腫瘤學(xué)-血管肉瘤

CasePresentation-Sasha

案例陳述PresentingComplaint(April1)

現(xiàn)病史（4月1日）Palemucusmembranes粘膜蒼白AcuteWeakness急性虛弱Signalment癥狀Sashaisa14yearoldFsmix/siberianhuskydog14歲母性混種/西伯利亞哈士奇History病史Foundinthebackyardweakandunabletostand.在后腕發(fā)現(xiàn)虛弱不能站立Saha-ontheground,weakandataxicuponrising.躺在地上，虛弱，共濟(jì)失調(diào)Sashapresentednonambulatorywithpalemucusmembranes.表現(xiàn)出粘膜蒼白不能行走PhysicalExam(Abnormalities)體格檢查（異常）GEN全身:Quiet,unresponsive.全身，無反應(yīng)EEN眼、耳、鼻、咽喉:Earscoldtothetouch耳朵冰冷MS:Nonambulatory無法行走CV心血管:HR心率=120,MM粘膜=pale,CRT血凝時間=3s.Weakpulses,nopulsedeficits.脈搏虛弱，無脈搏不條RR呼吸速率:RR呼吸速率=30.Quietbreathsoundsdorsally.Breathsoundsauscultableventrally.背側(cè)呼吸不清，腹側(cè)呼吸尚可聽診NE神經(jīng)末稍:Mioticpupils,noPLRdirectorconsensual. 瞳孔縮小或沒有直接的瞳孔光反射或交感性瞳孔光反射

GI/GU胃腸/泌尿生殖道:Splenicmasspalpated.觸及脾有團(tuán)塊ProblemList問題列表Shock休克Palemucusmembranes粘膜蒼白Splenicmass.脾臟腫塊EmergencyTreatment緊急治療LRS-bolused.乳酸林格氏溶液－快速注入Clinicallyimproved,BAR.Abletoriseandwalkcomfortably.

癥狀改善，四肢可站起，可輕松行走Reasonfortheshock-hypovolemiasecondarytointernalhemorrhage.

休克原因－繼發(fā)于內(nèi)出血的血容量過低。DiagnosticPlan診斷計劃CBC血常規(guī)Chemistrypanel生化Abdominalultrasound腹部超聲Abdominocentesis腹腔穿刺術(shù)Thoracicradiographs胸部X光Bloodwork血液CBC:HCT=33.6%,rareschistocytes.少量的裂紅細(xì)胞WBC=23,600,neut=20,532,bands=236.PostFluids液體治療后:PCV=23%,TP=4.8.

Chem:Na=145,K=4.3,Cl=104.Ca=9.2.Crea=0.9,BUN=38.Alb=2.6,Glob=2.9.DICpanel:PT凝血酶原時間,PTT部分凝血致活酶時間

WNL（正常）,Fibrinogen=265.纖維蛋白原AbdominalUltrasound腹部超聲revealedmildtomoderatehemo

abdomenandalargesplenicmass.顯示出輕度到中度的血腹水，脾有大腫塊AbdominalRadiographs腹部X光Abdominocentesis腹腔穿刺peritonealfluid:hemorrhagicexudate

腹水：血性滲出物PCV-29%,TPof5.7.(Peripheral外周血

PCV=23%(post-fluids液體治療后),PeripheralTP=4.8(post)).Thoracicradiographs胸腔X光Thoracicradiographswereunremarkable.

胸腔X光無異常Assessment估計Internalhemorrhage.內(nèi)出血Largesplenicmass.脾臟有大腫塊Assessment-Differentials

鑒別診斷a血管肉瘤Hemangioma血管瘤Hematoma血腫Schistocytes-supportiveofachangeinmicrovasculature 裂細(xì)胞－說明有微脈管系統(tǒng)改變Assessment估計Postfluidtherapyvalues(PCV,TP)-moderateanemiaandhypoproteinemia.

輸液治療后－中度貧血，低蛋白血Leukogram-stressedanimal.

白細(xì)胞像－應(yīng)激動物ChemistrypanelandU/A-hemorrhageandpossibledehydration

生化和尿檢－出血也可能脫水

Plan計劃Duetonootherabnormalitiesobservedintheabdomenandwithnometsseenonthoracicradssplenectomyisindicated.

