Key points
- •
Cardiac tumors consist of a diverse group of conditions with various characteristics, most of them being benign lesions
- •
Association of benign cardiac lesions with genetic syndromes mandates thorough evaluation of these patients
- •
Most common type of cardiac benign tumors is myxoma followed by fibroelastoma, rhabdomyoma, fibroma, lipoma, and other less common types
- •
Presentation and symptoms depend on the tumor location and histopathology, including constitutional symptoms, thromboembolism, obstructive symptoms, and arrhythmias
- •
Diagnosis is by echocardiography, and frequently cardiac MRI
- •
Treatment of benign tumors is mainly by excision or close observation
Cardiac tumors cause symptoms by various means. Aortic valve and left atrial tumors are more likely to be complicated by embolization, which is mostly systemic . Obstructing blood flow might result in heart failure symptoms. Valvular regurgitation is also possible if the tumor interferes with the heart valves. Though true invasion is less likely with benign tumors, direct pressure by the mass may lead to ventricular dysfunction, arrhythmias, or pericardial effusion. The pressure effect of tumor may also cause bronchogenic symptoms. Constitutional symptoms like fatigue, weakness, and light headedness are also noticeable.
Mechanism | Sign/symptom | Example | |
---|---|---|---|
Left atrium | Obstruction Embolization | Dyspnea, pulmonary edema, fatigue, cough, orthopnea Mitral regurgitation Tumor plop Stroke | Myxoma Lipoma |
Right atrium | Obstruction Embolization | Peripheral edema, hepatomegaly, ascites, fatigue Tricuspid regurgitation Pulmonary emboli | Myxoma Lipoma |
Left ventricle | Obstruction Embolization Direct effect | Heart failure Arrhythmia | Fibroma Rhabdomyoma |
Right ventricle | Obstruction | Peripheral edema, hepatomegaly, ascites, syncope | Fibroma Rhabdomyoma |
Valves | Valve interference | Regurgitation | Fibroelastoma |
Genetic syndrome | Gene involved | Inheritance | Tumor type | Association |
---|---|---|---|---|
Gorlin syndrome | PTCH1 | AD | Fibroma | Nevoid basal cell carcinoma, skeletal abnormalities, medulloblastoma, meningioma |
Carney complex | PRKAR1A | AD | Myxoma | Endocrine hyperfunction, skin pigmentation |
Von Hippel–Lindau syndrome | VHL | AD | Paraganglioma | Renal cell carcinoma, pancreatic NET |
Tuberous Sclerosis | TSC1 , TSC2 | AD | Rhabdomyoma | Hamartoma, brain tumors, angiomyolipoma, skin changes |
Indication | Considerations | |
---|---|---|
Imaging | ||
Echocardiography Cardiac CT Cardiac MRI PET scan | Initial study MRI unavailable MRI prohibited Functional assessment |
|
Coronary angiography | ||
Epicardial tumors |
| |
Biopsy | ||
Unresectable or diffuse tumors |
|