摘要
目的总结1例嗜铬细胞瘤导致的ACTH依赖性高皮质醇血症患者的临床及生化特征。方法 1报告我院收治的1例嗜铬细胞瘤所致异位ACTH综合征(EAS)的患者的诊治经过;2回顾性分析国内外文献报道的30余例嗜铬细胞瘤所致EAS的临床和生化特点。结果本例男性,23岁。1年体重增加5 kg。有多饮、多尿、多汗及手抖等症状。经详细检查最终诊断为嗜络细胞瘤所致的EAS。结论 1流行病学:嗜铬细胞瘤所致EAS占全部EAS的5%~25%,发病年龄12~74岁,女性比例高(F:M=7:1);2临床特点:低血钾和高血压更常见,多为单侧肾上腺占位,偶有双侧病变;3生化特点:尿儿茶酚胺升高,大剂量地塞米松抑制试验(HDDST)后的血皮质醇反向升高;4病理特点:病理标本免疫组化染色ACTH阳性;5术后转归:切除病变侧肾上腺后ACTH明显下降。
Objective To explore the clinical and biochemical characteristics of a 23 year old male patient with ectopic ACTH syndrome caused by pheochromocytoma, and the related literature were reviewed. Methods ① The diagnosis and treatment of a case of EAS caused by pheochromocytoma from Peking Union Medical College Hospital was described. ② Retrospective analyzed the clinical and biochemical characteristics of 30 cases of ACTH-producing pheochoromocytoma reported in PUBMED or CNKI. Results The patient was male, twenty-three years old. He gained 5 kg in weigh in the past one year. His symptoms included polydipsia, dieresis, hidrosis and tremor in hand. He was diagnosed with EAS caused by pheochromocytoma after carefully examinations. Conclusions ① ACTH-secreting pheochromocytoma could be seen in 5%-25% of patients with the ectopic ACTH syndrome. Patient age ranged from 25 to 74 years and the majority are female（F：M =7：1）. ② Hypokalemia and hypertension is more common for unilateral adrenal occupying lesions, occasionally for bilateral lesions③ Urine catecholamine is elevated and paradoxically response to high dose dexamethasone suppression test. ④ Histopathology of the adrenal tumor showed immunopositive stain for ACTH. ⑤ ACTH is significantly decreased after the removal of adrenal lesions.
出处
《北京医学》
CAS
2017年第4期337-340,F0003,共5页
Beijing Medical Journal
