垂体癌肿酷似异位ACTH综合征

分泌ACTH的垂体癌肿极其罕见,现常用的术语为侵入性或转移性肿瘤,垂体癌肿的细胞类型不同于垂体腺瘤.除了肿瘤的转移和侵入以外,皮肤的色素沉着是这些病人突出的临床表现之一。高度色素沉着有时可以在肾上腺切除前观察到,测定ACTH提供了垂体癌肿的色素沉着的激素基础,且须在肾上腺没有切除之前获得。作者对一例由于侵入性垂体肿瘤伴恶性组织学特征的临床和生化改变而引起库兴氏综合征的病例进行了描述,并与异位ACTH综合征之间诊断进行了比较。     

胸腺瘤伴异位ACTH综合征一例

胸腺瘤伴异位ＡＣＴＨ综合征一例常桂香温晨东患者女，３５岁。因颜面、胸部、双上肢浮肿１月，以“上腔静脉综合征原因待查”收入院。体检：Ｔ、Ｐ、Ｒ大致正常，ＢＰ２１．３／１３ｋＰａ，身高１６１ｃｍ、体重５２ｋｇ。双掌纹、肘关节色素沉着，颜面、胸部、双上肢可...     

异位ACTH综合征与垂体性库欣病的鉴别诊断

临床显示 ,异位促肾上腺皮质激素 (ACTH)综合征患者血浆ACTH水平较垂体肿瘤高 ,同时由于其垂体ACTH分泌受到抑制 ,由垂体外肿瘤产生的ACTH一般不被大剂量地塞米松抑制。甲吡酮抑制皮质醇合成时 ,垂体ACTH腺瘤ACTH分泌增加 ,而分泌ACTH的异位肿瘤不出现此种反应。促肾上腺皮质激素释放激素 (CRH)可刺激大多数垂体腺瘤患者的ACTH释放 ,但在异位ACTH综合征患者则无此作用。异位分泌ACTH肿瘤检测常依赖于胸腹部CT及MRI。依赖垂体ACTH的库欣病患者垂体附近ACTH浓度较被稀释的周围静脉中ACTH高 ,故亦可用两者比值 (IPS/P)对两种疾病鉴别。     

隐匿性异位ACTH综合征

一例 38岁男性被诊断为Ｃｕｓｈｉｎｇ综合征。生化检查显示低钾血症和高皮质醇血症 ,且不能被大剂量地塞米松抑制 ,提示是一种异位的或肾上腺源性的Ｃｕｓｈｉｎｇ综合征。血促肾上腺皮质激素水平 >1 0 0ＩＵ Ｌ。垂体磁共振成像、胸部放射成像、计算机Ｘ线断层扫描及气管镜检查均正常。肾上腺成像有腺体增生但无腺瘤 ,行 1 1 1Ｉｎ标记奥曲肽扫描 ,左侧胸部近中线位置有一区域示踪物质吸收增加。给患者行甲双吡丙酮治疗并双侧肾上腺切除 ,但术后死亡。尸检发现垂体正常 ,双侧肾上腺增生 ,左侧肺门气管示踪物质吸收增加的区域有一肿瘤样结…     

异位ACTH综合征研究进展

异位ACTH综合征（EAS）是Cushing综合征的一种特殊类型，是由于垂体以外的肿瘤组织分泌过量有生物活性的促肾上腺皮质激素（ACTH），刺激肾上腺皮质增生，产生过量皮质类固醇引起的临床综合征，占Cushing综合征患者总数的5％～10％。文献报道，最多见的病因为肺部或支气管肿瘤，约占50％，其次为胸腺及胰腺肿瘤，各约占10％，还可有甲状腺髓样癌、嗜铬细胞瘤及胃肠道、生殖系统、前列腺等部位的肿瘤。目前大部分异位ACTH综合征的肿瘤定位不能明确，给临床诊断和治疗带来很大困难。近年来，异位ACTH综合征课题组在异位ACTH的临床和基础研究中做了一定的工作，现整理如下。     

异位ACTH综合征与垂体性库欣病的鉴别诊断

临床显示,异位促肾上腺皮质激素（ACTH）综合征患者血浆ACTH水平较垂体肿瘤高,同时对由于其垂体ACTH分泌受到抑制,由垂体外肿瘤产生的ACTH一般不被大剂量地塞米松抑制。甲吡酮抑制皮质醇合成时,垂体ACTH腺瘤ACTH分泌增加,而分泌ACTH的位肿瘤不出现此种反应,促肾上腺皮质激素放激素（CRH）可刺激大多数垂体腺瘤患者的ACTH释放,但在异位ACTH综合征患者则无此作用,异位分泌ACTH肿瘤检测常依赖于胸腹部CT及MRI。依赖垂体ACTH的库欣病患者垂体附近ACTH浓度较被稀释的周围静脉中ACTH高,故亦可用两者比值（IPS／P）对两种疾病鉴别。     

