CAJ | 학술논문

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(χ2=6.604,P=0.01),与肿瘤大小(χ2=3.208,P=0.201)、功能状态(χ2=0.121,P=0.728)、肿瘤局部情况(χ2=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.
목적 탐토복막후부신경절류적림상특정급예후인소,제고복막후부신경절류적진치수평.방법 회고성분석본원1999년11월지2009년3월수술치료복막후부신경절류19례환자적림상표현、종류공능、술중소견、수술방식、병리화영상학자료,이급술후생존시간.결과 (1)본조19례복막후부신경절류남녀비례위1.375,중위년령50세,최상견적림상표현위복부종괴(9/19,47%),술전CT진단오진솔고(89%);(2)종류호발우하강정맥급복주동맥주(9/19,47%),평균최대직경위8.6 cm(3～23 cm),58%(11/19)종괴포막완정,42%(8/19)종괴여주위장기긴첩혹긴밀점련,26%(5/19)수작비린수루기관절제;(3)본조63%(12/19)종류위공능성,술전표현고혈압점67%(8/12),술중혈압파동극렬점33%(4/12);(4)16례18개종괴행면역조화염색,89%(16/18)Chg-A(+),67%(12/18)S-100(+),증식세포핵항원(PCNA)정불동정도표체(0%～48%),Ki-67여P53균지재악성병례양성표체(분별위20%,34%);(5)본조병례수방1～107개월,중위수방40개월,Kaplan-Meier급Log-Rank생존분석현시:전조총체적5년생존솔위77%,생존솔여종류출현원처전이상관(χ2=6.604,P=0.01),여종류대소(χ2=3.208,P=0.201)、공능상태(χ2=0.121,P=0.728)、종류국부정황(χ2=0.036,P=0.849)상관불현저.결론 조기진단복막후부신경절류교위곤난;환자생존예후여전이상관,응강조종생수방;술후병리면역조화검사대판단종류성질화예후비상필요.
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 (χ2=6.604,P =0.01).But it was not dependent on tumor diameter (χ2=3.208,P=0.201),the secreting function of tumor (χ2=0.121,P =0.728) and the status of tumor margins (χ2=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.