- Cardiac tumours are rare.
- They are most often from metastatic disease, but primary cardiac tumours can occur.
- Approximately 75% of primary cardiac tumours are benign the vast majority of these being atrial myxomas.
- Malignant tumours are usually sarcomas.
- Clinically presentation can vary from asymptomatic to life-threatening complications such as valve obstruction, embolism, or arrhythmia.
Benign Tumours
Atrial Myxoma
- Most common cardiac tumour identified.
- More commonly diagnosed in women in the 5th to 7th decade of life.
- Usually identified in the left atrium (80-90%), they have a stalk, and are often attached the interatrial septum.
- Patients may have tumour embolus, functional mitral stenosis from obstructing tumour, or constitutional symptoms.
- Atrial myxoma’s can be familial and family screening should be considered. “Carney Complex” autosomal dominant disease associated with multiple cardiac tumours and extra cardiac myxoma’s.
- Treatment involves surgical resection.
- They can reoccur and frequent screening with a transthoracic echo should be done post resection.
Papillary Fibroelastoma
- Small tumours that adhere to the valves.
- Most commonly effect the aortic valve.
- They are usually solitary lesions that often resemble a “Sea Anemone”
- Can be mistaken for valvular vegetations.
- Though previously though to be of little conciquence they are associated with thromboembolism.
- Surgical resection is generally indicated if greater than 1 cm or embolism or highly mobile.
Rhabdomyoma
- Most common cardiac tumour in children.
- Usually multiple tumours in the ventricle can be associated with outflow tract obstruction.
- Associated with tuberous Sclerosis
Lipoma
- Usually subendocardial or subpericardial.
- Can be associated with arrythmias or AV block
- MRI can be helpful at characterizing the lipoma.
Primary Malignant Tumours
Angiosarcoma
- Most commonly seen in men.
- Usually found in the right atrium usually beside the IVC.
- Frequently metastatic disease found at time of the diagnosis.
- Very poor prognosis, survival often less than a year.
Rhabdomyosarcoma
- Commonly seen in children and young adults.
- Often have multiple lesions with may involve any chamber.
- Very poor prognosis with survival less than a year.
Leiomyosarcoma
- Commonly seen in the left Atrium.
- Usually solitary lesion.
- Mean survival 6 months.
Cardiac Lymphoma
- Commonly seen in the right heart.
- Usually solitary.
- Often associated with pericardial effusion.
- Frequently have extracardiac manifestations.
Secondary Tumours
- Metastatic cancers are exceptionally more common than primary malignant tumours.
- Carcinoma of the breast and lung can have local invasion.
- Renal cell cancers and invade up the inferior vena cava.
- Melanomas often have a predilection for metastasizing to the heart.
References
Shapiro LM Cardiac tumours: diagnosis and management Heart 2001;85:218-222.