软组织肿瘤病理学病例讨论四

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1、Case4EX05-146(04/S10052).PresentedbyQHFan.History.Femaleaged12.Subcutaneouslesionextendingdowntoboneonthedorsumoftherightmiddlefinger.Diangosis?DiagnosisPlexiformfibrohistiocytictumor,subcutisextendingdowntobone,dorsumofrightmiddlefinger.Plexiformfibrohistiocytictumor,atumorfirstdescribedbyD

2、rs.EnzingerandZhangin1988whentheyreportedof65casesfromthefilesoftheArmedForcesInstituteofPathology.Itoccurschieflyinchildrenandyoungadults.Themostcommonlocationwastheupperextremity(63.1%),especiallytheregionsofshoulderandforearm.Themedianageoftheir65patientswas14.5years;two-thirds(67.7%)were

3、youngerthan20years;therewere46femalesandonly19males.Thetumorspresentedasaslow-growing,poorlydemarcateddermalorsubcutaneousmassthatrarelyexceeded3cmingreatestdiameter.Histologically,lesionswerecharacterizedbyamultinodularorplexiformproliferationofhistiocyte-andfibroblast-likecellsassociatedwi

4、thmultinucleategiantcells,morphologicfeaturesreminiscentofbothafibroushistiocytomaandafibromatosis.Thedifferentialdiagnosisincludes:1.Cutaneousfibroushistiocytoma2.Plexiformneurofibroma,3.Fibromatosis,4.BenignandmalignantgiantcelltumorsPrognosisTwentyoftheir32cases(Good:62.5%)withfollow-upin

5、formationwerealiveandwellafterlocalexcision,butthetumorrecurredin12cases(Recurred:37.5%).Intwopatientswithrecurrence,thedisease(Metastasized:6%)metastasizedtoregionallymphnodes9and36monthsaftertheinitialexcision,respectively.EnzingerandZhang’soriginalfindingshavebeenconfirmedinsubsequentpape

6、rs.TreatmentPlexiformfibrohistiocytictumorisalowgradesofttissuetumorofintermediatemalignancywhichfrequentlyrecurslocallybutrarelymetastasizes.Wideexcisionisbelievedtobethebesttreatment.Case5(SlideSeminarCase04BX5833)Presentedby:Dr.Wai-KuenNg讲者：吴伟权医生DivisionofAnatomicalPathology,Departmentof

7、Pathology&ClinicalBiochemistry,QueenMaryHospital,HongKongSAR,China香港玛丽医院病理及临床生化学部门ClinicalHistory病历10-year-oldboywithtorticollis&bilateralupperlimbweaknessfor3months患者男，10岁。因斜颈及双手无力达3个月而就诊。SpineX-ray:OsteolyticlesionoverC2vertebralbody脊柱X线：有一溶骨性病变在