1、Cushing syndromeCushing syndrome Cushing 综合症 DefinitionDefinition Cushing syndrome,the Cushing syndrome,the constellation of clinical signs constellation of clinical signs and symptoms resulting form and symptoms resulting form chronic chronic glucocorticoidglucocorticoid excess,excess,was probably
2、first described in was probably first described in 1899 1899 定定 义义 为各种病因造成肾上腺分泌过多的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）所致糖皮质激素（主要是皮质醇）所致病症的总称病症的总称 主要临床表现为满月脸，多血质外主要临床表现为满月脸，多血质外貌，向心性肥胖、痤疮、皮肤紫纹貌，向心性肥胖、痤疮、皮肤紫纹高血压和骨质疏松等高血压和骨质疏松等 AetiologyAetiology Clinical presentationClinical presentation laboratory examinatio
3、nlaboratory examination Diagnosis and differential Diagnosis and differential diagnosisdiagnosis Treatment (principle)Treatment (principle)PrognosisPrognosis 病因分类病因分类 临床表现临床表现 实验室检查实验室检查 诊断及鉴别诊断诊断及鉴别诊断 治疗治疗(原则原则)预后预后 AetiologyAetiology 病因分类病因分类 HypothalamusHypothalamus 下丘脑下丘脑 （CRHCRH）pituitary gland
4、pituitary gland 垂体垂体 （ACTHACTH）adrenal cortexadrenal cortex肾上腺皮质肾上腺皮质 zonazona glomerulosaglomerulosa zonazona fasciculatafasciculata zonazona reticularisreticularis （AldosteroneAldosterone）（）（cortisolcortisol）（）（gonadalgonadal hormonehormone）+-AetiologyAetiology ACTHACTH-dependent Cushing syndromed
5、ependent Cushing syndrome Cushing diseaseCushing disease Ectopic ACTH syndromeEctopic ACTH syndrome ACTHACTH-independent Cushing syndrome independent Cushing syndrome AdrenocorticalAdrenocortical adenomaadenoma AdrenocorticalAdrenocortical adenocarcinomaadenocarcinoma MicronodularMicronodular adrena
6、l diseaseadrenal disease Massive Massive macronodularmacronodular adrenal diseaseadrenal disease 病因分类病因分类 依赖依赖ACTH ACTH 的的cushingcushing综合症综合症 cushingcushing 病病 异位异位ACTHACTH综合症综合症 非依赖非依赖ACTH ACTH 的的cushingcushing综合症综合症 肾上腺皮质腺瘤肾上腺皮质腺瘤 肾上腺皮质癌肾上腺皮质癌 原发性色素结节性肾上腺病原发性色素结节性肾上腺病 大结节性巨大肾上腺病大结节性巨大肾上腺病 Clinica
7、l Clinical featurefeature 各型临床特征各型临床特征 Cushing DiseaseCushing Disease It is the most common type of Cushing syndrome,It is the most common type of Cushing syndrome,and accounts for about 70%of the syndromeand accounts for about 70%of the syndrome PathogenesisPathogenesis Most patients have ACTHMost
8、patients have ACTH-secreting anterior pituitary secreting anterior pituitary corticortropecorticortrope microadenomasmicroadenomas(10mm in diameter),but a 10mm in diameter),but a small number have diffuse small number have diffuse corticotropecorticotrope hyperplasia and hyperplasia and pituitary pi
9、tuitary macroadenomamacroadenoma CushingCushing病病 最常见，约占最常见，约占cushingcushing综合症的综合症的70%70%,多见于多见于成人，女性多于男性，是由于垂体分泌过成人，女性多于男性，是由于垂体分泌过多的多的ACTHACTH所致所致 病因病因 垂体微腺瘤垂体微腺瘤80%80%垂体大腺瘤垂体大腺瘤1010：压迫、侵袭等症状：压迫、侵袭等症状 下丘脑功能紊乱下丘脑功能紊乱 Ectopic ACTH syndromeEctopic ACTH syndrome The majority of patients with ectopic
10、ACTH syndrome The majority of patients with ectopic ACTH syndrome have outside pituitary tumor have outside pituitary tumor Other sites are pulmonary Other sites are pulmonary carcinosiscarcinosis,carcinoidcarcinoid of of bronchus,bronchus,thymicthymic carcinoma pancreatic cancer,carcinoma pancreati
11、c cancer,chromaffinchromaffin tumor,tumor,medullarymedullary carcinoma of thyroid carcinoma of thyroid and so onand so on 异位异位ACTHACTH综合症综合症 继发于垂体以外的恶性肿瘤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、神经母细胞瘤癌、嗜铬细胞瘤、神经母细胞瘤 等等等等 临床上分两型临床上分两型 缓慢进展型缓慢进展型 迅速进展型迅速进展型 AdrenocorticalAdrenocortical
12、adenomaadenoma Patients with adrenal adenoma usually have gradual Patients with adrenal adenoma usually have gradual onset of sign of onset of sign of hypercortisolismhypercortisolism,accounts for,accounts for about 15about 15-20%of the syndrome20%of the syndrome Adenoma is round or ellipse,diameter
13、 is 3Adenoma is round or ellipse,diameter is 3-4cm,4cm,weight is about 40gweight is about 40g 肾上腺皮质腺瘤肾上腺皮质腺瘤 多见于成人，男性多于女性多见于成人，男性多于女性 15%15%-20%20%腺瘤呈圆形或椭圆形，直径约腺瘤呈圆形或椭圆形，直径约3 3-4 4cmcm，重重4040g g左右，包膜左右，包膜完整完整 其病缓慢，病情中等，多毛及雄激素增多少见其病缓慢，病情中等，多毛及雄激素增多少见 AdrenocorticalAdrenocortical adenocarcinomaadenoca
14、rcinoma It occurs in lower than 5%patients It occurs in lower than 5%patients of of cushingcushing syndromesyndrome Patients with Patients with adenocarcinomaadenocarcinoma tend tend to have a more acute and to have a more acute and progressive course and progressive course and virilizingvirilizing
15、effects may predominateeffects may predominate The bulk of tumor is largerThe bulk of tumor is larger 肾上腺皮质腺癌肾上腺皮质腺癌 占占cushingcushing综合症的综合症的5 5以下以下 瘤体积大（直径瘤体积大（直径5 5-6 6cm)cm)，肿瘤可浸润包膜肿瘤可浸润包膜 病情重，进展快，重度病情重，进展快，重度cushingcushing综合症的表现综合症的表现(低钾性碱中毒、低钾性碱中毒、女性男性化）女性男性化）MicronodularMicronodular adrenal ad
16、renal diseasedisease The disease is also known as Meadors syndrome The disease is also known as Meadors syndrome About half of patients with this disorder have no About half of patients with this disorder have no distinctive clinical presentation other than being distinctive clinical presentation other than being youngyoung-always younger than 30 years of age,half always younger than 30 years of age,half younger than 15 years and some infantsyounger than 15 years and some infants 原发性色素结节性肾上腺病原发性
