异位促肾上腺皮质激素综合征

<正>概述异位促肾上腺皮质激素(adrenocorticotropic hormone,ACTH)综合征(ectopic ACTH syndrome,EAS)作为库欣综合征的一种特殊类型,是因垂体以外ACTH的肿瘤组织分泌过量具有生物活性的ACTH,刺激肾上腺皮质增生并产生过量皮质类固醇引起的临床综合征,占库欣综合征患者总数的5%～10%^[1]。国外文献报道最多见病因为肺部或支气管肿瘤,约占50%,其次分别为胸腺及胰腺肿瘤,各约占10%,还可有甲状腺髓样癌(medullary thyroid carcinoma,MTC),嗜铬细胞瘤,胃肠道及生殖系统、前列腺等部位的肿瘤^[2]。     

类癌致异位促肾上腺皮质激素综合征病例报道

异位促肾上腺皮质激素(ACTH)综合征属库欣综合征的一种特殊类型,其发病机制是非垂体的肿瘤组织分泌过量的具有生物活性的ACTH或ACTH类似物,刺激.肾上腺皮质增生并分泌过量的皮质醇,引起一系列临床症状.本义列举了2例由不同部位类癌所敛的异位ACTH综合征,详细阐述了其临床特征、实验室及影像学检查特点,并总结了在诊治过程中的经验与教训,使临床医生能够对此种疾病有充分的了解,及早诊断和治疗.     

继发于脑膜瘤的异位促肾上腺皮质激素综合征一例

患者男，61岁。5个月前出现颜面及双下肢水肿，夜尿增多．5～6次／晚，食欲增加，但体重无明显增加，乏力渐明显，生活不能自理，近1个月血压160／100mmHg（1mmHg=0．133kPa)，右跟视力下降，降压利尿浩疗，疗效不佳。无头痛、恶心、呕吐，无毛发增多，无畏寒、发热，无咳嗽、咳痰、腹痛、腹泻。体检：BP150／90mmHg，体重指数21．9kg/m^2，神清，无满月脸、水牛背及向心性肥胖,无皮肤紫纹、痤疮，面部及胸部皮肤发红、全身浅表淋巴结不大，眼睑水肿，眼球活动自如，瞳孔对光反射正常，颈软，甲状腺不大，心肺正常，腹部未扪及包头，肝脾未及，双下肢轻度水肿，四肢肌力、肌张力正常。膝反射减弱，病理征阴性。     

腹膜后副神经节瘤1例

腹膜后副神经节瘤１例梁启廉吴江广东医学院附属医院肿瘤科广东省湛江市５２４００１主题词腹膜后肿瘤副神经节瘤中国图书资料分类号Ｒ７３５４病例报告男，５７岁．因上腹部持续胀痛１个月于１９９６－０５－３０入院．查体：Ｔ３７℃，Ｐ８５次／ｍｉｎ，Ｒ１９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…     

肾上腺副神经节瘤引起顽固性腹泻1例

1例顽固性腹泻患者,经影像学初步诊断,病理组织学确诊为肾上腺副神经节瘤,为一罕见病例．     

老年肺癌合并异位促肾上腺皮质激素综合征2例

异位促肾上腺皮质激素综合征(ectopic adrenocorticotropic hormone syndrome,EAS)是库欣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的有生物活性的促肾上腺皮质激素(Adrenocorticotropic hormone,ACTH)刺激肾上腺皮质增生,从而产生过量皮质类固醇而引起的临床综合征。老年肺癌     

SDHB基因错义突变致心脏副神经节瘤一例

患者青年女性。以阵发性心悸、多汗、头痛、血压升高为表现,检测儿茶酚胺及代谢产物甲氧基肾上腺素类（MNs）水平增高,基因检测为琥珀酸脱氢酶亚基B（SDHB）错义突变,影像学检查发现由冠状动脉左回旋支供血的纵隔肿瘤（40 mm×38 mm）,经充分药物准备后在体外循环下行肿瘤手术切除,术后病理确诊为心脏副神经节瘤。患者术后...     

