摘要
目的总结副神经节瘤引起异位促肾上腺皮质激素(ACTH)综合征(EAS)的临床特点及治疗方法。方法回顾性分析1例腹膜后副神经节瘤引起EAS患者的临床资料,并复习国内外相关文献。结果本例患者,女,53岁,因"发现高血压15年,乏力3个月,头晕、头痛1个月"入院。生化提示低钾血症,血、尿皮质醇明显升高,同步ACTH不被抑制,24 h尿香草杏仁酸(VMA)及血浆游离甲氧基肾上腺素类物质(MNs)升高,腹部CT提示腹膜后腹主动脉旁包块。行左侧腹膜后占位切除术,免疫组化:Syn(+),CgA(+),ACTH(局灶+)。回顾分析16篇文献报道中的22例及本例患者资料,发现大部分异位分泌ACTH的副神经节瘤可有典型的库欣综合征临床表现,且少有典型副神经节瘤临床表现,且大部分病例未按照副神经节瘤进行术前准备。结论异位分泌ACTH的副神经节瘤非常罕见,建议术前对EAS患者常规检测儿茶酚胺、MNs及24 h尿VMA等指标,以期提高术前诊断率,手术切除是最佳治疗方案,需同时按照副神经节瘤和库欣综合征进行术前准备,并建议所有患者完善嗜铬细胞瘤/副神经节瘤相关基因检测,术后终身随访。
Objective To present the clinical characteristics and treatment on patients with ectopic adrenocorticotropic hormone(ACTH)syndrome(EAS)caused by the retroperitoneal paraganglioma.Methods The clinical data of a case of EAS caused by retroperitoneal paraganglioma were analyzed retrospectively,and the related literature at home and abroad was reviewed.Results The 53-year-old female patient was admitted to endocrinology department due to a fifteen-year history of hypertension,accompanied by fatigue for three months,headache and dizziness for one month.The laboratory data demonstrated severe hypokalemia,high level of serum and urinary cortisol,while the ACTH level remained unsuppressed.The 24 h urinary vanillyl mandelic acid(VMA)and serum free methoxyepinephrine(MNs)level were elevated.The abdominal computed tomographic scan suggested a retroperitoneal mass next to the abdominal aorta.After the retroperitoneal tumor resection was performed,immunohistochemical staining of the tumor revealed Syn(+),CgA(+),ACTH(focal+).By the retrospective analysis of 22 similar cases from 16 papers and the case summarized above,we found that most patients with EAS caused by the paraganglioma could demonstrate the typical clinical features of Cushing′s syndrome,while lack of the manifestation of paraganglioma.Therefore,preoperative preparations for paraganglioma were usually neglected.Conclusions Ectopic ACTH syndrome(EAS)originating from paraganglioma is very rare.To improve the diagnosis rate,examination for catecholamine,MNs and 24 h urinary VMA before surgery in patients with EAS is suggested.Considering surgical resection as the optimal treatment,comprehensive preoperative preparations for both paraganglioma and Cushing′s syndrome are significant.A genetic test for pheochromocytoma/paraganglioma and lifelong postoperative follow-up are also recommend.
作者
乐颖
张莹
吴炎
刘岩峰
彭全洲
陈立波
胡园园
张庆梅
Le Ying;Zhang Ying;Wu Yan;Liu Yanfeng;Peng Quanzhou;Chen Libo;Hu Yuanyuan;Zhang Qingmei(Department of Endocrinology and Metabolism,Shenzhen People′s Hospital(The Second Clinical Medical College,Jinan University,The First Affiliated Hospital,Southern University of Science and Technology),Shenzhen 518020,China;Department of Urology,Shenzhen People′s Hospital(The Second Clinical Medical College,Jinan University,The First Affiliated Hospital,Southern University of Science and Technology),Shenzhen 518020,China;Department of Pathology,Shenzhen People′s Hospital(The Second Clinical Medical College,Jinan University,The First Affiliated Hospital,Southern University of Science and Technology),Shenzhen 518020,China;Department of Endocrinology and Metabolism,Huazhong University of Science and Technology Union Shenzhen Hospital(Nanshan Hospital),Shenzhen 518052,China)
出处
《中国医师杂志》
CAS
2022年第10期1498-1503,共6页
Journal of Chinese Physician
基金
广东省医学科研基金项目(A2021302)
