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National Cancer Institute®
Ultima Vez Modificado: 1 de marzo del 2002
UI - 11701664
AU - Koch CA; Chrousos GP
TI - Editorial: Is the diminuto/dwarf1 gene involved in physiologic adrenocortical size regulation and tumor formation?
SO - J Clin Endocrinol Metab 2001 Nov;86(11):5127-9
UI - 11712085
AU - Forneris M; Gottardo L; Albertin G; Malendowicz LK; Nussdorfer GG
TI - Expression and function of adrenomedullin and its receptors in Conn's adenoma cells.
SO - Int J Mol Med 2001 Dec;8(6):675-9
AD - Department of Human Anatomy and Physiology, Section of Anatomy, University of Padua, I-35121 Padua, Italy.
Adrenomedullin (ADM) is a hypotensive peptide, that derives from the proteolytic cleavage of pro(p)ADM and acts through two subtypes of receptors, called L1-receptor (L1-R) and calcitonin receptor-like receptor (CRLR). CRLR may function as a calcitonin gene-related peptide or a selective ADM receptor depending on the expression of the subtype 1 or the subtypes 2 and 3 of a family of proteins, named receptor-activity modifying proteins (RAMPs). Reverse transcription (RT)-polymerase chain reaction (PCR) allowed the detection of pADM mRNA in dispersed cells of eight Conn's adenomas (aldosteronomas). These cells also expressed peptidyl-glycine alpha-amidating monooxigenase, the enzyme converting immature ADM to the mature form, and contained sizeable amounts of ADM-immunoreactivity as measured by radioimmunoassay. RT-PCR also demonstrated the presence in aldosteronoma cells of the specific mRNAs of L1-R, CRLR and RAMPs 1-3. ADM (10(-8) M) inhibited angiotensin-II (10(-9) M)-simulated aldosterone secretion from cultured aldosteronoma cells, without affecting basal production. ADM (10(-8) M) also enhanced basal proliferation rate of cultured cells, as estimated by the 5-bromo-2'-deoxyuridine immunocytochemical technique. Both effects of ADM were annulled by the ADM-receptor selective antagonist ADM22-52 (10(-7) M). In conclusion, our study provides evidence that aldosteronoma cells express both ADM and ADM22-52-sensitive receptors. These findings, coupled with the demonstration that ADM exerts an aldosterone antisecretagogue action and a proliferogenic effect on cultured aldosteronoma cells, make it likely that endogenous ADM system plays a potentially important role in the paracrine or autocrine functional control of Conn's adenomas.
UI - 11808146
AU - Ichikawa T; Ito H
TI - [Adrenocortical carcinoma]
SO - Nippon Rinsho 2001 Nov;59 Suppl 7():393-400
AD - Department of Molecular Oncology, Graduate School of Medicine, Chiba University.
UI - 11837629
AU - Yarman S; Gurbuz L; Tanakol R; Kapran Y; Alagol F
TI - Association of Fallot Tetralogy with Carney's complex.
SO - Ir Med J 2001 Nov-Dec;94(10):305-7
AD - Department of Internal Medicine, Istanbul Faculty of Medicine, Turkey. firstname.lastname@example.org
The Carney complex is an inherited, autosomal disease of multicentric tumors in many organs. Some components of Carney's complex are cardiac myxoma, spotty pigmentation, and endocrine overactivity. Primary pigmented nodular adrenocortical dysplasia (PPNAD) is an exceedingly rare cause of Cushing's syndrome in infants, children, and young adults. PPNAD occurs sporadically or as part of a familial syndrome called Carney's complex. Up to our knowledge, the association of Fallot Tetralogy with Carney's complex has not been previously reported. We presented, a 20-year-old woman, who had been operated for Fallot Tetralogy at the age of 3 years, had Carney's complex, i.e. left atrial myxoma, two facial spotty pigmented areas, and PPNAD.
UI - 11602999
AU - Becherer A; Vierhapper H; Potzi C; Karanikas G; Kurtaran A; Schmaljohann
TI - J; Staudenherz A; Dudczak R; Kletter K FDG-PET in adrenocortical carcinoma.
SO - Cancer Biother Radiopharm 2001 Aug;16(4):289-95
AD - Department of Nuclear Medicine, University of Vienna, Austria. email@example.com
Adrenal cortical carcinoma (ACC) is a rare malignant neoplasm with a poor prognosis. Radical surgery of the primary tumor and of local as well as of distant recurrence is the only effective treatment, and requires accurate and early localization of recurrent tumors. In this regard, we prospectively scanned 10 patients with ACC, 8 during follow-up and 2 at primary work-up. In all patients PET scans from the neck to the upper thighs were obtained 45 minutes after injection of 370 MBq [18F]FDG. Reading was done visually, with the investigator blinded to the results of other diagnostic modalities. All known sites of ACC lesions showed markedly increased FDG uptake. In 3 patients, previously unknown lesions were identified by PET in the lung (one lesion), the abdomen (3 lesions), and the skeleton (multiple), respectively. One false positive liver focus was shown by PET aside from the true positive lung metastases in the same patient. The sensitivity/specificity of PET based on different organs was 100/97%, that based on the number of PET-detected lesions (N = 23) was 100/95%. PET altered or influenced the tumor stage in 3/10 patients, modifying the subsequent therapeutic management in 2/10 patients. We conclude that FDG-PET is highly useful in ACC and should be included in the work-up for initial staging as well as for follow-up.
