Primary heart tumors in the pediatric age group: A review of salient pathologic features relevant for clinicians

Because primary tumors of the heart in infants and children are extremely r are, most knowledge is based on collections of case reports rather than lar ge cohort studies. The types of heart tumors encountered in the pediatric a ge group differ from those seen in adults. In the latter, cardiac myxomas a re by far the most common tumor; in infants and children the most common pr imary tumor of the heart is the rhabdomyoma. Spontaneous regression of thes e tumors has been well established so that surgical intervention is no long er indicated unless there are clinical manifestations from the heart. There is a high incidence of associated tuberous sclerosis. Any intracavitary ma ss in infants is suggestive for a cardiac rhabdomyoma unless otherwise prov en. The second most common tumor in this age group is cardiac fibroma. Thes e tumors probably represent hamartomatous lesions and this has led to a str ategy in which radical surgical excision is not indicated if the procedure endangers postoperative heart function. Cardiac myxomas are of interest in this age group because of their familial occurrence as part of the myxoma s yndrome. Other types of primary heart tumors occur, including malignant var iants, but all are exceedingly rare. It is because of the sporadic nature o f these tumors that treatment and insights into prognosis are based largely on case documentations and analogies from similar tumors originating elsew here.