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By David E. Schteingart (auth.), Gary D. Hammer, Tobias Else (eds.)

This first version of the textbook “Adrenocortical Carcinoma: simple Sciences and scientific recommendations” offers a concise compilation of appropriate information advancing our present figuring out of this infrequent melanoma. Chapters are devoted to a number of elements of either the elemental biology and the scientific technological know-how when it comes to adrenocortical carcinoma (ACC). specialist clinicians introduce the reader to present cutting-edge scientific care of sufferers with ACC. As a substantial physique of information on ACC has gathered over the past a long time, professional scientists have authored extra sections that function a reference resource for the skilled researcher in addition to an creation for the trainee within the laboratory or health facility. scientific issues span the epidemiology, prognosis and remedy of ACC. clinical issues components span the pathology and pathophysiology of ACC with a unique specialise in experimental types of adrenocortical tumors and up to date advances within the genetic features and signaling pathways in ACC. The content material of this e-book was once compiled and edited with the rationale of facilitating the interpretation of data into destiny diagnostic and healing instruments that might eventually increase sufferer care.

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Additional resources for Adrenocortical Carcinoma: Basic Science and Clinical Concepts

Example text

Based on this data, it is very likely that the percentage of endocrine-inactive tumors is largely overestimated 38 B. Allolio and M. 1 Hormonal work-up in patients with suspected or proven ACC. Recommendations of the ACC Working Group of the European Network for the Study of Adrenal Tumors (ENSAT), May 2005 • Glucocorticoid excess (minimum three out of four tests) • Sexual steroids and steroid precursors • Mineralocorticoid excess • Exclusion of a pheochromocytoma - Dexamethasone suppression test (1 mg, 23:00 h) - Excretion of free urinary cortisol (24-h urine) - Basal cortisol (serum) - Basal ACTH (plasma) - DHEAS (serum) - 17-OH-Progesterone (serum) - Androstenedione (serum) - Testosterone (serum) - 17-Beta-estradiol (serum, only in men and postmenopausal women) - Potassium (serum) - Aldosterone/renin ratio (only in patients with arterial hypertension and/or hypokalemia) - Catecholamine or metanephrine excretion (24-h urine) - Meta- and normetanephrines (plasma) concept that virtually all ACCs secret steroids.

Preparation for possible aortopulmonary bypass in cases where tumor extends into the vena cava. This assumes appropriate pre-operative imaging in all patients (Fig. 2a, b). 6. Nephrectomy is not beneficial unless kidney or renal vein is invaded (Fig. 3). 7. A reoperative procedure is indicated where local recurrence can be excised without excessive risks (see Fig. 1). 8. Use of mitotane postoperatively in all patients without anaplastic histopathology providing serum monitoring can maintain serum levels above 14 μg/ml and below 20 μg/ml.

Thus, it is very likely that the percentage of endocrine inactive tumors is largely overestimated. Therefore, in cases in which detailed hormone analyses reveal no autonomous hormone secretion, caution is warranted not to misdiagnose a nonadrenocortical tumor of the adrenal region as an ACC. As demonstrated in other series also, the data from the German Registry confirm that glucocorticoid and androgen excess are by far the most frequent types of hormonal abnormality. 6%) androgen excess (Fig. 4).

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