上皮样型恶性周围神经鞘瘤

Epithelioidvariantofmalignantperipheralnervesheathtumor

我们拟探讨上皮样型恶性周围神经鞘瘤形态学特征与预后的关系。１２例经光镜和免疫组化观察，其中７例作了电镜观察。组织学可分为纯上皮样型（５例）；混合型、伴有梭形细胞区（５例）和骨化型（２例）。１２例均呈Ｓ－１００蛋白阳性表达，并显示多种细胞骨架异质性，包括（１）神经细胞分化：表现为ＮＳＥ和神经微丝阳性反应，节细胞分化；（２）上皮性分化：角蛋白阳性反应，电镜见有桥粒和张力微丝束；（３）间叶性分化：波形蛋白阳性、骨化生和横纹肌样表型。该瘤的异质性与侵袭性同步，异质性明显的３例均因肺转移死于就诊后７个月至４年。

Epithelioidvariantofmalignantperipheralnervesheathtumorarisingfromdeepsofttis-sueisanuncommonneoplasm,histopathologicfeaturesofwhichareusualynothomogenous.Theaimofthisstudywasdesignedtoinvestigatetherelationbetweenmorphologicfeaturesandprognosisofthetumor.Twelvecaseswerestudiedwithlightmicroscopy,immunohistochemistry,and7caseswerestud-iedwitheletronmicroscopy.Histologicaly,therewerethreepatens:purelyepithelioid(5cases),mixedwithareasofspindlecels(5cases)andossifying(2cases).TheresultsshowedS-100proteinpositivityandmultiplecytoskeletalheterogeneitythatincluded:(1)neuronaldiferentiationwhichindicatedex-pressionforNSEandneurofilament,andanglion,cel;(2)epithelialdiferentiationwhichrevealedreac-tivityforKeratin,anddesmosomesandtonofilament-likebundlesinultrastructurealfindings;(3)mes-enchymaldiferentiationwhichmanifestedreactivityforvimentin,osteoidmetaplasis,andrhabdoidphe-notype.Thepresentstudyindicatesthattherelationbetweentheheterogeneityandtheaggressivenessofthetumorsissynchronous.Threeofthepatientswithprominentheterogenietydiedwithclinicalevi-denceoflungmetastaseswithinsevenmonthstofouryearsafterdiagnosis.