Primary Cardiac tumors Benign M a li g n a nt Secondary Cardiac Tumors Benign M a li g n a nt
General N eo p l as ia o f t h e h e a rt o r pe r i ca r d i u m : Secondary more common than Primary
General Primary tumors <3/10,000 autopsies Over ¾ are benign Secondary 20-40 x more common Present in 20% of patients dying of malignancy Prevalence :
Primary Cardiac Tumors B e n i g n Myxoma Papillary fibroelastoma Fibroma Lipoma Rhabdomyomas
Benign Primary Cardiac Tumors The most common primary cardiac tumor in the Age > 35 75% in the Lt. Atrium near fossa ovalis 15% Rt. Atrium 5 - 1 0% Lt. V e n t r i c l e 5% multiple sites M y x om as
Manangement High propensity for embolization Surgical results excellent
Benign Primary Cardiac Tumors Familial pattern of Myxomas with autosomal dominant features Age < 30 Carney Complex
Papillary Fibroelastoma The most common valvular tumor, followed by Sarcoma Melanoma Present in all age groups, but most commonly in Age>60
Papillary Fibroelastoma 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 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 patien ts
F i b r o m a s Encapsulated, solitary tumors Frequently in the septal myocardium Often encroach on the conduction system as they grow
F i b r o m a s 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 a lways possible
L i po m a s Affect both myocardium and pericardium Can reach several centimeters in size
Rhabdomyoma: Most common tumor of the heart for Age < 30 yr Almost exclusively in children Associated with tuberous sclerosis R e g r es s i on of tu m or i n i nfan c y ha s b e en reported
A large firm, white tumor mass was found filling the left ventricle. This is a cardiac rhabdomyoma.
Malignant Primary Cardiac Tumors Angiosarcomas R h a b d o m y o sa r co m as Mesotheliomas Lymphoma Intrapericardial Pheochromocytomas
Angiosarcomas 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 Diffuse, irregularly shaped Mean survival one year Successful Rx with Chemo and XRT followed by transplant reported
Rhabdomyosarcomas 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.
M esoth e l i o m a s Diffuse pericardial tumor Involve both parietal and visceral pericardium Superficially invade the myocardium Rarely invade the cardiac chambers
M esoth e l i o m a s Sx of pericarditis or tamponade Poor prognosis XTR or chemo only offer temporary improvement
L y m ph o m a
Malignant Secondary Cardiac Tumors Solid Tumors involving the heart: Lung B r ea s t Soft tissue sarcomas Renal carcinoma Melanoma L e uke m i a a n d L y m p h o m a s a l s o co m m o n culprits
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