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Tumor Frequency
- Metastatic > primary (20-40x)
- Primary benign > malignant (75% vs 25%)
- Myxoma is #1 primary benign
- Angiosarcoma is #1 primary malignant
Myxoma Triad
- Obstructive symptoms (HF, syncope, âtumor plopâ)
- Embolic phenomena (50% to brain)
- Constitutional symptoms (fever, weight loss, â ESR)
Carney Complex
- PRKAR1A mutation, autosomal dominant
- Multiple myxomas, pigmented lesions, endocrine tumors
- NAME syndrome: Nevi, Atrial myxoma, Myxoid neurofibroma, Ephelides
- LAMB syndrome: Lentigines, Atrial myxoma, Blue nevi
Tuberous Sclerosis + Cardiac
- Rhabdomyoma in > 50% of TS patients
- TSC1/TSC2 mutation
- Multiple, ventricular
- Often regresses; mTOR inhibitor (everolimus) for symptomatic
Imaging Modalities
- Echo (TTE/TEE): first-line
- CMR: tissue characterization (T1, T2, LGE, perfusion)
- PET-CT: benign vs malignant (FDG avidity)
- CT: calcifications (fibroma)
Most Common Tumor at Each Site
- LA: myxoma
- RA: angiosarcoma (malignant)
- Valves: papillary fibroelastoma
- Ventricular (children): rhabdomyoma, fibroma
- Pericardium: metastatic, primary lymphoma
Tumor Behavior
- Mobile + pedunculated: myxoma, fibroelastoma
- Invasive + ill-defined: angiosarcoma, metastases
- Multiple: tuberous sclerosis (rhabdomyoma), metastatic
- Calcified: fibroma, calcified myxoma
ææ··æ·æ¯èŒ
| Myxoma |
LA fossa ovalis |
F adults |
T2 hyper, mobile |
Surgical excision |
| Papillary fibroelastoma |
Aortic valve |
Adults |
Small, frond-like |
Excision if symptomatic |
| Lipoma |
LA / subepicard |
Adults |
T1 hyper (fat) |
Excision if symptomatic |
| Rhabdomyoma |
Ventricles |
Children, TS |
Multiple, calcified |
mTOR / observation |
| Fibroma |
Ventricular |
Children |
Calcified |
Surgery |
| Angiosarcoma |
RA |
30-50 yo |
Heterogeneous, vascular |
Surgery + chemo |
| Metastatic |
Pericardium |
Cancer hx |
Pericardial effusion |
Treat primary |
Special Topics
Tumor vs Thrombus on Imaging
- Thrombus: non-enhancing on CMR, no FDG
- Tumor: enhances, ± FDG (malignant)
- AC trial helpful in atypical cases
Genetic Syndromes + Cardiac Tumors
- Carney complex (PRKAR1A) â myxoma
- Tuberous sclerosis (TSC1/2) â rhabdomyoma
- Gorlin syndrome â fibroma
- NF1 â neurofibroma (rare cardiac)
- Beckwith-Wiedemann â rhabdomyoma