TY - Jour A2 - Appetecchia，Marialuisa Au - Pozza，Carlotta Au - Graziadio，Chiara Au - Giannetta，Elisa Au - Lenzi，Andrea Au - Isidori，Andrea M. Py - 2012 DA - 2012/08/09 TI - 2012/08/09Aggressive Cushing's Syndrome: From Macroadenomas to Ectopics SP - 685213 VL - 2012 AB - Cushing’s syndrome (CS) is a rare but severe clinical condition represented by an excessive endogenous cortisol secretion and hence excess circulating free cortisol, characterized by loss of the normal feedback regulation and circadian rhythm of the hypothalamic-pituitary axis due to inappropriate secretion of ACTH from a pituitary tumor (Cushing’s disease, CD) or an ectopic source (ectopic ACTH secretion, EAS). The remaining causes (20%) are ACTH independent. As soon as the diagnosis is established, the therapeutic goal is the removal of the tumor. Whenever surgery is not curative, management of patients with CS requires a major effort to control hypercortisolemia and associated symptoms. A multidisciplinary approach that includes endocrinologists, neurosurgeons, oncologists, and radiotherapists should be adopted. This paper will focus on traditional and novel medical therapy for aggressive ACTH-dependent CS. Several drugs are able to reduce cortisol levels. Their mechanism of action involves blocking adrenal steroidogenesis (ketoconazole, metyrapone, aminoglutethimide, mitotane, etomidate) or inhibiting the peripheral action of cortisol through blocking its receptors (mifepristone “RU-486”). Other drugs include centrally acting agents (dopamine agonists, somatostatin receptor agonists, retinoic acid, peroxisome proliferator-activated receptor γ.“PPAR- γ.“配体”和新型化学治疗剂（替替莫唑胺和酪氨酸激酶抑制剂）对侵袭性垂体或异位肿瘤具有显着的活性。SN - 1687-8450 UR - https://doi.org/10.1155/2012/685213 do - 10.1155 / 2012/685213 jf - 肿瘤学杂志Pb - Hindwi Publishing Corporation KW - ER -