小嗜铬细胞瘤副神经节瘤21例临床特征分析  

Analysis of the clinical characteristics of small pheochromocytoma and paraganglioma

作　　者：蒋鹏 吴珏莅 罗亚[2] 王德林[3] 吕发金[4] 甄倩娜 曹友德[5] 周波[2] JIANG Peng;WU Jue-li;LUO Ya;WANG De-lin;LYU Fa-jin;ZHEN Qian-na;CAO You-de;ZHOU Bo(University-Town Hospital of Chongqing Medical University,Chongqing 401331,China)

机构地区：[1]重庆医科大学附属大学城医院,重庆401331 [2]重庆医科大学附属第一医院,重庆400016 [3]重庆医科大学附属第一医院泌尿外科,重庆400016 [4]重庆医科大学附属第一医院影像科,重庆400016 [5]重庆医科大学附属第一医院病理科,重庆400016

出　　处：《中国实用内科杂志》2018年第10期950-954,共5页Chinese Journal of Practical Internal Medicine

基　　金：国家临床重点专科建设项目(2011170)

摘　　要：目的分析小的嗜铬细胞瘤副神经节瘤(pheochromocytoma and paraganglioma,PPGL)的临床特征,提高医务人员对小PPGL的认识。方法回顾性分析2011年4月至2016年2月就诊于重庆医科大学附属第一医院的103例PPGL患者的临床资料,根据术前CT提示最大直径分为小PPGL组(直径≤3 cm)和较大PPGL组(直径>3 cm),比较两组间临床表现、血浆游离甲氧基肾上腺素类物质(MNs)、影像学和病理学特征、围术期情况等的差异。结果小PPGL组21例,肿瘤平均直径为(2.39±0.67) cm。小PPGL组患者头痛、心悸、多汗、其他PPGL临床表现和至少具有嗜铬细胞瘤经典三联征的两种表现的发生率均低于较大肿瘤组,且小PPGL组内三联征症状均不具备者所占比例高于较大肿瘤组,但差异均缺乏统计学意义。小PPGL组血浆甲氧基肾上腺素和甲氧基去甲肾上腺素均显著低于较大PPGL组,小PPGL组血浆游离MNs水平轻微升高和正常的发生率均显著高于较大肿瘤组。两组PPGL在CT上有一些相似表现,平扫时CT值均大于10 Hu,绝大部分PPGL平扫CT值大于20 Hu,在增强CT上均主要表现为明显强化。小PPGL组患者术前误诊率明显高于较大肿瘤组。小PPGL组患者手术时间及术中出血量明显少于较大肿瘤组。结论小PPGL并不少见,临床表现往往不典型,生化标志物更多的表现为轻微升高或正常,而解剖和功能成像有助于识别本病,尤其多种临床资料综合分析可减少小PPGL的漏诊、误治。Objective To improve the knowledge and the diagnostic level of small pheochromocytoma and paraganglioma （PPGL） through analysis of the clinical characteristics of small PPGL. Methods Retrospectively analyze the clinical data of 103 patients with P P GL between Ap rtl 2011 and Feb ruary 2016. All objects were divided into small P P G L group （diameter being 3 cm o r less） and larger PPGL group （〉 3 cm in diameter） according to the maximum diameter measured by preoperative CT. The differences of clinical manifestations, plasma free metanephrines （MNs）, features of image and pathology and clinical conditions in perioperative period were compared between the two groups. Results Tolally 21 cases were enrolled in small PPGL group, the average diameter of tumors being 2.39±0.67 cm. The incidence of headache, palpitation, sweating, other clinical manifestations of PPGL and having two or more symptoms of the classic triad in small PPGL were lower than those in large PPGL. The proportion of no symptoms of the triad in small PPGL group was higher than that in larger PPGL group. But all the differences were not significant. The average of metanephrine in small PPGL was significantly lower than that in larger PPGL, so was normetanephrine. The incidence of slightly elevated or normal plasma free MNs in small PPGL was higher than that in larger PPGL. There were some similar features in CT scan between the two groups. All of PPGL exhibited an attenuation of more than 10 Hu on plain CT scan and most of PPGL had an attenuation value of more than 20 Hu. Obvious enhancement was very common in both groups on enhanced CT. The preoperative misdiagnosis rate was significantly higher in small PPGL group. Operation time and intraoperative blood loss were significantly less in small PPGL group. Conclusion Small PPGLs are not rare. The clinical manifestations of them are ofen atypical. Many patients with the disease exhibit modestly elevated or normal levels of biochemical marker. Anatomical and functional imaging are use

关 键 词：嗜铬细胞瘤 副神经节瘤 临床表现 甲氧基肾上腺素类物质 

分 类 号：R58[医药卫生—内分泌]