Medical and surgical management of primary cardiac tumours in infants and children

Abstract

Primary cardiac tumours in infants and children are extremely rare, with an estimated incidence of 0.2% according to echocardiographic studies. Owing to their rarity, there is very little literature available, and most knowledge is based on collections of case reports. Therefore, we reviewed retrospectively our 27 years of clinical experience on the overall management of cardiac tumours among children in order to improve not only our knowledge but also to provide others with information about the incidence, clinical presentation, management, and long-term outcome of this rare disease. Between April, 1982 and April, 2009, 52 children were diagnosed with cardiac tumours at our Institution. Medical records and follow-up echocardiographic evaluations were studied. The diagnosis was prenatal in 35% of the patients. The most frequent tumour types were rhabdomyomas (61.5%), fibromas (15.4%), and myxomas (5.8%). There were no cases of primary malignant tumours. All diagnoses were achieved using two-dimensional echocardiography, and for 12 patients a pathological analysis was carried out. A total of 41 patients (79%) were managed medically, whereas 11 (21%) patients underwent surgical treatment. At a mean follow-up of 7.2 ± 5.4 years, two patients died of complications related to cardiac transplantation; all the remaining patients are in excellent clinical condition. In conclusion, cardiac tumours in paediatric practice are usually clinically and histologically benign. Only a few cases need surgery. Up to one-third of the cardiac masses are detectable prenatally. Rhabdomyoma is the most common histotype, followed by fibroma and myxoma. The long-term prognosis is generally good.

Rapid Regression of Left Ventricular Outflow Tract Rhabdomyoma After Sirolimus Therapy

“…4 Such patients occasionally need surgical resection of their tumor to alleviate hemodynamically significant critical outflow tract obstruction. 5 We report such a child in whom an alternative therapeutic strategy was used.…”

Concomitant percutaneous aortic embolectomy and excision of left atrial myxoma in a child

“…Primary cardiac tumors are very rare in children, with a reported incidence of approximately 0.17% according to a review of the echocardiographic database at Boston Children's Hospital . Rhabdomyoma is the most common, but cardiac myxoma is a very rare cardiac tumor during fetal life and childhood . In adults, myxoma is the most common cardiac neoplasm and a life‐threatening disease with symptoms and signs of dyspnea due to cavity or valve obstruction, fever, arrhythmia, systemic or pulmonary embolism, or sudden death .…”

“…Cardiac myxoma, the most common primary cardiac tumor in adults, is rare and comprises only 3-5.8% of primary cardiac tumors in infants and children. 1,2 Despite its benign pathologic nature, cardiac myxoma often presents with life-threatening clinical manifestations such as cerebral or pulmonary embolism, cardiac arrhythmia, and dyspnea due to valvular obstruction, which requires immediate therapeutic intervention. 3 We report a rare case of huge left atrial (LA) myxoma presenting with acute obstruction of the aortic bifurcation leading to acute ischemia of the both lower extremities.…”

An unusual cause of stroke in a 55-year-old woman

“…The definitive treatment of choice for cardiac myxoma is surgical resection [7,8]. Diuretics, angiotensin converting enzyme (ACE) inhibitors, beta blockers or antiarrhythmic drugs may be used for symptom management or complications such as heart failure or cardiac arrhythmias while patient is awaiting surgery [9]. There are no definitive guidelines for anticoagulation in patients presenting with embolic complications of cardiac myxoma.…”