腹腔內(nèi)無觀察到其他異常，胸部X光無異物，考慮脾切除術(shù)。Sashawastakentosurgeryandasplenectomywasperformed.進(jìn)行脾切除術(shù)Splenica

脾血管肉瘤PostOp手術(shù)后ComplicationsseenincludeVPCs,paincontrol,andbleeding.

并發(fā)癥包括心室期外收縮，疼痛控制和出血Sashawasdischarged3daysaftersurgerywithinstructionstoreturntotheVMTHforsutureremovalandchemotherapy. Sasha在手術(shù)后3天按指示拆線和化療，出院SashaSashawastreatedwithchemotherapy-6treatmentswithadriamycin(30mg/m2each)

用阿霉素進(jìn)行6個療程的化療Noevidenceofthoracicmetastasisorabdominalmetastasiswerenotedatthelasttreatment.

無明顯證據(jù)顯示在最后一次治療有任何胸腔轉(zhuǎn)移和腹腔轉(zhuǎn)移。SashaInNov.shereturnedforarecheckwithpalemucusmembranes

在11月份，再次出現(xiàn)粘膜蒼白AbdominalUltrasoundimagedmetastasisintheliverandatthepedicleofthespleen. 腹部超聲顯示肝臟和脾臟根部有轉(zhuǎn)移。ShewaseuthanizedinNovember. 11月份實施安樂死。a血管肉瘤amalignanttumorofendothelialcells

一種惡性內(nèi)皮細(xì)胞腫瘤Epidemiology流行病學(xué)Increasedincidence-GermanShepherds

增加發(fā)病率－德國牧羊犬Otherbreedsaffected-Pointer,Boxer,LabradorRetriever,GoldenRetriever,EnglishSetter,GreatDane,Poodle,SiberianHusky.其他易發(fā)品種－波達(dá)，拳師犬，拉布拉多，金毛，英國塞特，大丹，貴賓，西伯利亞哈士奇Age-mean-8-10years.平均年齡8－10歲Nosexpredilection(conflictingreports)無性別差異（與報道沖突）Noetiologicagenthasbeenidentified病因尚未確定Epidemiology(cont)流行病學(xué)Cutaneousa-morecommoninfemales.Dogswithlightlyhaired,poorlypigmentedskinarepredisposed:whippets,salukis,bloodhounds,andEnglishpointers.Maybesunlightinduced. 皮膚血管肉瘤在雌性中更常見。毛少，皮膚色素少的犬易發(fā)，威伯賽犬，薩盧基犬，巡回獵犬和英國波達(dá)?；蛟S是光照導(dǎo)致。Locationandbehaviour

位置和表現(xiàn)Primary-Spleen,Cardiac(rightatrialappendageorrightatrium),liver,lungs,kidney,skin(SQ),bone,oralcavity,muscle,urinarybladder,peritoneum.

初級－脾，心臟（右心耳或右心房），肝，肺，腎，皮膚，骨，口腔，肌肉，膀胱，腹膜Locationandbehaviour

區(qū)域和表現(xiàn)Metastasis:mostoften-liverandlungs.Inaddition-kidney,skeletalmuscle,peritoneum,omentum,lymphnodes,mesentery,brain,spinalcord,urinarybladder,adrenalgland,diaphragm.

轉(zhuǎn)移：最常見－肝，肺。其他－腎，骨骼肌，腹膜，網(wǎng)膜，淋巴結(jié)，腸系膜，大腦，脊髓，膀胱，腎上腺，隔膜Duetowidespreadmetastasisidentificationoftheprimarytumormaybedifficult.

