·574·恶性副神经节瘤的诊治探讨王先进1沈周俊1钟山1朱照伟1周文龙1昊瑜璇1祝宇1孙福康1邵远1张存明1[摘要]目的：提高恶性副神经节瘤(MPGL)的诊治水平。方法：回顾性分析2003年4月一2011年1月诊治的12例MPGI。患者的临床资料。高血压10例(83．3％)，腰腹部疼痛3例(25．o％)。血浆游离变肾上腺素类物质(MNs)和24h尿儿茶酚胺(cA)升高者分别为100．O％(12／12)和83．3％(10／12)。B超、CT、MRI和⋯H可碘苄胍扫描(131}MIBG)定位准确率分别为83．3％(10／12)、91．7％(11／12)、80．o％(4／5)和88．9％(8／9)。1例行放射治疗，11例行开放手术治疗。结果：原发性肿瘤9例，复发性肿瘤3例；单发性肿瘤8例，多发性肿瘤4例。根据肿瘤的中心定位：腹主动脉旁6例，下腔静脉后2例，膀胱2例，肾门2例。根据术中所见确诊恶性5例(41．7％)。病理检查11例，确诊恶性8例(72．7％)。肺转移l例，肝转移1例，骨转移1例，局部淋巴结转移3例，广泛淋巴结转移2例，2个或多个脏器同时发生转移2例。9例随访4～84个月，平均39个月；复发4例，再次手术；因多发浸润及远处转移死亡2例。结论：病理难以区分副神经节瘤的良恶性，需结合影像学检查、生化检查及术中所见。根治性肿瘤切除是治疗MPGI．最有效的方法，肿瘤复发时再次手术仍然有效；无法手术者可用放化疔控制高血压及延缓疾病进展。长期随访观察肿瘤的转移情况是确诊疑似病例的重要方法。[关键词]副神经节瘤；恶性；诊断；治疗doi：10．3969／j．issn．100卜1420．2011．08．005[中图分类号]R736．6[文献标志码]A[文章编号]1001一1420(2011)08一0574一05TheDiagnosisandTreatmentOfMalignantParagangIiomaXianjinWANGlZhoujunSHENlShanZHoNGlZhaoweiZHUlWenlongZHoUlYuxuanWUlYuZHUlFukangSUNlYuanSHA01CunmingZHANGl(1DP户口，．￡7孢P”￡o，L陪ozogy，R“i歹i咒Hospi￡口Z，S矗盘以g^口i-，inoTb行g【，陀i"ersifySf^oDZo，Medi—ci咒P，S^口，29^口i，200025，C’^i咒口)．．Abst憎ctobjective：Toimprovethediagnosisandtreatmentofmalignantparaganglioma(MPGL)．Methods：Theclinicaldataof12casesofMPGLfromApril2003toJanuary2011wereretrospectivelyanalyzed．Hyperten—sionwasobservedin10cases(83．3％)andabdominalpainwasin3cases(25．O％)．Thepositiverateofplasma-freeMNsand24一houfurineCAindiagnosingMPGLwas100．0％(12／12)and83．3％(10／12)respectively．Themainlocalizatic：ndiagnosesincludedB—Ultrasound，CT，MRIandl31I_MIBG，withthepositiveratesof83．3％(10／12)，．91．7％(11／12)，80．O％(4／5)and88．9％(8／9)respectively．onecaseunderwentradiotherapy，andtherestof11casesunderwentopensurgicaltreatment．ResuIts：Ninecaseswereprimarytumorsand3recurrenttumors；Eightcasesweresingletumorand4multipleones．Themainanatomiclocationsoftumorswereasfollows：6wereadja—centtotheabdominalaorta，2adjacenttotheinferiorvenacava，2inthebladder，2adjacenttotherenalhilum．Fivecases(41．7％)werediagnosedMPGLint}aoperativelyaccordingtotumorfeatures．Elevencasesunderwentpatho—logicalexaminationand8(72．7％)werediagnosedMPGL．Themetastaticsitesoftumorincluded1casesof1ung，1casesof“ver，1casesofbone，3casesofregionallymphnode，2casesofextensivelymphnodeand2casesofmulti—pleorganssimultaneously．Ninepatientswerefollowedupfor4to84months(mean，39months)，duringwhich4caseshadtumorrecurrenceandunderwentre_operation．Twocasesdiedfromtumorprogressionandmetastasisandtheremaininghavebeensurvivaltodate．C：onclusions：AsthebiologicalbehaviourofMPGLcannotbepredictedwithcertaintyfrompathology，thediagnosisofmalignancyisratherdifficult．Itisnecessarytocombinepathologicalexaminationwithimagingexaminations，laboratoryinspectionandintraoperativefindingsespecially．Tomedicallyinoperablepatients，radiotherapyandchemotherapymaybeusefu...
