Features of cardiac rhabdomyoma:
(1) They are the most common primary cardiac tumor during childhood and can present in utero.
(2)
They often are associated with the tuberous sclerosis complex.
(3) There can be multiple tumors, especially in patients with tuberous sclerosis.
(4) They usually arise in the ventricles.
(5) The patient may present with heart failure, cardiac arrhythmia and/or murmur.
(6) They often appear as a pedunculated mass on imaging studies.
(7) They may regress spontaneously.
Reasons for surgery may include:
(1) The patient is symptomatic from flow obstruction.
(2) There is uncertainty about the diagnosis.
Therapy with everolimus can cause the tumors to regress and even disappear. Everolimus is an inhibitor of mammalian target of rapamycin (mTOR).