垂体促肾上腺皮质激素瘤并发急性左心衰竭一例

患者女性 ,36岁。因多食、肥胖 5年 ,突发气急、咯血 1次 ,于 2 0 0 2年 9月 13日入院。 5年来患者体重增加 10kg。 3个月前突发气急、胸闷、咳嗽、咯粉红色泡沫样痰 ,端坐呼吸 ,不能平卧。当时测血压 170 / 110mmHg(1mmHg =0 133kPa) ,诊断为“高血压病、高血压性心脏病、急性左心衰竭” ,予以强心、利尿、降压等对症治疗后缓解。以后一直服用降压药物 ,但血压控制差。血压波动在 15 0～ 16 0 / 95～ 10 0mmHg之间。体格检查 :身高 16 0cm ,体重 77 5kg ,血压 15 2 / 94mmHg。轻度向心性肥胖 ,水牛背 ,满月脸 ,多血质面容。双肺呼吸音清…     

Thoracoscopic resection of functional posterior mediastinal paraganglioma: a case report

A 48-year-old man with posterior mediastinal mass was diagnosed as functional mediastinal paraganglioma during surgical exploration via open thoracotomy in another hospital. The operation was terminated because of severe hypertension when touching the tumor. He was transferred to our center later. After systemic evaluation, the patient was medicated with oral alpha- and beta-blockades, as well as intravenous fluid resuscitation for two weeks. His blood pressure became stable and a second operation was planned. The tumor was removed completely via the thoracoscopic approach, and was finally confirmed as functional paraganglioma by immunohistochemistry. The patient recovered uneventfully after surgery, with no recurrence during one year follow-up visit.     

A case report of mediastinal paraganglioma and a review of the literature in Japan]

Paraganglioma is a tumor arising from the extra-adrenal paraganglion system and is rarely observed in the mediastinum. The authors experienced a case of nonfunctioning paraganglioma of the upper posterior mediastinum. The patient was a 40-year-old female in whom abnormal shadows were noted in chest X-ray films taken in March, 1989. She was examined at our hospital with a diagnosis of mediastinal tumor in June of the same year. CT of the chest revealed a tumor in the upper posterior mediastinum and it was shown to be hypervascular by a DSA study. Urinary hormone levels were normal. The tumor was resected in May, 1990. The tumor, measuring 6.8 X 4.0 X 3.5 cm, was encapsulated, appeared dark red and had relatively clear borders. A histopathologic diagnosis of paraganglioma was made. The patient remains disease-free at present, 6 months after operation. Problems concerning diagnosis and treatment of mediastinal paraganglioma were evaluated with a review of cases reported in Japan.     

类癌致异位促肾上腺皮质激素综合征病例报道专家点评

异位促肾上腺皮质激素(ACTH)综合征属库欣综合征的一种特殊类型,其发病机制是非垂体的肿瘤组织分泌过量的具有生物活性的ACTH或ACTH类似物,刺激肾上腺皮质增生并分泌过量的皮质醇引起的临床综合征.引起异位ACTH综合征的最常见病因为肺癌,尤其是小细胞肺癌,约占50%.但类癌与异位ACTH综合征一文的特点是总结了临床较为少见的类癌引起异位ACTH综合征的病例,主要因为:(1)类癌引起异位ACTH综合征发病率有增加的趋势,如以往有关类癌致异位ACTH综合征的报道仅占异位ACTH综合征的10%,但在20世纪80年代以后的病例报道中,类癌的比例增大,约占36%～46%.     

Case of small cell lung cancer complicated with diabetes insipidus and Cushing syndrome due to ectopic adrenocorticotropic hormone secretion]

A 54-year-old woman had been given a diagnosis with scleroderma and interstitial pneumonia due to scleroderma when she was 45 years old. Thirst, with resulting polydipsia and polyuria (about 7 liters/day) were present since May, 2004, and bloody sputum appeared in June of 2004. The patient was admitted to our hospital. Chest CT examination showed multiple nodules in the bilateral lower lung field and multiple movable subcutaneous nodules on the abdomen. Small-cell lung cancer (metastases in the pituitary, subcutaneous tissue, and lungs) was diagnosed by transbronchial lung biopsy and subcutaneous nodule biopsy of the abdomen. The final diagnosis was diabetes insipidus and Cushing syndrome. Chemotherapy was done with CDDP and VP-16, which resulted in reduction of the tumor and improvement in endocrinological findings. Nevertheless, chemotherapy could not be continued because of infected bullae. The patient died of deteriorating illness after 91 sickness days. We concluded that this case was Cushing syndrome caused by ectopic adrenocorticotropic hormone-producing small cell lung cancer, and that it presented with diabetes insipidus because of pituitary metastasis. Therefore, when drastic endocrinological changes are found, it is important to examine for cancer, including lung cancer, as soon as possible.