Arhabdomyoma is a benigntumorofstriated muscle. Rhabdomyomas may be either cardiac or extracardiac (occurring outside the heart). Extracardiac forms of rhabdomyoma are sub-classified into three distinct types: adult type, fetal type, and genital type.
Cardiac rhabdomyomas are the most common primary tumor of the heart in infants and children. It has an association with tuberous sclerosis.[1] In those with tuberous sclerosis, the tumor may regress and disappear completely, or remain consistent in size.[2] A common histological feature is the presence of spider cells, which are cardiac myocytes with enlarged glycogen vacuoles separated by eosinophilic strands, resembling the legs of a spider.
It is most commonly associated with the tongue,[3] and heart,[4] but can also occur in other locations, such as the vagina.[5]
It is much more common to find metastasis from an alternate site than a primary heart tumor.[7] However, primary cardiac tumors do occur. One of these is the cardiac rhabdomyoma. Approximately 80-90% of these tumors are found in patients with tuberous sclerosis, a genetic condition causing multiple tumors, with most found prior to the age of one.[8] Although these tumors are most commonly treated with resection, symptomatic tumors in fetuses have been shown to decrease in size after maternal sicrolimus administration.[9] If clinically silent, they can be watched with routine imaging as the tumor will likely spontaneously regress.[2]
The specific clinical picture of a patient with a cardiac rhabdomyoma is determined by its location in the heart.[10] However, if symptomatic, most patients will present with heart failure or an arrhythmia such as ventricular tachycardiaorheart block.[11] The severity of the symptoms will vary based on the size of the mass.
^Bader RS, Chitayat D, Kelly E, et al. (November 2003). "Fetal rhabdomyoma: prenatal diagnosis, clinical outcome, and incidence of associated tuberous sclerosis complex". J. Pediatr. 143 (5): 620–4. doi:10.1067/S0022-3476(03)00494-3. PMID14615733.