腹膜后副神经节瘤19例临床诊治分析  

Surgical treatment and prognostic analysis of retroperitoneal paragangliomas： a study of 19 cases

作　　者：季晓克[1] 曾其强[1] 吴秀玲[2] 黄颖鹏[1] 周蒙滔[1] 黄卡特[2] 余正平[1] 韩少良[1] 张启瑜[1] 

机构地区：[1]温州医学院附属第一医院普通外科,325000 [2]温州医学院附属第一医院病理科,325000

出　　处：《中华医学杂志》2010年第34期2385-2388,共4页National Medical Journal of China

摘　　要：目的 探讨腹膜后副神经节瘤的临床特征及预后因素,提高腹膜后副神经节瘤的诊治水平.方法 回顾性分析本院1999年11月至2009年3月手术治疗腹膜后副神经节瘤19例患者的临床表现、肿瘤功能、术中所见、手术方式、病理和影像学资料,以及术后生存时间.结果 （1）本组19例腹膜后副神经节瘤男女比例为1.375,中位年龄50岁,最常见的临床表现为腹部肿块（9/19,47%）,术前CT诊断误诊率高（89%）;（2）肿瘤好发于下腔静脉及腹主动脉周（9/19,47%）,平均最大直径为8.6 cm（3～23 cm）,58%（11/19）肿块包膜完整,42%（8/19）肿块与周围脏器紧贴或紧密粘连,26%（5/19）需作毗邻受累器官切除;（3）本组63%（12/19）肿瘤为功能性,术前表现高血压占67%（8/12）,术中血压波动剧烈占33%（4/12）;（4）16例18个肿块行免疫组化染色,89%（16/18）Chg-A（＋）,67%（12/18）S-100（＋）,增殖细胞核抗原（PCNA）呈不同程度表达（0%～48%）,Ki-67与P53均只在恶性病例阳性表达（分别为20%,34%）;（5）本组病例随访1～107个月,中位随访40个月,Kaplan-Meier及Log-Rank生存分析显示：全组总体的5年生存率为77%,生存率与肿瘤出现远处转移相关（χ^2=6.604,P=0.01）,与肿瘤大小（χ^2=3.208,P=0.201）、功能状态（χ^2=0.121,P=0.728）、肿瘤局部情况（χ^2=0.036,P=0.849）相关不显著.结论 早期诊断腹膜后副神经节瘤较为困难;患者生存预后与转移相关,应强调终生随访;术后病理免疫组化检查对判断肿瘤性质和预后非常必要.Objective To investigate the clinical characteristics,surgical treatment and prognostic analysis of retroperitoneal paragangliomas and to enhance the diagnostic and therapeutic levels of retroperitoneal paragangliomas.Methods The clinical data of all patients undergoing paraganglioma resection at our department from November 1999 to March 2009 were retrospectively analyzed.The parameters included clinical manifestations,tumor function,surgical findings,operative approach,tumor pathology,imaging study and post-operative survival time.Results （1） The ratio of male to female was 19,47%）.And the preoperative CT misdiagnosis rate was high （89%）.（2） The most common tumor location was periaortic and pericaval （9/19,47%）.The average maximal diameter of tumors was 8.6 cm.58% （11/19） tumors had integral peplos,42% （8/19） adhered to adjacent organs and 26% （5/19）required adjacent organ resection.（3） The rate of functional tumor was 63% （12/19）.Preoperative and intra-operative hypertension occurred in 67% （8/12） and 33% （4/12） respectively.（4）Immunohistochemical staining was performed in 18 tumors of 16 patients.Among all tumors,89% （16/18）showed positive immunoreactivity for chromogranin and 67% （12/18） for S-100.PCNA staining showed different proliferative activities （0%-48% positive）.Only malignant tumors showed positive immunoreactivity for Ki-67 staining and P53 staining （20% ＆ 34% respectively）.（5） The overall 5-year survival was 77%.Survival was significantly worse after metastasis （χ^2=6.604,P =0.01）.But it was not dependent on tumor diameter （χ^2=3.208,P=0.201）,the secreting function of tumor （χ^2=0.121,P =0.728） and the status of tumor margins （χ^2=0.036,P=0.849）.Conclusion It is difficult to make an early diagnosis of retroperitoneal paragangliomas.Survival is significantly worse after metastasis.Lifelong follow-up for recurrence is important.And it is absolutely essential to perform immunohistochemical staining for tumors.

关 键 词：腹膜后肿瘤 副神经节瘤 外科手术 生存率 

分 类 号：R735.4[医药卫生—肿瘤]