异位ACTH综合征研究进展

异位ACTH综合征(EAS)是Cushing综合征的一种特殊类型,是由于垂体以外的肿瘤组织分泌过量有生物活性的促肾上腺皮质激素(ACTH),刺激肾上腺皮质增生,产生过量皮质类固醇引起的临床综合征,占Cushing综合征患者总数的5%~10%。文献报道,最多见的病因为肺部或支气管肿瘤,约占50%,其次     

纵隔类癌伴异位ACTH综合征一例

患者 ,女性 ,44岁。 1999年 5月始出现脸变圆、变红、多毛、月经紊乱、高血压 (最高达 180 / 10 0mmHg)在当地医院查血皮质醇检查 :晨 8时为 3 5 5 .9μg/L ,16时为 410 .8μg/L ,2 4时为3 66.4μg/L ,空腹血糖 15 .6mmol/L ,肾上腺CT扫描提示为左侧肾上腺增生 ,诊断库欣氏综合征。于 2 0 0 0年 6月 15日在当地医院行左侧肾上腺切除术 ,术后病理诊断左侧肾上腺增生。患者术后血压下降 ,脸红、头昏症状消失 ,月经恢复正常 ,血皮质醇水平下降。但术后 4个月起患者上述症状复发 ,于当地医院再次复查血皮质醇升高 ,血ACTH 175 pg/ml,CT检…     

异位ACTH综合征二例

例1，女性，47岁，因全身浮肿一个月余，发热、咳嗽一周入院。患者于一个月前无明显诱因渐起面及下肢浮肿伴乏力，渐次出现面长痤疮、唇周长胡须，皮肤变黑，逐渐加重，一周前出现发热、咳嗽。起病后即停经。曾至某医院住院发现顽固性低血钾转诊来院。体检：体温38℃，血压128／83mmHg(1mmHg=0．133kPa)，皮肤呈褐黑色，全身皮肤呈凹陷性水肿，未见紫     

Ectopic ACTH syndrome

Ectopic adrenocorticotropic secretion (EAS) is responsible for 12-17% of cases of Cushing's syndrome (CS) and covers a range of tumours, from undetectable benign lesions to widespread metastases. The syndrome is often associated with severe hypercortisolaemia, which aggravates the underlying condition. EAS requires a complete workup that includes the establishment of endogenous CS, diagnosis of adrenocorticotropic hormone (ACTH) dependency, localization of the source of ACTH secretion and rapid biochemical control of hypercortisolaemia. Dynamic endocrine tests should include inferior petrosal sinus sampling with CRH stimulation. Localization studies depend on the availability of reliable high-resolution cross-sectional imaging. This systematic review of the largest published series of patients with EAS (over 380 patients) reveals the common trends in the prevalence and management of this syndrome. The concept of 'occult' EAS has been revisited and the terms 'overt' and 'covert' EAS introduced. In addition to small cell lung carcinoma, the most common causes of ectopic EAS are bronchial carcinoids, thymic tumours, islet cell tumour of the pancreas, medullary thyroid carcinomas, and phaeochromocytomas. Their prevalence and the best localization modalities are presented. Medical and surgical management is discussed on the basis of the extensive experience of major referral centres.     

异位ACTH综合征的研究进展

能引起异位促肾上腺皮质激素(ACTH)综合征的肿瘤种类较多,β连环蛋白相关的Wnt信号通路、Nouch信号通路、P21活化的蛋白激酶3在异位分泌ACTH肿瘤的发生、发展中起重要作用,促阿片黑素细胞皮质素原基凶启动子区去甲基化与肿瘤异位分泌激素密切相关.在诊疗方面,血清嗜铬粒蛋白A的测定大大提高了异位ACTH综合征的阳性预测率.除手术治疗外,联合用药较单独使用生长抑素或选择性多巴胺受体激动剂更为有效.     

