临床与实验病理学杂志JClinExpPathol2010Jan；26(1)·77·肾上腺外副神经节瘤临床病理分析胡维维1，王辉1，陶金华1，李广民1，赖日权2摘要：目的探讨肾上腺外副神经节瘤临床病理学特征、分类、良恶性组织学判断标准及鉴别诊断。方法对2l例肾上腺外(副交感神经和交感神经)副神经节瘤进行光镜及免疫组化观察，同时跟踪随访并复习相关文献。结果肾上腺夕bSJJ神经节瘤2l例，男性lO例、女性11例，年龄9—8l岁，平均年龄46．5岁。肿瘤位于腹膜后lO例，头颈部7例，纵隔、膀胱、精索及肾脏各1例。有功能者3例，无功能者18例。组织学上肿瘤主要由主细胞和支持细胞两种细胞组成，主细胞排列成巢状、柬状、腺泡状或实体样结构，周围被支持细胞部分或完全包绕。1例诊断为恶性，瘤细胞异型性显著，核分裂象多见，并见灶性或融合性坏死和血管侵犯，伴局部淋巴结转移，余20例诊断为良性。免疫组化肿瘤细胞表达NSE、cgA、Syn、NF，而CK、EMA和SMA阴性；支持细胞S-100蛋白阳性。I例恶性副神经节瘤仅NSE阳性，CgA弱阳性。随访18例，其中I例1年后因多脏器转移而死亡，另有1例组织学诊断为良性者3年后肿物复发并侵犯邻近器官。结论肾上腺外副神经节瘤组织形态学改变与其生物学行为不一，临床上应长期随访。同时应与形态相似的肿瘤鉴别。关键词：副神经节瘤／病理学；腹膜后肿瘤；鉴别诊断中图分类号：R730．264；R735．4文献标识码：A文章编号：1001-7399(2010)01—0077—04Extra-adrenalparagangfioma：aclinicopathologicstudyof21casesandliteraturereviewHUWei·weil，WANGHuil，TAOJin—hual，LIGuang-rainl，1．JdRi—quan2(1DepartmentofPathology，彬池甜FoshanHospitalofSunYat一．sell．Umversi秒，Foshan528000，China；2DepartmentofPathology，GeneralHospitalofGuangzhouCommand，Guangzhou510010，China)Abstract：PurposeTostudytheclinicopathologicfeatllre，classification，benignormalignanthistologicalfeaturesanddifferentialdi-agnesisofextra-adrenalparaganghoma．MethodsWeperformedaretrospectiveanalysisof21casc％ofextra-adrenal(sympatheticandparasympathetic)paraganglioma．Histological，immunohistochemicalfindings，andfollow-updatawerereportedwithreviewoftherela-fiveliteratures．ResultsTherewere10maleand11femalepatientsagedfrom9to81years(mean46．5years)．Tentumorsoccurredintheretroperitoneum，sevenintheneck，andonecaseinthemediastinum，urinarybladder，spermaticcordandkidney，respective-ly．Therewere3casesoffunctionaland18casesofnon·functionalparagangliomas．Histologically．thetumorwagcomposedofchiefceilsandsustentacularcells．Thechiefcellswerearrangedinnests，alveolar-likeandstereo-likestructures．TheyweresurroundedbyflUS-tentacularcellspartlyorcompletely．Therewasonemalignantcase，inwhichtumorcellshadsignificantcellatypiaandnuclearmitosis，andfocalordiffusenecrosis，regionallymphnodemetastasisandbloodvesselinvolvementwerealsoobserved。lmmtmohistochemicalin-vestigationsrevealedchiefceilswereintenselypositiveforneurocndocrinemarkerssuchasNSE，CgA，SynandNF，butnegativeforCK，EMAandSMA．Sustentacu]arcellswerepositiveforS-100protein．ThemalignantcellswereonlypositiveforNSEandweaklypos-itiveforCgA．Eighteencases慨followedup．Onepatientdevelopedtomulti—organmetastasisanddied．OnepatientWasdiagnoseda8benignextra-adrenalparagangliomathatrecurredandinvolvedadjacentorgansafter3years．ConclusionsItisgenerallyconsideredthatmalignantpotential．10calinvasivenessanddistantmetastasisa糟notcorrelatedwiththehistologicalappearancesoftheextra—adre-nal耻ml乎mgliolna．So，furtherfollow—upwillbeneeded．Itisnecessarytoemphasizethedifferentialdiagnosesofextra-adrenalpal醒an—OiomafromotherpleiomorphictIImors．Keywords：paragangiioma／...
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