cardiac masses - nt cardiovascular心脏肿瘤- nt心血管

CARDIAC MASSES Nick Tehrani, MD Outline General Primary Cardiac tumors Benign Malignant Secondary Cardiac Tumors Benign Malignant Q U I Z TIME General Neoplasia of the heart or pericardium is more likely to be: Secondary, than Primary General Primary tumors 35 75% in the Lt. Atrium near fossa ovalis 15% Rt. Atrium 5-10% Lt. Ventricle 5% multiple sitesWill discuss further Myxomas: Benign Primary Cardiac Tumors Grossly Typically pedunculated Gelatinous consistency Friable Histologically Copious mucopolysaccharide stroma Scattered solitary or clustered polygonal cells. Myxomas: Left atrial Myxoma Benign Primary Cardiac Tumors Extra cardiac manifestations suggestive of Collagen Vascular dz : Fever ESR elevation Anemia Thrombocythemia Circulating autoantibodies Myxomas: Benign Primary Cardiac Tumors Etiology of immunologic manifestations ? ? ? ? Tumor necrosis Anitimyocardial antibody titers decline post resection Myxomas: Benign Primary Cardiac Tumors High propensity for embolization Surgical results excellent Decision to operate: Lt. versus Rt. Sided tumor Sx. Age Co morbidities Management of Myxomas: Benign Primary Cardiac Tumors Familial pattern of Myxomas with autosomal dominant features Age 60 Papillary Fibroelastoma: (aka Papilloma) Benign Primary Cardiac Tumors Small tumors (1cm) with homogeneous speckeled pattern Commonly pedunculated with multiple fronds Affect the Lt and Rt sides with same frequency Attach to Atrial surface of AV valves, and Ventricular surface of semilunar valves Papillary Fibroelastoma: Benign Primary Cardiac Tumors Adults Aortic valve Children Tricuspid valve Rarely on endocardial surfaces Symptomatic only in the aortic position (other than embolic Sx) Ostial occlusion angina, sudden death Rarely valvular dysfunction Source of embolization in up to 30% of patients Papillary Fibroelastoma: Benign Primary Cardiac Tumors Surgical resection is not uniformly necessary Anticoagulation may be sufficient in many patients Surgery indicated for Embolic events Ostial occlusion Topol Textbook of Cardiovascular Medicine All fibroelastomas, “any size, any age” are to be resectedSeward, ACC 2000 Management of Papillary Fibroelastoma Primary Cardiac Tumors Benign Myxoma Papillary fibroelastoma Fibroma Benign Primary Cardiac Tumors Encapsulated, solitary tumors Frequently in the septal myocardium Often encroach on the conduction system as they grow Fibromas: Benign Primary Cardiac Tumors With septal involvement V.Fib is often the first presentation Indications for surgical resection: Mechanical problems due to size Arrhythmogenic nidus Resection of septal fibromas is not always possible Fibromas: Benign Primary Cardiac Tumors Lipomas: Affect both myocardium and pericardium Can reach several centimeters in size Benign Primary Cardiac Tumors Rhabdomyoma: Most common tumor of the heart for Age 30 yo Almost exclusively in children Associated with tuberous sclerosis Regression of tumor in infancy has been reported Outline General Primary Cardiac tumors Benign Malignant Malignant Primary Cardiac Tumors Angiosarcomas Rhabdomyosarcomas Mesotheliomas Lymphoma Intrapericardial Pheochromocytomas Malignant Primary Cardiac Tumors Most common primary malignancy of the heart Malignant cells that form vascular channels Most commonly affect the Rt. Heart Rt. Atrium Pericardium Hemorrhagic effusion Thrombus Angiosarcomas: Malignant Primary Cardiac Tumors Diffuse, irregularly shaped Mean survival one year Successful Rx with Chemo and XRT followed by transplant reported Angiosarcomas: Malignant Primary Cardiac Tumors Most commonly seen in adults No chamber selectivity No pericardial involvement Multiple sites of cardiac involvement is common Poor prognosis Limited success with resection and adjuvant Rx. Rhabdomyosarcomas: Undifferentiated Sarcoma Malignant Primary Cardiac Tumors Diffuse pericardial tumor Involve both parietal and visceral pericardium Superficially invade the myocardium Rarely invade the cardiac chambers Mesotheliomas: Malignant Primary Cardiac Tumors Sx of pericarditis or tamponade Poor prognosis XTR or chemo only offer temporary improvement Mesotheliomas: Malignant Primary Cardiac Tumors Lymphoma: Heart and pericardium are the only affected organs No predilection for any particular site Malignant Primary Cardiac Tumors Soft, fleshy, highly vascular Anatomic location AV groove Atrium Interatrial septum Coronary, Pulmonary, Aorticopulmonary regions Generally very difficult to resect Intrapericardial Pheochromocytomas: Malignant Primary Cardiac Tumors Generalizations: Pulmonary vein mass Malignancy Lateral wall of the LA Malignancy Atrial septum Myxoma Outline General Primary Cardiac tumors Benign Malignant Secondary Cardiac Tumors Malignant Malignant Secondary Cardiac Tumors Incidence of Solid Tumors involving the heart: Lung Breast Soft tissue sarcomas Renal carcinoma Melanoma Leukemia and Lymphomas also common culprits What is this? Malignant Secondary Cardiac Tumors IVC tumors in General Renal carcinoma 80% 5 year survival for surgical resection of tumors migrating up the IVC Hepatoma Ovarian CA Outline General Primary Cardiac tumors Benign Malignant Secondary Cardiac Tumors Malignant Benign Benign Secondary Cardiac Tumors Hints: IVC tumor Long stringy appearance Seen many years post hysterectomy LEIOMYOMATOSIS Benign Secondary Cardiac Tumors Akin to Fibroids Controlled by hormone suppression Can degenerate into very low grade sarcoma Histologically, similar structures are present in the venous channels of the uterus Leiomyomatosis: HIV and cardiac tumors Kaposis sarcoma can involve the myocardium or pericardium Non-Hodgkins lymphoma Can present as primary cardiac lymphoma When involving the heart usually diffusely infiltrative Variable results with chemo and XRT Another Benign Secondary CardiacTumor How about this one? APICAL MASSES Differential Dx for Apical Masses Thrombus Leoflers Endomyocardial fibrosis (seen in tropics) NL Wall Motion Outline General Primary Cardiac tumors Benign Malignant Secondary Cardiac Tumors Malignant Benign Surgical Options Surgical Options Primary Cardiac Tumors Rare entity, 3/10,000 autopsies Surgical options Benign majority resectable 6% rate of recurrence Malignant seldom resectable Surgery often intended for tissue diagnosis (benign vs. malignant) Surgical Options If surgical resection is contemplated, thorough metastatic evaluation is necessary: CT Head Chest Abd Bone scan if indicated Bone marrow Bx if indicated Surgical Options Total of 28 patients have undergone orthotopic heart transplantation for inoperable primary cardiac tumors: 7 had benign histology Mean survival of 46 months 21 had malignant histology Mean survival 12 months Surgical Options OF the 7 with benign histology Fibroma 5 patients Rhabdomyoma 1 Pheochromocytoma 1 Survival range: 8 to 105 months Survival mean: 46 months No patients had recurrence of tumor Two deaths due to rejection Surgical Options OF the 21 with malignant histology Sarcoma 15 Malignant fibrohistiocytoma 3 Lymphoma 3 14/21 died between 1 and 36 months (mean 12 months) 13/14 died of recurrent mets 1/14 cause of death unknown 7 others; follow up 6 66 months one had recurrence malignancy Surgical