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Cardiac Tumors,Cynthia Taub, M.D., F.A.C.C., F.A.S.E. Director, Non-invasive Cardiology Director, Womens Heart Health Center Einstein Division Montefiore Medical Center October 2009,Dyspnea,A 33 year old male presented with dyspnea on exertion for 1 week No angina, palpitations, syncope, fever, night sweats or weight loss,33 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 1,Non-specific fibrous tissue and fibrinoid material非特异性纤维组织和纤维素样物质,Biopsy 2,Cardiac arrest during anesthesia induction,Burkits Lymphoma,PET Scan,Hypermetabolic 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,Treatment,Systemic chemotherapy with Ommaya reservior/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(-) adult,1-2% of lymphoma in adults in the US Fast growing Usually involves abdomen May respond to chemotherapy,Anterior 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 Mediastinal 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 pressure,Perivascular spaces with lighter staining matrix,Multinucleated myxoma cells,Myxoma粘液瘤,Most common primary cardiac tumor (20-30%) Most common 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-up,Myxoma粘液瘤,Most common primary cardiac tumor (20-30%) Most common 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-up,One week later,肿块侵入右房,并出现右房腔内可移动的游离端,Contrast uptake: hypervascular,Angiosarcoma: Vascular structures with spindle-shaped cells and oval nuclei,Immunostain with CD31 shows tumor cells are strongly positive for this specific vascular marker,Angiosarcoma血管肉瘤,7.3% of all cardiac tumors Most 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 Tumors,Primary 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 Tumors,BenignMyxoma Papillary fibroelastoma Lipoma Hemangioma Mesothelioma Fibroma Teratoma Granular cell tumor,MalignantAngiosarcoma Rhabdomyosarcoma Mesothelioma Fibrosarcoma Lymphoma Osteosarcoma Thymoma Neurogenic sarcoma,What Are The Cardiac Manifestations?,Depend on LOCATION, LOCATION,LOCATION!,Cardiac Manifestations,LA: 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, 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 Manifestations,Summary 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 common valvular tumor乳头状弹力纤维瘤是最常见的瓣膜肿瘤 Angiosarcoma is most common adult cardiac malignancy,Thank You!,
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