Stigliano Antonio, Cerquetti Lidia, Toscano Vincenzo
Endocrinologia, Dipartimento di Medicina Clinica e Molecolare, Ospedale Sant'Andrea, Sapienza Università di Roma.
Recenti Prog Med. 2016 Nov;107(11):574-581. doi: 10.1701/2484.25964.
ACTH-independent adrenal Cushing's syndrome is the least common form of endogenous hypercortisolism. Recently, advances in genetics have allowed the description of several forms different to pathogenetic etiology, morphostructural characteristics and evolution towards the hypercortisolism. Alongside these, the adrenocortical carcinoma is also frequently responsible of a hypercortisolism clinical picture. The availability of steroidogenesis inhibitors, such as metyrapone and ketoconazole, provides to endocrinologist a therapeutic chance against different metabolic disorders sustained by hypercortisolism. Mitotane, an adrenolitic compound, is used alone in adjuvant therapy or in combination with different chemotherapy drugs in the treatment of adrenocortical carcinoma and in the treatment of severe Cushing's syndrome.
促肾上腺皮质激素非依赖性肾上腺库欣综合征是内源性皮质醇增多症最不常见的形式。最近，遗传学的进展使得人们能够描述几种在致病病因、形态结构特征以及向皮质醇增多症发展方面不同的形式。除此之外，肾上腺皮质癌也常常导致皮质醇增多症的临床表现。米替拉酮和酮康唑等类固醇生成抑制剂的出现，为内分泌学家提供了治疗由皮质醇增多症引发的不同代谢紊乱的治疗机会。米托坦，一种肾上腺溶解化合物，单独用于辅助治疗，或与不同的化疗药物联合用于治疗肾上腺皮质癌以及严重的库欣综合征。
