1乳腺型肌纤维母细胞瘤.ppt

Mammary-typemyofibroblastoma,乳腺型肌纤维母细胞瘤,一、Definition定义Abenignmesenchymalneoplasm良性间叶性肿瘤composedofspindle-shapedcellswithfeaturesofmyofibroblasts,由梭形有肌纤维母细胞特征的细胞构成的embeddinastromathatcontainscoarsebandsofhyalinizedcollagen间质内含有玻璃样变的粗大胶原纤维条带,andconspicuousmastcells可见大量的肥大细胞andadmixedwithavariableamountofadiposetissue.并伴有数量不等的脂肪组织。thetumourishistologicallyidentiacaltomyofibroblastomaofbreast.其组织学特点与乳腺的肌纤维母细胞瘤相似。,二、Epidemiology流行病学Lesionhaveariseninadultswithanagerangeof35to67years(median55.5years)andamalepredilection(8males,2females).发病年龄见于35-67成年人（平均55.5）男性多见（8例男性，2例女性）Theextrammarylocationofsomemyofibroblastomashasonlyrecentlybeendefinedwhen10caseswerereported.乳腺外的肌纤维母细胞瘤直到最近才有10例报告。,Therefore,conclusionsrelatedtoepidemiologycouldalterwithincreasedtumourrecognition因此随着对肿瘤认识的增加，流行病学方面也会发生改变。,三、sitesofinvolvement受累部位Themostcommonlocationofmammary-typemyofibroblastomaistheinguinal/groinarea.乳腺型肌纤维母细胞瘤最常见于会阴/腹股沟区域。Otherreportedsitesincludeabdominalwall，buttock,backandvaginalwall.其他部位有腹壁，臀部，背部和阴道壁。,Lesionsarisemostcommonlyinsubcutaneoustissue.最常见的发病部位是皮下组织。However,caseshavearisendeeptoabdominalwallmuscle，intheposteriorvaginalwallandinaparatesticularlocation.但也可位于腹壁肌肉，阴道后壁和睾丸旁。Thereisanapparentpredilectionformyofibroblastomastoarisealongtheputativeanatomic“milk-line”thatextendsfromaxillatomedialgroin.肌纤维母细胞瘤明显好发于从腋窝至腹股沟中部的“白线”,四、Clinicalfeatures临床特点Thetumoursgenerallypresentaseitherpainlessmassesorincidentallesionsthataredetectedduringsurgicalproceduressuchasinguinalherniarepair.多为无痛性的肿块或由外科进行腹股沟疝修补术时偶然发现隐匿肿块。Occasionallesionsaretenderorpainful偶尔病变区触痛或疼痛。,Tumourshavebeendescribedtobepresentforuptoayearbeforeclinicalpresentation.肿瘤就诊前可能已经存在达1年之久Therearenoimagingdata无影像学资料数据,五、Aetiology病因学Unknown病因不明Ithasbeenpostulatedthatmyofibroblastomasarisinginthebreastmayberelatedtoapatientshormonalstatus,hesettingofgynaecomastiaandanti-androgentherapy.据推测发生于乳腺的肌纤维母细胞瘤与患者的激素水平有关.这种情况多见于老年男性。如：男性乳腺发育和抗雄激素治疗的患者。,Mammary-typemyofibroblastomaofsofttissuearisesmostcommonlyinolderadultmales.软组织的乳腺型肌纤维母细胞瘤常见于老年男性Theapparentpredilectionfororiginofmyofibroblastomasalongaputativemilk-linesugeststhepossibelexistenceofhormonally-responsivemesenchymaltissue明显好发于从腋窝至腹股沟中部的解剖学“白线”区域。暗示这一区域存在对激素敏感的间叶组织细胞。,六、Macroscopy肉眼大体观Reportedlesionsrangedinsizefrom2to13cm(median5.8cm).已报告病例的肿块直径从到13cm，平均5.8cmThetumourarewellcircumsribedandfirm.界清，质硬Thecolourcanbevariable(white,pink,tanorbrown)色泽多变，可为白色，粉红色，黝黑色棕褐色,Thecutsurfacemaybewhorledornodular.切面可呈漩涡状或结节样Soft“mucoid”-appearingareasreflectingmyxoidchangewerepresentinonecase一例病变质软、粘液变。,七、Histopathology组织学Tumoursareunecapsulatedbutwellcircumscribed.肿瘤无包膜，但是分界清楚Theyarecomposedofanadmixtureofspindlecellsandadiposetissue由梭形细胞与脂肪细胞混合构成Andaremorphologicallyidenticaltomammarymyofibroblastoma形态类似于乳腺肌纤维母细胞瘤,Thespindlecellshistologicallyresemblemyofibroblasts梭形细胞特点类似于肌纤维母细胞andarecharacterizedbyovaltotaperednuclei其核形从梭形至卵圆形withfinelydispersedchromatin，smallnucleoli核内染色质均匀散布，常见小核仁,Eosinophilictoamphophiliccytoplasm胞浆从嗜伊红到嗜双色性Andpoorlydefinedcytoplasmicborders胞质界限不清Thespindlecellsarefrequentlywavyincontour梭形细胞的轮廓常呈波浪样andgenerallyarearrangedinvariablysizedfascicles一般排列成不同大小的束状,Thestromaiscollegenouswithbroadbandsofcoarsehyalinizedcollagenthatoftenadoptazig-zagpattern基质为边缘粗糙的，带状的，玻璃样变的胶原带，胶原带，常形成Z字锯齿样图案Stromalmastcellsareusuallynumerous基质内常有大量肥大细胞Epithelioidchangeofthelesioncells病变细胞常呈上皮样变Andfocalnuclearatypiawithenlargednucleiandmultinucleationhavebeendescribed.局灶上皮样细胞核不典型性，伴核增大，多核,Suchmorphologicvariationiswellrecongnizedinmayofibroblastomaofbreast以上形态学的变化在乳腺肌纤维母细胞瘤已有清楚的认识。,Thebloodvesselsinmyofibroblastomaaregenerallynotconspicuous，beingsmall肌纤维母细胞瘤肿块内的血管不明显，常为小血管Andcommonlyhavingaperivascularlymphocyticinflitrate并且，血管周围有淋巴细胞渗出。,incontrasttotheprominentmediumtolargevesselswithmarkedlyhyalinizedwallsthatarecharacteristiccellularangiofibroma与之不同的是，细胞性血管纤维瘤内有醒目的中等血管和大血管,并且血管壁显著地玻璃样变Orthelargebranching“haemangiopericytomatous”bloodvesselsthatareseeninlipomatoushaemangiopericytoma而脂肪瘤样血管外周细胞瘤组织内有大的分支状“血管外周细胞瘤样”血管。twopotentialmorphologicmimics.以上二种肿瘤在形态学上与肌纤维母细胞瘤有相似之处。,八、Immunophenotype免疫表型Asischaracteristicofthebreastcounterpart，thetypicalimmunphenotypeofextramammarymyofibroblastomaisdiffuseco-expressionbythespindlecellsofdesminandCD34.乳腺外的肌纤维母细胞瘤的免疫表型对应于乳腺肌纤维母细胞瘤（双阴性），乳腺外的肌纤维母细胞瘤的梭形细胞弥漫性，双表达desmin（肌间线蛋白）和CD34Expressionofsmoothmuscleactinisseeninathirdofcase1/3的梭形细胞表达SMA(平滑肌动蛋白)。,九、Prognosticfactors预后Alltumourshavefollowedabenigncoursefollowingmar