UI - 11825112
AU - Loy TS; Phillips RW; Linder CL
TI - A103 immunostaining in the diagnosis of adrenal cortical tumors: an immunohistochemical study of 316 cases.
SO - Arch Pathol Lab Med 2002 Feb;126(2):170-2
AD - Department of Pathology, University of Missouri Health Sciences Center, 1 Hospital Drive, Columbia, MO 65212, USA. firstname.lastname@example.org
CONTEXT: The monoclonal antibody A103 recognizes an antigen on melanoma cells known as Melan-A or MART-1. Recent studies have shown that A103 also reacts with adrenal cortical cells and may be useful in the diagnosis of adrenal cortical tumors. However, only small numbers of some of the tumors in the differential diagnosis of adrenal cortical neoplasms have been studied. OBJECTIVE: To study the specificity of A103 immunohistochemistry in a large number of tumors in the differential diagnosis of adrenal cortical neoplasms. DESIGN: Formalin-fixed, paraffin-embedded tissue from 21 adrenal cortical tumors, 16 cases of metastatic carcinoma to the adrenal, 10 pheochromocytomas, and 269 extra-adrenal carcinomas was evaluated for A103 immunoreactivity using a commercially available antibody (Novocastra, Newcastle, UK). RESULTS: Positive staining was seen in all of the adrenal cortical tumors but in none of the adrenal metastases or pheochromocytomas. In the 269 extra-adrenal carcinomas, A103 immunoreactivity was limited to a single ovarian serous carcinoma. CONCLUSION: A103 immunostaining is useful in distinguishing adrenal cortical neoplasms from other carcinomas and pheochromocytoma.
UI - 11844815
AU - Stojadinovic A; Ghossein RA; Hoos A; Nissan A; Marshall D; Dudas M;
TI - Cordon-Cardo C; Jaques DP; Brennan MF Adrenocortical carcinoma: clinical, morphologic, and molecular characterization.
SO - J Clin Oncol 2002 Feb 15;20(4):941-50
AD - Department of Surgery, Memorial Sloan-Kettering Cancer Center, New York, NY 10021, USA.
PURPOSE: To define multimolecular phenotypes of adrenocortical carcinoma (ACC) and to correlate outcome with morphologic and molecular parameters. PATIENTS AND METHODS: Clinical data were analyzed for 124 patients, histopathologic slides for 67 primary tumors, and tissue specimens for 74 patients (38 primary and 36 metastatic tumors) with ACC and for 38 normal adrenal tissue samples. Molecular expression profiles were investigated by immunohistochemistry. The prognostic significance of 12 gross and histologic parameters in 67 primary ACCs was evaluated. Morphologic and protein expression patterns were correlated with disease-specific survival (DSS). Univariate influence of prognostic factors on DSS was analyzed by log-rank test and multivariate analysis by Cox regression. RESULTS: The median follow-up period was 4.7 years. Significant predictors of DSS included distant metastasis at time of initial presentation; venous, capsular, and adjacent organ invasion; tumor necrosis, mitotic rate, atypical mitosis, and mdm-2 overexpression. Five-year DSS by number (one to six) of adverse histologic parameters was as follows: one to two, 84%; three to four, 37%; more than four, 9% (P =.005).The phenotype Ki-67(-)p53(-)mdm-2(+)cyclinD1(-)Bcl-2(-)p21(-)p27(+) was observed in 83% of normal and 3% of malignant adrenal tissue (P =.01). Molecular phenotypic expression was more heterogeneous in malignant than in normal (10 v five phenotypes) adrenal tissue. CONCLUSION: Meticulous morphologic evaluation, mitotic count, and tumor stage are essential in determining prognosis for patients with ACC. Multimolecular phenotyping demonstrates that the molecular complexity and heterogeneity of these neoplasms are such that targeted therapy needs to be patient specific.
UI - 11809538
AU - Vierhapper H
TI - Adrenocortical tumors: clinical symptoms and biochemical diagnosis.
SO - Eur J Radiol 2002 Feb;41(2):88-94
AD - Department of Internal Medicine III, Division of Endocrinology and Metabolism, University of Vienna, Wahringer Gurtel 18-20, A-1090, Vienna, Austria.
Patients who are suspected clinically to suffer from hypersecretory disorders of their adrenal(s) should undergo an appropriate endocrinological investigation to confirm or exclude the presence of such disorders prior to any radiological investigation. In those patients in whom an adrenal mass is found 'incidentally' on imaging clinical symptoms of hormonal activity should be carefully followed up. Asymptomatic patients should be screened biochemically for latent hormonal hypersecretion syndromes including pheochromocytoma (urine catecholamine excretion), hypercortisolism (overnight dexamethason suppression test) and aldosteronism (blood pressure and serum potassium). These investigations are mandatory in all patients scheduled for surgery. The decision to refer patients with inactive adrenal tumors to surgery is, in the absence of valid biochemical markers of malignancy, mainly influenced by tumor size but remains arbitrary. Patients who are not at first treated by surgery should be operated if follow-up indicates a progression in tumor size.
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