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.