulting in paucity in the medical literature regarding their pathological features. We report a case of bilateral synchronous mass-forming TARTs with marked cytological and nuclear atypia misinterpreted as malignant testicular tumors in a 40-years-old man with CAH and CT and MRI data for pheochromocytoma of the right adrenal gland and paraaortal and paracaval lymphadenomegaly...
July 2015: Urology Case Reports
https://www.readbyqxmd.com/read/26765762/metastatic-adrenal-cortical-carcinoma-to-t12-vertebrae
#20
Daniel Lee, Vijay Yanamadala, Ganesh M Shankar, John H Shin
We report spinal metastasis of adrenal cortical carcinoma (ACC) to the T12 vertebrae with epidural extension. ACC is a rare malignancy with poor prognosis and high rates of metastasis. However, spinal lesions of ACC are rare, and few have been reported in the literature. We discuss our management of this lesion and review the current understanding and treatment of ACC and spinal metastasis.
May 2016: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
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