由于廣泛轉(zhuǎn)移，故原發(fā)腫瘤的診斷較為困難。ClinicalPresentation臨床表現(xiàn)Dependsontheprimarytumorsiteandtheorgansaffected.根據(jù)原發(fā)腫瘤位置和受影響器官Progressiveorintermittentlethargy

進(jìn)行性或間斷性昏睡Anorexia厭食Collapse虛脫WeightLoss體重減輕ExerciseIntolerance運動不耐受ClinicalPresentation臨床表現(xiàn)Dependsontheprimarytumorsiteandtheorgansaffected.根據(jù)原發(fā)腫瘤位置和受影響器官Dyspnea呼吸困難Pallor顏色蒼白AcutehypovolemicShockcanbeseenduetoruptureofthetumor由于腫瘤破裂，可見急性的低血容量性休克Splenica

脾血管肉瘤palpableabdominalmass

可在腹部觸及到腫塊abdominaldistention腹部鼓脹hemoperitoneum(fluidwaveonpalpation).腹腔積血（觸及有液體流動）Cardiaca

心血管肉瘤syncope昏厥ataxia共濟(jì)失調(diào)cyanosis發(fā)紺cardiacarrythmias心律失常signsofbloodflowobstruction:peripheraledema,hepatomegaly,dyspneaandpleuraleffusion.

血液流動障礙：外周性水腫，肝腫大，呼吸困難，胸腔積液aofthebone

骨血管肉瘤Clinicalsignscharacteristicofmalignantbonetumors-

惡性骨腫瘤的臨床癥狀lameness跛pain疼痛softtissue swelling

軟組織腫脹Cutaneousa

皮膚血管肉瘤Mayhaveconcurrenthemangiomas. 可能同時有血管瘤as-血管肉瘤Dermis>>subcutis 真皮>>皮下組織Predelictionforventralabdominalskin 腹部皮膚易發(fā)Overlyingepidermisisthickenedandulcerated. 上層皮膚變厚和成潰瘍LaboratoryFindings實驗室診斷HematologicDisorders:血液學(xué)失調(diào)Normocytic,normochromic,regenerativeanemia(ifearly,anonregenerativeanemia)withincreasedreticulocytes,nucleatedrbc’s,polychromasia,poikilocytosis,anisocytosis,schistocytes.

正常紅血球，正常色素，再生性貧血（如果是早期，非再生性貧血），網(wǎng)狀細(xì)胞，有核紅細(xì)胞，多染性紅細(xì)胞，異形紅細(xì)胞癥，紅血球大小不一，裂紅細(xì)胞增多Neutrophilicleukocytosis嗜中性白細(xì)胞增多癥HemostaticAbnormalities:

凝血異常DIC-lowplateletcounts,highlevelsoffibrindegradation,prolongedPTandPTT DIC彌散性血管內(nèi)凝血－血小板數(shù)量低，纖維蛋白降解快，PT和PPT延長StagingandDiagnosis分階段和診斷Minimumdatabase:最小數(shù)據(jù)

CBC血常規(guī)

ChemProfile生化

Urinalysis尿檢StagingandDiagnosis分階段和診斷Radiographs:X光pulmonarymetastasis (characterizedbyawidelydisseminatednodularpattern)

肺部轉(zhuǎn)移（表現(xiàn)出廣泛彌散的小結(jié)節(jié)）StagingandDiagnosis分階段和診斷Radiographs:X光abdomen(alargeintraabdominalmassofsofttissuedensityinthemid-abdomen).

腹部（在腹腔中部有一塊軟組織密度的大的團(tuán)塊）StagingandDiagnosis

分階段和診斷Ultrasonography:enlargedspleen,peritonealeffusion,sitesofabdominalmetastasis

超聲：脾腫大，腹膜滲出，腹腔內(nèi)轉(zhuǎn)移的位置StagingandDiagnosis分階段和診斷Echocardiogram:examinerightatrialappendage,pericardialeffusion 超聲心動圖檢查：右心耳、心包滲出Bonea-bonelysiswithminimalperiostealreaction 骨血管肉瘤－伴有輕微骨膜反應(yīng)的骨溶解Diagnosis診斷Definitivediagnosisrequireshistopathology需組織病例學(xué)確診Cytologyisproblematicduetotheheterogenousnatureofthetumor-hematomas,fibroticareas,extramedullaryhematopoiesis. 細(xì)胞學(xué)無法判定，由于腫瘤血腫異源的本質(zhì)，纖維變性的區(qū)域，骨髓外的造血存在Prognosis-SplenicHMS

