多血质外貌，向心性肥胖.ppt

Definition Cushing syndrome, the constellation of clinical signs and symptoms resulting form chronic glucocorticoid excess, was probably first described in 1899 定 义 为各种病因造成肾上腺分泌过多的糖皮质激素（主要是皮质醇）所致病症的总称 主要临床表现为满月脸，多血质外貌，向心性肥胖、痤疮、皮肤紫纹高血压和骨质疏松等 Aetiology ACTH-dependent Cushing syndrome Cushing disease Ectopic ACTH syndrome ACTH- independent Cushing syndrome Adrenocortical adenoma Adrenocortical adenocarcinoma Micronodular adrenal disease Massive macronodular adrenal disease 病因分类 依赖ACTH 的cushing综合症 cushing 病 异位ACTH综合症 非依赖ACTH 的cushing综合症 肾上腺皮质腺瘤 肾上腺皮质癌 原发性色素结节性肾上腺病 大结节性巨大肾上腺病 Cushing Disease It is the most common type of Cushing syndrome, and accounts for about 70% of the syndrome Pathogenesis Most patients have ACTH-secreting anterior pituitary corticortrope microadenomas(＜10mm in diameter), but a small number have diffuse corticotrope hyperplasia and pituitary macroadenoma Cushing病 最常见，约占cushing综合症的70%,多见于成人，女性多于男性，是由于垂体分泌过多的ACTH所致 病因 垂体微腺瘤－80% 垂体大腺瘤－10％：压迫、侵袭等症状 下丘脑功能紊乱 Ectopic ACTH syndrome The majority of patients with ectopic ACTH syndrome have outside pituitary tumor Other sites are pulmonary carcinosis, carcinoid of bronchus, thymic carcinoma pancreatic cancer, chromaffin tumor, medullary carcinoma of thyroid and so on 异位ACTH综合症 继发于垂体以外的恶性肿瘤 小细胞肺癌、支气管类癌、胸腺癌、胰腺癌、嗜铬细胞瘤、神经母细胞瘤 等等 临床上分两型 缓慢进展型 迅速进展型 Adrenocortical adenoma Patients with adrenal adenoma usually have gradual onset of sign of hypercortisolism, accounts for about 15-20% of the syndrome Adenoma is round or ellipse, diameter is 3-4cm, weight is about 40g 肾上腺皮质腺瘤 多见于成人，男性多于女性－ 15%-20% 腺瘤呈圆形或椭圆形，直径约3-4cm，重40g左右，包膜完整 其病缓慢，病情中等，多毛及雄激素增多少见 Adrenocortical adenocarcinoma It occurs in lower than 5% patients of cushing syndrome Patients with adenocarcinoma tend to have a more acute and progressive course and virilizing effects may predominate The bulk of tumor is larger 肾上腺皮质腺癌 占cushing综合症的5％以下 瘤体积大（直径5-6cm)，肿瘤可浸润包膜 病情重，进展快，重度cushing综合症的表现(低钾性碱中毒、女性男性化） Micronodular adrena