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1、Cardiac TumorsCynthia Taub, M.D., F.A.C.C., F.A.S.E.Director, Non-invasive CardiologyDirector, Womens Heart Health CenterEinstein DivisionMontefiore Medical CenterOctober 2009DyspneaA 33 year old male presented with dyspnea on exertion for 1 weekNo angina, palpitations, syncope, fever, night sweats
2、or weight loss33 岁男性，活动后气急1周无心绞痛，心悸，晕厥，发热，盗汗，或体重下降右室流入道的梗阻肿块明显侵入右房心肌1. Right heart cath右心导管2. Mediastinoscopy纵隔镜3. Anterior Mediastinotomy前纵隔切开(Chamberlain procedure)4. Thoracotomy开胸Optimal Approach for Biopsy最佳活检方式Biopsy 1Non-specific fibrous tissue and fibrinoid material非特异性纤维组织和纤维素样物质Biopsy 2Card
3、iac arrest during anesthesia inductionBurkits LymphomaPET ScanHypermetabolic region in the anterosuperior mediastinum前上纵隔高代谢灶Intense uptake corresponding to prevascular and right paratracheal lymphadenopathy血管前、气管旁淋巴结呈高摄取No bone marrow involvement无骨髓累及TreatmentSystemic chemotherapy with Ommaya reser
4、vior/CNS prophylaxis全身化疗：Ommaya贮器/CNS 预防Surgery has limited role手术治疗价值有限Burkitts Lymphoma in HIV(-) adult1-2% of lymphoma in adults in the USFast growingUsually involves abdomenMay respond to chemotherapyAnterior Mediastinal Masses前纵隔肿块Thymoma胸腺瘤Lymphoma淋巴瘤Germ cell tumor生殖细胞肿瘤Mediastinal cysts纵隔囊肿M
5、ediastinal thyroid tissue纵隔异位甲状腺组织Parathyroid tissue甲状旁腺组织A 45 year old waitress complains of dyspnea收缩期和舒张期室间隔平坦，提示右室压力和容量负荷增加CW of TR: supra-systolic PA pressurePerivascular spaces with lighter staining matrixMultinucleatedmyxoma cellsMyxoma粘液瘤Most common primary cardiac tumor (20-30%)Most common
6、location: LA with attachment to atrial septum左房贴近房间隔Appear gelatinous and friable胶样、脆弱外观Embolic events AV valve obstruction房室瓣梗阻Carneys complex (autosomal dominant, 7% of all myomas)Recurrent, need follow-upMyxoma粘液瘤Most common primary cardiac tumor (20-30%)Most common location: LA with attachment t
7、o atrial septum左房贴近房间隔Appear gelatinous and friable胶样、脆弱外观Embolic events AV valve obstruction房室瓣梗阻Carneys complex (autosomal dominant, 7% of all myomas)Recurrent, need follow-upOne week later肿块侵入右房，并出现右房腔内可移动的游离端Contrast uptake: hypervascularAngiosarcoma: Vascular structures with spindle-shaped cell
8、s and oval nucleiImmunostain with CD31 shows tumor cells are strongly positive for this specific vascular markerAngiosarcoma血管肉瘤7.3% of all cardiac tumorsMost common malignant primary cardiac tumor最为常见的原发性恶性心脏肿瘤Occurs most commonly in the RA in conjunction with pericardial effusion最常见于右房伴心包积液Cardiac
9、 TumorsPrimary cardiac masses原发性心脏肿瘤Benign良性malignant恶性Metastatic cardiac masses转移性心脏肿瘤Fake-outs “假性肿瘤”Thrombus血栓Vegetations赘生物Artifact伪影Primary Cardiac TumorsBenignMyxomaPapillary fibroelastomaLipomaHemangiomaMesotheliomaFibromaTeratomaGranular cell tumor MalignantAngiosarcomaRhabdomyosarcomaMesoth
10、eliomaFibrosarcomaLymphomaOsteosarcomaThymomaNeurogenic sarcomaWhat Are The Cardiac Manifestations?Depend on LOCATION, LOCATION,LOCATION!Cardiac ManifestationsLA: most common site for benign tumor, DOE, orthopnea, PND, crackle, S4, tumor flop, diastolic murmur左房: 良性肿瘤的最常见部位, 劳力性呼吸困难, 端坐呼吸, PND, 罗音,
11、S4, 肿瘤扑击音, 舒张期杂音RA: most common site for malignant tumor, right heart failure (fatigue, swelling), edema, JVD, TS/TR murmur右房: 恶性肿瘤的常见部位, 右心衰 (疲乏, 水肿), 颈静脉充盈, 三尖瓣杂音RV: right heart failure右室: 右心衰LV: often asymptomatic, arrhythmia or obstructive syncope (positional)左室: 常无症状, 心律失常或梗阻性晕厥 (体位性)Cardiac Ma
12、nifestationsSummary of Cardiac Tumors Cardiac tumors are rare More likely metastatic than primary转移性原发性May be symptomatic or asymptomatic Symptoms depend on location症状取决于肿瘤的位置 Myxoma is the most common primary tumor Papillary fibroelastoma is the most commonvalvular tumor乳头状弹力纤维瘤是最常见的瓣膜肿瘤Angiosarcoma is most common adult cardiacmalignancyThank You!