Ectopic ACTH syndrome associated with anorectal carcinoma

Summary A 25-year-old black homosexual was noted to be hypertensive, hypokalemic, and to have a rectal mass. Histopathology of the biopsied lesion revealed a mixture of poorly differentiated squamous cell and undifferentiated small cell carcinoma. Abdominopelvic CT showed multiple liver metastases, minimal local tumor extension, and normal adrenal glands. Despite aggressive treatment, he remained hypertensive and hypokalemic. Endocrine work-up revealed: normal 24-hr VMA and catecholamines, normal serum aldosterone and renin levels, elevated urinary free cortisol (3360 g/24 hr), elevated serum cortisol (60 g/dl), and elevated serum ACTH (1697 pg/dl). Liver biopsy confirmed metastatic anorectal carcinoma, and immunohistochemical stains of the rectal biopsy were positive for ACTH and neuron-specific enolase. Although many types of neoplasms have been associated with ectopic ACTH production, small cell carcinoma of the lung is the most common. While there are many reports of colorectal and anorectal neuroendocrine small cell carcinomas, few of these tumors have been associated with clinical ectopic hormone production. This case represents the first report of the ectopic ACTH syndrome associated with anorectal carcinoma.Presented in abstract form at the Virginia State American College of Physicians meeting, Charlottesville, Virginia, March 1991.     

Ectopic ACTH syndrome associated with ovarian steroid-cell tumor

Several cases of ovarian neoplasms resulting in Cushing's syndrome due to ectopic secretion of ACTH or ectopic secretion of F have been reported. Tumors producing ACTH include adenocarcinoma, androblastoma, Sertoli cell carcinoma, carcinoid tumor and teratoma. Cortisol secretion has been reported in ovarian steroid cell tumor (unclassified steroid cell tumor). We present a case of a 19-year-old woman with Cushing's syndrome in course of an ovarian steroid cell tumor with ectopic ACTH production. To our knowledge, it is the first reported case of ACTH secreting ovarian steroid cell tumor causing Cushing's syndrome.     

胸腺类癌致异位ACTH综合征

目的 总结胸腺类癌引起的异位ACTH综合征临床表现，提高对此综合征的诊断率。方法 分析6例患者的临床表现，尤其是非典型胸腺类癌所致异位ACTH综合征的症状和体征、实验室相关测定值以及影像学表现。结果 6例患者均行胸腺肿瘤切除术并经病理证实为胸腺类癌导致异位ACTH综合征。结论 有明确库欣综合征临床表现，且伴有低血钾，浮肿，蛋白尿且出现不能解释的8mg地塞米松抑制试验不被抑制，均应考虑异位ACTH综合征，胸腺类癌是异位ACTH综合征的可能病因。     

Ectopic ACTH syndrome caused by bronchial carcinoid

A 35-year male patient with clinical and biochemical manifestations of severe hypercorticism was thought to have Cushing's syndrome of pituitary origin. However, the surgically removed pituitary adenoma was not confirmed by pathological examination. There was no improvement after transsphenoidal microsurgery Chest CT scan showed a small mass located at the upper-lobe of the right lung. This tumor (1.8 x 1.0 x 1.0 cm) was removed and one course of radiotherapy with linear accelerator was given. Remission was achieved clinically and biochemically after these therapies. The diagnosis of bronchial carcinoid was confirmed by pathological findings. Metastasis of a lymph node was also proved. The tumor cells was found to contain ACTH and related peptides with radioimmunoassay, immunocytochemistry and electron microscopy. Gel filtration of the tumor extracts showed molecular heterogeniety of ACTH related peptides and that the big-form were in large percentage.     

Ectopic ACTH syndrome caused by pulmonary carcinoid tumourlets

The differential diagnosis of Cushing's syndrome is a major challenge to clinical endocrinologists, especially those infrequent cases referred to as occult ectopic ACTH syndromes. Although bronchial carcinoids are well known to be a cause of Cushing's syndrome due to ectopic ACTH secretion, very few cases of carcinoid tumourlets causing an ACTH ectopic syndrome have been reported, and their origin remains controversial. For some authors, tumourlets and typical carcinoids represent distinct pathological entities, whilst others hold that tumourlets are merely microscopic carcinoid tumours. We report a patient with an aggressive Cushing's syndrome that required bilateral adrenalectomy, diagnosed 22 years before a 3-cm lung nodule became apparent on routine chest X-ray. The biopsy after lung surgery revealed a typical peripheral bronchial carcinoid surrounded by tumourlets. Both tumourlets and carcinoid tumour showed strongly positive ACTH immunostaining. Recently, Arioglu et al. (1998) reported a case of Cushing's syndrome caused by pulmonary carcinoid tumourlets, concluding that this entity should be considered in the differential diagnosis of occult ectopic ACTH syndrome. Furthermore, we consider that the carcinoid tumourlets found in our patient, were the initial source of ACTH, leading to Cushing's syndrome with a rapid onset, and that a loss of cell proliferation control in one of such tumourlets many years later, could have resulted in the development of a typical carcinoid tumour, reinforcing the theory of a common origin of these lesions.