Chabre Olivier
CHU de Grenoble, Hôpital Nord, Service d'Endocrinologie-Diabétologie-Nutrition, BP 217, 38043 Grenoble cedex 9, France; Faculté de Médecine, Université Joseph Fourier, Grenoble-1, 38042 Grenoble cedex 9, France.
Presse Med. 2014 Apr;43(4 Pt 1):376-92. doi: 10.1016/j.lpm.2014.02.001. Epub 2014 Mar 20.
The most frequent cause of Cushing's syndrome is iatrogenic, as Cushing's syndrome is the unavoidable consequence of long-term glucocorticoid treatment using more than 7.5 mg prednisone per day. The most frequent cause of endogenous Cushing's syndrome is Cushing's disease (CD), which is an ACTH dependent hypercortisolism linked to a pituitary corticotroph adenoma. This adenoma is often very small, its diagnosis may require bilateral inferior petrosal sinus sampling and the first line treatment of CD is transsphenoidal surgery by an expert neurosurgeon. The second line treatments include drugs that can act either on the pituitary adenoma or on adrenal steroidogenesis, pituitary radiotherapy or bilateral adrenalectomy. Ectopic ACTH dependent Cushing's syndrome is linked either to poorly differentiated endocrine tumors with a very poor prognosis, such as small cell lung cancer, or to well differentiated endocrine tumors, such as bronchial carcinoid tumors, which have a good prognosis when treated by surgery, but may be very difficult to localize. Adrenal Cushing's syndromes, which are independent of pituitary ACTH secretion, include adrenal cortex carcinoma, which requires abdominal surgery with extended adrenalectomy by an expert surgeon, adrenal adenoma which is treated by laparoscopic unilateral adrenalectomy and bilateral macronodular hyperplasia, whose surgical treatment may require unilateral or bilateral adrenalectomy. Treatment of Cushing's syndrome generally leads to spectacular clinical results, which must not hide the fact that the reversibility of some signs is actually incomplete. This underlines the need for a timely multidisciplinary management of the patients by an expert team.
库欣综合征最常见的病因是医源性的，因为库欣综合征是长期每日使用超过7.5毫克泼尼松进行糖皮质激素治疗不可避免的后果。内源性库欣综合征最常见的病因是库欣病（CD），它是一种与垂体促肾上腺皮质激素细胞腺瘤相关的促肾上腺皮质激素依赖性皮质醇增多症。这种腺瘤通常非常小，其诊断可能需要双侧岩下窦取样，CD的一线治疗是由专业神经外科医生进行经蝶窦手术。二线治疗包括可作用于垂体腺瘤或肾上腺类固醇生成的药物、垂体放疗或双侧肾上腺切除术。异位促肾上腺皮质激素依赖性库欣综合征要么与预后极差的低分化内分泌肿瘤有关，如小细胞肺癌，要么与高分化内分泌肿瘤有关，如支气管类癌肿瘤，手术治疗时预后良好，但可能很难定位。与垂体促肾上腺皮质激素分泌无关的肾上腺性库欣综合征包括肾上腺皮质癌，这需要由专业外科医生进行扩大肾上腺切除术的腹部手术，肾上腺腺瘤通过腹腔镜单侧肾上腺切除术治疗，以及双侧大结节性增生，其手术治疗可能需要单侧或双侧肾上腺切除术。库欣综合征的治疗通常会带来显著的临床效果，但这不应掩盖某些体征实际上不完全可逆的事实。这突出了由专家团队对患者进行及时多学科管理的必要性。
