Substantial further advances in our understanding of adrenal development, steroid biosynthesis and adrenocortical disorders necessitate another look at this small but complex organ. In this volume, leaders in the fields of adrenal development, biochemistry and genetics review the most recent findings from bench and bedside research. New aspects of adrenal development and zonation and the novel ‘backdoor’ pathway to androgen synthesis are discussed. P450 oxidoreductase deficiency (a newly described form of congenital adrenal hyperplasia) and long-term outcome of prenatal treatment of 21-hydroxylase deficiency are examined. An update on ACTH resistance and adrenal Cushing’s syndrome is provided. Finally, the role of adrenal steroidogenesis in arterial hypertension and fetal programming of adrenal androgen excess as a possible explanation for polycystic ovary syndrome in later life are analyzed. This book will be of great interest to pediatric, adult and reproductive endocrinologists, clinical geneticists, developmental biologists and basic scientists working in the fields of endocrinology and steroidogenesis.
117 - 132: Cushing Syndrome Caused by Adrenocortical Tumors and Hyperplasias (Corticotropin- Independent Cushing Syndrome)
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Published:2008
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Book Series: Endocrine DevelopmentSubject Area: Endocrinology , Further Areas , Genetics , Pathology and Cell Biology , Women's and Children's Health
Constantine A. Stratakis, 2008. "Cushing Syndrome Caused by Adrenocortical Tumors and Hyperplasias (Corticotropin- Independent Cushing Syndrome)", Disorders of the Human Adrenal Cortex, C.E. Flück, W.L. Miller
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Abstract
Endogenous Cushing syndrome (CS) is caused by excess adrenal glucocorticoid secretion that is adrenocorticotropin(ACTH)-dependent or independent; ACTH-independent adrenocortical causes of CS account forup to 20% of CS in adults, and 15% in children over age 7 years. In younger children, ACTH-independent CSmay account for as many as half of the CS cases. In both adults and children, adrenocortical lesions causingCS include the common, isolated and sporadic, solitary cortisol-producing adenoma, the rare adrenocorticalcancer, and a spectrum of recently recognized, bilateral hyperplasias (bilateral adrenocortical hyperplasias,BAHs): micronodular adrenal disease and its pigmented variant, primary pigmented nodular adrenocorticaldisease are mostly genetic processes. Macronodular BAHs, ACTH-independent macronodular hyperplasia ormassive macronodular adrenocortical disease are less frequently genetic and almost never present in children(except in McCune-Albright syndrome); they present often with atypical CS in middle-aged or elderlyadults. The majority of benign adrenocortical tumors associated with CS are associated with defects of thecAMP signaling pathway, whereas adrenal cancer is linked to aberrant expression of growth factors andgermline or somatic mutations of tumor suppressor genes such as TP53. Adrenalectomy is the preferredmode of treatment for all adrenocortical causes of CS.