診斷－脾HMS高遷移率綜合征Staging階段Stage1-confinedtothespleenwithnoevidenceofmetastasis限制于脾內(nèi)，并且脾內(nèi)無明顯轉(zhuǎn)移Stage2-mayhavearupturedspleen,withorwithoutregionallymphnodeinvlovement

或許脾有破裂，同時有或無局部淋巴受累Stage3-largeinvasivetumorswithdistantmetastasis伴隨遠(yuǎn)距離轉(zhuǎn)移的較大的侵入性腫瘤Treatment-SplenicHMS

治療－脾高遷移率綜合征Surgery-treatmentofchoice.Relievesabdominaldistentionprovidespalliationbystoppingbleeding手術(shù)－選擇治療方法。 減少出血來減輕腹部腫脹Doeslittletoprolongsurvival.

對延長生命效果甚微。MedianSurvivalforsurgeryalone-19-86days

單純手術(shù)平均存活19－86天。Treatment-SplenicHMS

治療－脾高遷移率綜合征Chemotherapy-化療Doxorubicin/Dox&cyclophosphamide.

阿霉素和環(huán)磷酰胺Averagesurvivaltimes–6months.平均存活時間6個月Conflictingreportaboutstageandprognosis-

關(guān)于階段和預(yù)后的報道有沖突Sorenmo(1993)Stage1-250d,Stage2-186,Stage3-87(NS)Sorenmo(2004)-Stage1-257d,Stage2-210,Stage3-107(difbetween1,2and3-sig)Others-nodifference其他無不同Prognosis-CardiacHMS

診斷－心臟高遷移率綜合征Dieorareeuthanizedatorshortlyafterdiagnosisduetohighrateofmetastasis.

確診或確診后不久，由于其高轉(zhuǎn)移性，死亡或者安樂死Treatment-CardiacHMS

治療－心臟高遷移率綜合征Surgery-onlypossibleifthetumorisconfinedtotherightauricle.手術(shù)－僅僅腫瘤局限在右心耳可行Meansurvivaltimesinonestudy-4months.Alldogsdevelopeddisseminatedmetastaticdisease.一項研究中認(rèn)為平均存活為4個月，所有犬都彌漫性轉(zhuǎn)移。Chemotherapy-reportsarerare化療－很少報道Treatment-CardiacHMS

治療－心臟高遷移率綜合征Weisseetal-JAVMA-2005-SurvivaltimesindogswithrightatrialHMStreatedbymeansofsurgicalresectionwithorwithoutadjuvantchemotherapy-23cases

右心房的HMS－通過手術(shù)切除配合化療或不化療-23個病例Staging-nonehadevidenceofpulmonarymets,9dogshadeithernodulesormassesinthespleen(imaging)無明顯肺部轉(zhuǎn)移，9只犬在脾有小結(jié)節(jié)或腫塊19-pericardialeffusion,7-cardiactamponade19只心包滲出，7只心臟阻塞11-cardiacmassesseenonecho:6-rightatrial,5-rightatrialappendagemass11只在超聲心動圖上顯示心臟有團(tuán)塊，6只在右心房，5只在右心耳Pericardialcentesiswasperformedin21dogs-5dogs-submittedforcytology-nondiagnostic.21只犬在心包有發(fā)現(xiàn)－5只委托進(jìn)行細(xì)胞學(xué)分析，無診斷。Treatment-CardiacHMS

治療－心臟高遷移率綜合征8dogs-rightatrialmassresection-43days8只犬－右心房團(tuán)塊切除－存活43天2dogs-splenectomy(histowasWNL組織學(xué)正常)2只脾切除15dogs-rightatrialappendagemassresection-118days 15只犬右心耳團(tuán)塊切除－存活118天Post-opcomplications-within7daysofsurgery-12dogs-pain,cardiacarrythmias,wounddehiscence,infection,pneumonia…

手術(shù)后，并發(fā)癥－－手術(shù)后7天內(nèi)，12只犬疼痛，心律失常，傷口開裂，感染，肺炎...8receivedchemotherapyDox/Dox&vinc&cyc